Intravascular hemolysis and the pathophysiology of sickle cell disease
Open accessAbstract
flow, normoxia to hypoxia, isotonic to hypertonic environment, and acidotic to alkalotic milieu.Multiple components contribute to sickle hemoglobinopathy pathophysiology, including primary components arising from HbS polymerization and secondary components that are downstream effects of the HbS polymer. Understanding how these components' complexity is compounded by genetic and environmental modulation provides insight into the well-known clinical heterogeneity of sickle cell disease (SCD).A cardinal feature of SCD pathogenesis involves inflammation, accompanied by heterocellular leukocyte-platelet-erythrocyte-endothelial adhesive events that trigger vaso-occlusive episodes, acute organ ischemia, and reperfusion injury.Twenty-five years ago, epidemiological studies identified leukocytosis, lower HbF levels, and higher total hemoglobin levels as risk factors associated with increasing incidence of acute painful episodes and acute chest syndrome (ACS) (5).The independent association of high total hemoglobin levels with more pain, ACS events, and osteonecrosis was never mechanistically explained; however, it was implied to be a result of increased blood viscosity (Table 1).Recent epidemiological studies found that lower hemoglobin levels and higher intensity of steady-state hemolytic anemia consistently associate with vasculopathic complications of disease, such as stroke, leg ulcers, PH, priapism, and renal failure.This suggests that certain subphenotypes of SCD relate more to hemolytic anemia severity rather than sickle vaso-occlusion.The reader is referred to recent reviews describing the exceptional strides made in understanding the roles of red cell rigidity (6), inflammation, and cell adhesion in sickle vaso-occlusion (7-9).Here, we review the complementary role of intravascular hemolysis and anemia.Hemolysis is a fundamental feature of sickle cell anemia that contributes to its pathophysiology and phenotypic variability.Decompartmentalized hemoglobin, arginase 1, asymmetric dimethylarginine, and adenine nucleotides are all products of hemolysis that promote vasomotor dysfunction, proliferative vasculopathy, and a multitude of clinical complications of pulmonary and systemic vasculopathy, including pulmonary hypertension, leg ulcers, priapism, chronic kidney disease, and large-artery ischemic stroke.Nitric oxide (NO) is inactivated by cell-free hemoglobin in a dioxygenation reaction that also oxidizes hemoglobin to methemoglobin, a non-oxygen-binding form of hemoglobin that readily loses heme.Circulating hemoglobin and heme represent erythrocytic danger-associated molecular pattern (eDAMP) molecules, which activate the innate immune system and endothelium to an inflammatory, proadhesive state that promotes sickle vaso-occlusion and acute lung injury in murine models of sickle cell disease.Intravascular hemolysis can impair NO bioavailability and cause oxidative stress, altering redox balance and amplifying physiological processes that govern blood flow, hemostasis, inflammation, and angiogenesis.These pathological responses promote regional vasoconstriction and subsequent blood vessel remodeling.Thus, intravascular hemolysis represents an intrinsic mechanism for human vascular disease that manifests clinical complications in sickle cell disease and other chronic hereditary or acquired hemolytic anemias.
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As HbF falls and fewer F-cells are protected from HbS polymer damage, the propensity for vascular occlusion and intravascular hemolysis increases with the release of heme products into the circulation that scavenge nitric oxide causing endothelial damage and vasculopathy.
“As HbF falls and fewer F-cells are protected from HbS polymer damage, the propensity for vascular occlusion and intravascular hemolysis increases with the release of heme products into the circulation that scavenge nitric oxide causing endothelial damage and vasculopathy.[57][58][59]”
From Fetal hemoglobin in sickle cell anemia (2020) · cites this paper as [58]
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Experimental — accuracy not yet measuredIL-1β binding to IL-1R on leukocytes and vascular cells activates neutrophils and platelets and upregulates endothelial E-selectin, P-selectin, VCAM-1, ICAM-1, and IL-8, promoting vaso-occlusion.
“Once released, IL-1β binds to IL-1 receptor (IL-1R) on leukocytes and vascular cells, promoting a cascade of downstream events that lead to activation of neutrophils and platelets and upregulation of E-selectin, P-selectin, VCAM-1, ICAM-1, and chemokines such as IL-8 in endothelial cells, all of which promote vaso-occlusion (14, 17) (Figure 1).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [17]
The Abstract describes an inflammatory, proadhesive endothelial state that promotes sickle vaso-occlusion, and “Evidence linking cell-free hemoglobin to SCD complications” reports platelet activation. Neither passage attributes those effects to IL-1β binding IL-1R or describes the claimed neutrophil activation and endothelial upregulation of E-selectin, P-selectin, VCAM-1, ICAM-1, and IL-8. Page numbers were not provided in our copy. (LLM, conf=0.84)
Experimental — accuracy not yet measuredVaso-occlusion causes ischemia-reperfusion injury, which together with released erythrocyte damage-associated molecular patterns drives progression of sterile inflammation in SCD.
“Vaso-occlusion contributes to ischemia-reperfusion injury, which, along with release of eDAMPs, promotes the progression of sterile inflammation (Figure 1d) in SCD (14, 17, 18).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [17]
The section ‘Evidence linking cell-free hemoglobin to SCD complications’ says eDAMPs promote sterile inflammation and may be central to the inflammation of sickle vaso-occlusion. The cited paper also links vaso-occlusive episodes to enhanced eDAMP release, but the passages do not establish the claimed ischemia-reperfusion injury step; a search for that term found no supporting passage. (LLM, conf=0.85)
Experimental — accuracy not yet measuredThe hyperhemolysis phenotype of sickle cell disease is linked to stroke risk in adults.
“Studies in adults with SCD lend further support to the role of hemolysis by showing a link between the hyperhemolysis phenotype of SCD and stroke risk (17).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [17]
Table 1 (The hemolysis hypothesis; page unavailable) lists increased risk of stroke or elevated transcranial Doppler velocity with the hyperhemolytic subphenotype, and the Hyperhemolysis-associated SCD subphenotypes section includes cerebrovascular disease. The table combines stroke with Doppler findings and does not establish that the reported stroke-risk association specifically came from studies in adults. (LLM, conf=0.78)
Experimental — accuracy not yet measuredUnequal protection by HbF across the gamut of sickle cell disease complications might be partially explained by differences in the pathophysiologic roots of these complications.
“Unequal protection by HbF across the gamut of sickle cell disease complications might be partially explained by differences in the pathophysiologic roots of these complications.57,58”
From Fetal hemoglobin in sickle cell anemia (2020) · cites this paper as [58]
Acute painful episodes and acute chest syndrome are a result of sickle vasoocclusion, and HbF has the most noticeable beneficial effect on these subphenotypes.
“Acute painful episodes and acute chest syndrome are a result of sickle vasoocclusion; HbF has the most noticeable beneficial effect on these subphenotypes.”
From Fetal hemoglobin in sickle cell anemia (2020) · cites this paper as [58]
Priapism, nephropathy, and sickle vasculopathy are associated with hemolysis.
“Priapism, nephropathy, and sickle vasculopathy are associated with hemolysis.”
From Fetal hemoglobin in sickle cell anemia (2020) · cites this paper as [58]
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Cites
Survival in Patients with Primary Pulmonary Hypertension
D’Alonzo, G. E., Barst, R. J., Ayres, S. M., Bergofsky, E. H., Brundage, B. H., Detre, K. M., Fishman, A. P., Goldring, R. M., Groves, B. Μ., Kernis, J. T., et al. (1991) · Annals of Internal Medicine · doi:10.7326/0003-4819-115-5-343 · Cited by 3,488 · see citing works
Mortality in sickle cell disease. Life expectancy and risk factors for early death
Platt, O. S., Brambilla, D. J., Rosse, W. F., Milner, P. F., Castro, O., Steinberg, M. H., & Klug, P. P. (1994) · New England Journal of Medicine · doi:10.1056/nejm199406093302303 · Cited by 3,320 · see citing works
Self-citationCerebrovascular accidents in sickle cell disease: rates and risk factors.
(1998) · PubMed · doi:10.1182/blood.v91.1.288 · Cited by 1,694 · see citing works
The Clinical Sequelae of Intravascular Hemolysis and Extracellular Plasma Hemoglobin
Rother, R. P., Bell, L. B., Hillmen, P., & Gladwin, M. T. (2005) · JAMA · doi:10.1001/jama.293.13.1653 · Cited by 1,551 · see citing works
Self-citationPain in Sickle Cell Disease: Rates and Risk Factors
Platt, O. S., Thorington, B. D., Brambilla, D. J., Milner, P. F., Rosse, W. F., Vichinsky, E., & Kinney, T. R. (1991) · New England Journal of Medicine · doi:10.1056/nejm199107043250103 · Cited by 1,546 · see citing works
Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease
Reiter, C. D., Wang, X., Tanus-Santos, J. E., Hogg, N., Cannon, R. O., Schechter, A. N., & Gladwin, M. T. (2002) · Nature Medicine · doi:10.1038/nm1202-799 · Cited by 1,330 · see citing works
Self-citationPulmonary Hypertension as a Risk Factor for Death in Patients with Sickle Cell Disease
Gladwin, M. T., Sachdev, V., Jison, M. L., Shizukuda, Y., Plehn, J. F., Minter, K., Brown, B., Coles, W. A., Nichols, J. S., Ernst, I., et al. (2004) · New England Journal of Medicine · doi:10.1056/nejmoa035477 · Cited by 1,325 · see citing works
Self-citationCell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease
Reiter, C. D., Wang, X., Tanus-Santos, J. E., et al. (2002) · Nature Medicine · doi:10.1038/nm799 · Cited by 1,097 · see citing works
Kato, G. J., Gladwin, M. T., & Steinberg, M. H. (2007) · Blood Reviews · doi:10.1016/j.blre.2006.07.001 · Cited by 876 · see citing works
Self-citationCastro, O. L., Brambilla, D. J., Thorington, B. D., Reindorf, C. A., Scott, R., GILLETTE, P. N., Vera, J., & Levy, P. S. (1994) · Blood · doi:10.1182/blood.v84.2.643.643 · Cited by 805 · see citing works
Morris, C. R. (2005) · JAMA · doi:10.1001/jama.294.1.81 · Cited by 762 · see citing works
Belcher, J. D., Chen, C., Nguyen, J., Milbauer, L., Abdulla, F., Alayash, A. I., Smith, A., Nath, K. A., Hebbel, R. P., & Vercellotti, G. M. (2014) · Blood · doi:10.1182/blood-2013-04-495887 · Cited by 695 · see citing works
Kato, G. J., McGowan, V., Machado, R. F., Little, J. A., Taylor, J., Morris, C. R., Nichols, J. S., Wang, X., Poljakovic, M., Morris, S. M., et al. (2006) · Blood · doi:10.1182/blood-2005-06-2373 · Cited by 661 · see citing works
Self-citationOxidation of nitrogen-oxides by bound dioxygen in hemoproteins
Doyle, M. P., & Hoekstra, J. W. (1981) · Journal of Inorganic Biochemistry · doi:10.1016/s0162-0134(00)80291-3 · Cited by 643 · see citing works
Castro, O. L., Brambilla, D. J., Thorington, B. D., et al. (1994) · Blood · doi:10.1182/blood.v84.2.643.bloodjournal842643 · Cited by 615 · see citing works
ATMOSPHERIC OZONE AND FEMORAL FRACTURES
Burton, J. L., Ensell, F. J., Leach, J. F., & Hall, K. (1975) · The Lancet · doi:10.1016/s0140-6736(75)92450-2 · Cited by 587 · see citing works
Donadee, C., Raat, N. J. H., Kanias, T., Tejero, J., Lee, J. S., Kelley, E. E., Zhao, X., Liu, C., Reynolds, H., Azarov, I., et al. (2011) · Circulation · doi:10.1161/circulationaha.110.008698 · Cited by 577 · see citing works
Self-citationFetal hemoglobin in sickle cell anemia
Akinsheye, I., Alsultan, A., Solovieff, N., Ngo, D., Baldwin, C. T., Sebastiani, P., Chui, D. H. K., & Steinberg, M. H. (2011) · Blood · doi:10.1182/blood-2011-03-325258 · Cited by 553 · see citing works
Self-citationGill, F., Sleeper, L., Weiner, S. R., Ak, B., Bellevue, R., Grover, R., Pegelow, C. H., & Vichinsky, E. P. (1995) · Blood · doi:10.1182/blood.v86.2.776.bloodjournal862776 · Cited by 513 · see citing works
A Hemodynamic Study of Pulmonary Hypertension in Sickle Cell Disease
Parent, F., Bachir, D., Inamo, J., Lionnet, F., Driss, F., Loko, G., Habibi, A., Bennani, S. L., Savale, L., Adnot, S., et al. (2011) · New England Journal of Medicine · doi:10.1056/nejmoa1005565 · Cited by 511 · see citing works
Prediction of Adverse Outcomes in Children with Sickle Cell Disease
Miller, S. T., Sleeper, L. A., Pegelow, C. H., Enos, L. E., Wang, W. C., Weiner, S. J., Wethers, D. L., Smith, J., & Kinney, T. R. (2000) · New England Journal of Medicine · doi:10.1056/nejm200001133420203 · Cited by 508 · see citing works
Zhang, D., Xu, C., Manwani, D., & Frenette, P. S. (2016) · Blood · doi:10.1182/blood-2015-09-618538 · Cited by 413 · see citing works
Sickle Cell Disease as a Cause of Osteonecrosis of the Femoral Head
Milner, P. F. A., Kraus, A. P., Sebes, J. I., Sleeper, L. A., Dukes, K. A., Embury, S. H., Bellevue, R., Koshy, M., Moohr, J. W., & Smith, J. A. (1991) · New England Journal of Medicine · doi:10.1056/nejm199111213252104 · Cited by 384 · see citing works
Chronic Renal Failure in Sickle Cell Disease: Risk Factors, Clinical Course, and Mortality
Powars, D. R., Elliott-Mills, D. D., Chan, L. S., Niland, J. C., Hiti, A. L., Opas, L. M., & Johnson, C. S. (1991) · Annals of Internal Medicine · doi:10.7326/0003-4819-115-8-614 · Cited by 357 · see citing works
Heme-induced neutrophil extracellular traps contribute to the pathogenesis of sickle cell disease
Chen, G., Zhang, D., Fuchs, T. A., Manwani, D., Wagner, D. D., & Frenette, P. S. (2014) · Blood · doi:10.1182/blood-2013-10-529982 · Cited by 354 · see citing works
Hemolysis-induced lethality involves inflammasome activation by heme
Dutra, F. F., Alves, L. S., Rodrigues, D., Fernandez, P. L., de Oliveira, R. B., Golenbock, D. T., Zamboni, D. S., & Bozza, M. T. (2014) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1405023111 · Cited by 353 · see citing works
Minneci, P. C. (2005) · Journal of Clinical Investigation · doi:10.1172/jci25040 · Cited by 329 · see citing works
Genetic modifiers of sickle cell disease
Steinberg, M. H., & Sebastiani, P. (2012) · American Journal of Hematology · doi:10.1002/ajh.23232 · Cited by 315 · see citing works
Self-citationBarabino, G. A., Platt, M. O., & Kaul, D. K. (2010) · Annual Review of Biomedical Engineering · doi:10.1146/annurev-bioeng-070909-105339 · Cited by 311 · see citing works
Silent cerebral infarcts in sickle cell anemia: a risk factor analysis
Kinney, T. R., Sleeper, L. A., Wang, W. C., Zimmerman, R. A., Pegelow, C. H., Ohene-Frempong, K., Wethers, D. L., Bello, J. A., Vichinsky, E. P., Moser, F. G., et al. (1999) · PEDIATRICS · doi:10.1542/peds.103.3.640 · Cited by 309 · see citing works
Pulmonary hypertension in patients with sickle cell disease: a longitudinal study
Ataga, K. I., Moore, C. G., Jones, S., Olajide, O., Strayhorn, D., Hinderliter, A., & Orringer, E. P. (2006) · British Journal of Haematology · doi:10.1111/j.1365-2141.2006.06110.x · Cited by 298 · see citing works
Yeo, T. W., Lampah, D. A., Gitawati, R., Tjitra, E., Kenangalem, E., McNeil, Y., Darcy, C. J., Granger, D. L., Weinberg, J. B., Lopansri, B. K., et al. (2007) · The Journal of Experimental Medicine · doi:10.1084/jem.20070819 · Cited by 295 · see citing works
Extracellular hemin crisis triggers acute chest syndrome in sickle mice
Ghosh, S., Adisa, O. A., Chappa, P., Tan, F., Jackson, K. A., Archer, D. R., & Ofori-Acquah, S. F. (2013) · Journal of Clinical Investigation · doi:10.1172/jci64578 · Cited by 282 · see citing works
Baek, J. H., D’Agnillo, F., Vallelian, F., Pereira, C. P., Williams, M. C., Jia, Y., Schaer, D. J., & Buehler, P. W. (2012) · Journal of Clinical Investigation · doi:10.1172/jci59770 · Cited by 280 · see citing works
Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease
Fonsêca, G. H. H., Souza, R., Salemi, V. M. C., Jardim, C., & Gualandro, S. F. M. (2011) · European Respiratory Journal · doi:10.1183/09031936.00134410 · Cited by 273 · see citing works
Anthi, A., Machado, R. F., Jison, M. L., Taveira‐DaSilva, A. M., Rubin, L. J., Hunter, L. A., Hunter, C. J., Coles, W. A., Nichols, J., Avila, N. A., et al. (2007) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200610-1498oc · Cited by 270 · see citing works
Self-citationVinchi, F., Costa da Silva, M., Ingoglia, G., Petrillo, S., Brinkman, N., Zuercher, A., Cerwenka, A., Tolosano, E., & Muckenthaler, M. U. (2016) · Blood · doi:10.1182/blood-2015-08-663245 · Cited by 269 · see citing works
Fetal hemoglobin in sickle cell anemia: a glass half full?
Steinberg, M. H., Chui, D. H. K., Dover, G. J., Sebastiani, P., & Alsultan, A. (2014) · Blood · doi:10.1182/blood-2013-09-528067 · Cited by 267 · see citing works
Self-citationHsu, L. L., Champion, H. C., Campbell-Lee, S. A., Bivalacqua, T. J., Manci, E. A., Diwan, B. A., Schimel, D. M., Cochard, A. E., Wang, X., Schechter, A. N., et al. (2007) · Blood · doi:10.1182/blood-2006-08-039438 · Cited by 263 · see citing works
Self-citationCamus, S. M., Moraes, J. A., Bonnin, P., Abbyad, P., Jeune, S. L., Lionnet, F., Loufrani, L., Grimaud, L., Lambry, J., Charue, D., et al. (2015) · Blood · doi:10.1182/blood-2014-07-589283 · Cited by 261 · see citing works
Heme Degradation and Vascular Injury
Belcher, J. D., Beckman, J. D., Balla, G., Balla, J., & Vercellotti, G. (2010) · Antioxidants & Redox Signaling · doi:10.1089/ars.2009.2822 · Cited by 257 · see citing works
Machado, R. F., Barst, R. J., Yovetich, N. A., Hassell, K. L., Kato, G. J., Gordeuk, V. R., Gibbs, J. S. R., Little, J. A., Schraufnagel, D. E., Krishnamurti, L., et al. (2011) · Blood · doi:10.1182/blood-2010-09-306167 · Cited by 250 · see citing works
Self-citationCastro, L. M. D., Jonassaint, J., Graham, F. L., Ashley‐Koch, A. E., & Telen, M. J. (2007) · American Journal of Hematology · doi:10.1002/ajh.21058 · Cited by 243 · see citing works
Leg ulcers in patients with sickle cell disease [see comments]
Koshy, M., Entsuah, R., Koranda, A., Kraus, A., Johnson, R., Bellvue, R., Flournoy-Gill, Z., & Levy, P. S. (1989) · Blood · doi:10.1182/blood.v74.4.1403.1403 · Cited by 239 · see citing works
Hemoglobins S and C Interfere with Actin Remodeling in Plasmodium falciparum –Infected Erythrocytes
Cyrklaff, M., Sanchez, C. P., Kilian, N., Bisseyé, C., Simporè, J., Frischknecht, F., & Lanzer, M. (2011) · Science · doi:10.1126/science.1213775 · Cited by 239 · see citing works
Vinchi, F., Franceschi, L. D., Ghigo, A., Townes, T. M., Cimino, J. J., Silengo, L., Hirsch, E., Altruda, F., & Tolosano, E. (2013) · Circulation · doi:10.1161/circulationaha.112.130179 · Cited by 233 · see citing works
Nitric oxide donor properties of hydroxyurea in patients with sickle cell disease
Gladwin, M. T., Shelhamer, J. H., Ognibene, F. P., Pease‐Fye, M. E., Nichols, J. S., Link, B., Patel, D. B., Jankowski, M. A., Pannell, L. K., Schechter, A. N., et al. (2002) · British Journal of Haematology · doi:10.1046/j.1365-2141.2002.03274.x · Cited by 230 · see citing works
Self-citationMortality in Adults With Sickle Cell Disease and Pulmonary Hypertension
Mehari, A., Gladwin, M. T., Tian, X., Machado, R. F., & Kato, G. J. (2012) · JAMA · doi:10.1001/jama.2012.358 · Cited by 229 · see citing works
Self-citationPriapism and Impotence in Homozygous Sickle Cell Disease
Emond, A. M. (1980) · Archives of Internal Medicine · doi:10.1001/archinte.1980.00330220022011 · Cited by 229 · see citing works
Ataga, K. I., Reid, M. E., Ballas, S. K., Yasin, Z., Bigelow, C. L., James, L. S., Smith, W. R., Galactéros, F., Kutlar, A., Hull, J. H., et al. (2011) · British Journal of Haematology · doi:10.1111/j.1365-2141.2010.08520.x · Cited by 227 · see citing works
Nouraie, M. S., Lee, J. S., Zhang, Y., Kanias, T., Zhao, X., Xiong, Z., Oriss, T. B., Zeng, Q., Kato, G. J., Gibbs, J. S. R., et al. (2012) · Haematologica · doi:10.3324/haematol.2012.068965 · Cited by 227 · see citing works
Self-citationJison, M. L., Munson, P. J., Barb, J. J., Suffredini, A. F., Talwar, S., Logun, C., Raghavachari, N., Beigel, J. H., Shelhamer, J. H., Danner, R. L., et al. (2004) · Blood · doi:10.1182/blood-2003-08-2760 · Cited by 221 · see citing works
Self-citationLow nitric oxide bioavailability contributes to the genesis of experimental cerebral malaria
Gramaglia, I., Sobolewski, P., Meays, D., Contreras, R., Nolan, J. P., Frangos, J. A., Intaglietta, M., & van der Heyde, H. C. (2006) · Nature Medicine · doi:10.1038/nm1499 · Cited by 215 · see citing works
Hemolysis-associated priapism in sickle cell disease
Nolan, V. G., Wyszynski, D. F., Farrer, L. A., & Steinberg, M. H. (2005) · Blood · doi:10.1182/blood-2005-04-1594 · Cited by 214 · see citing works
Self-citationPulmonary hypertension and nitric oxide depletion in sickle cell disease
Bunn, H. F., Nathan, D. G., Dover, G. J., Hebbel, R. P., Platt, O. S., Rosse, W. F., & Ware, R. E. (2010) · Blood · doi:10.1182/blood-2010-02-268193 · Cited by 200 · see citing works
N-Terminal Pro-Brain Natriuretic Peptide Levels and Risk of Death in Sickle Cell Disease
Machado, R. F., Anthi, A., Steinberg, M. H., Bonds, D., Sachdev, V., Kato, G. J., Taveira-DaSilva, A. M., Ballas, S. K., Blackwelder, W., Xu, X., et al. (2006) · JAMA · doi:10.1001/jama.296.3.310 · Cited by 197 · see citing works
Self-citationHemolysis and cell-free hemoglobin drive an intrinsic mechanism for human disease
Gladwin, M. T., Kanias, T., & Kim-Shapiro, D. B. (2012) · Journal of Clinical Investigation · doi:10.1172/jci62972 · Cited by 196 · see citing works
Self-citationNaik, R. P. P., Streiff, M. B., Haywood, C., Nelson, J. A., & Lanzkron, S. (2013) · The American Journal of Medicine · doi:10.1016/j.amjmed.2012.12.016 · Cited by 190 · see citing works
The Redox Activity of Hemoglobins: From Physiologic Functions to Pathologic Mechanisms
Reeder, B. J. (2010) · Antioxidants & Redox Signaling · doi:10.1089/ars.2009.2974 · Cited by 189 · see citing works
Minniti, C. P., Sable, C., Campbell, A., RANA, S. R., Ensing, G. J., Dham, N., Onyekwere, O. C., Nouraie, M. S., Kato, G. J., Gladwin, M. T., et al. (2009) · Haematologica · doi:10.3324/haematol.13812 · Cited by 186 · see citing works
Self-citationSilent infarcts in young children with sickle cell disease
Kwiatkowski, J. L., Zimmerman, R. A., Pollock, A. N., Seto, W., Smith‐Whitley, K., Justine Shults, A., Blackwood‐Chirchir, A., & Ohene‐Frempong, K. (2009) · British Journal of Haematology · doi:10.1111/j.1365-2141.2009.07753.x · Cited by 184 · see citing works
Taylor, J. G., Nolan, V. G., Mendelsohn, L. G., Kato, G. J., Gladwin, M. T., & Steinberg, M. H. (2008) · PLoS ONE · doi:10.1371/journal.pone.0002095 · Cited by 184 · see citing works
Self-citationSICKLE CELL DISEASE: ROLE OF REACTIVE OXYGEN AND NITROGEN METABOLITES
Wood, K. C., & Granger, D. N. (2007) · Clinical and Experimental Pharmacology and Physiology · doi:10.1111/j.1440-1681.2007.04639.x · Cited by 167 · see citing works
Hebbel, R. P. (2010) · American Journal of Hematology · doi:10.1002/ajh.21952 · Cited by 167 · see citing works
Glomerular Hyperfiltration in Adult Sickle Cell Anemia
Haymann, J., Stankovic, K., Lévy, P., Avellino, V., Tharaux, P., Letavernier, E., Grateau, G., Baud, L., Girot, R., & Lionnet, F. (2010) · Clinical Journal of the American Society of Nephrology · doi:10.2215/cjn.08511109 · Cited by 167 · see citing works
Bernaudin, F., Verlhac, S., Chevret, S., Torres, M., Coic, L., Arnaud, C., Kamdem, A., Hau, I., Grazia Neonato, M., & Delacourt, C. (2008) · Blood · doi:10.1182/blood-2008-03-143891 · Cited by 162 · see citing works
The painful crisis of homozygous sickle cell disease. A study of the risk factors
Baum, K. F. (1987) · Archives of Internal Medicine · doi:10.1001/archinte.147.7.1231 · Cited by 161 · see citing works
Red cell DAMPs and inflammation
Mendonça, R., Silveira, A. A. A., & Conran, N. (2016) · Inflammation Research · doi:10.1007/s00011-016-0955-9 · Cited by 157 · see citing works
Pulmonary hypertension in sickle cell disease
Ataga, K. I., Sood, N., Gent, G. D., Kelly, E. A., Henderson, A. G., Jones, S., Strayhorn, D., Lail, A. E., Lieff, S., & Orringer, E. P. (2004) · The American Journal of Medicine · doi:10.1016/j.amjmed.2004.03.034 · Cited by 152 · see citing works
Heme: Modulator of Plasma Systems in Hemolytic Diseases
Roumenina, L. T., Rayes, J., Lacroix‐Desmazes, S., & Dimitrov, J. D. (2016) · Trends in Molecular Medicine · doi:10.1016/j.molmed.2016.01.004 · Cited by 149 · see citing works
Cárdenes, N., Corey, C. G., Geary, L., Jain, S., Zharikov, S. I., Barge, S., Novelli, E. M., & Shiva, S. (2014) · Blood · doi:10.1182/blood-2013-09-529420 · Cited by 148 · see citing works
Red alert: labile heme is an alarmin
Soares, M. P., & Bozza, M. T. (2016) · Current Opinion in Immunology · doi:10.1016/j.coi.2015.11.006 · Cited by 148 · see citing works
Sachdev, V., Kato, G. J., Gibbs, J. S. R., Barst, R. J., Machado, R. F., Nouraie, M., Hassell, K. L., Little, J. A., Schraufnagel, D. E., Krishnamurti, L., et al. (2011) · Circulation · doi:10.1161/circulationaha.111.032920 · Cited by 147 · see citing works
Self-citationEmbury, S., Clark, M. R., Monroy, G., & Mohandas, N. (1984) · Journal of Clinical Investigation · doi:10.1172/jci111181 · Cited by 145 · see citing works
Cardiomyopathy With Restrictive Physiology in Sickle Cell Disease
Niss, O., Quinn, C. T., Lane, A., Daily, J., Khoury, P. R., Bakeer, N., Kimball, T. R., Towbin, J. A., Malik, P., & Taylor, M. D. (2016) · JACC: Cardiovascular Imaging · doi:10.1016/j.jcmg.2015.05.013 · Cited by 142 · see citing works
Sabaa, N., Franceschi, L. D., Bonnin, P., Castier, Y., Malpeli, G., Debbabi, H., Galaup, A., Maïer-Redelsperger, M., Vandermeersch, S., Scarpa, A., et al. (2008) · Journal of Clinical Investigation · doi:10.1172/jci33308 · Cited by 140 · see citing works
Renal Function in Infants with Sickle Cell Anemia: Baseline Data from the BABY HUG Trial
Ware, R. E., Rees, R. C., Sarnaik, S. A., Iyer, R. V., Alvarez, O. A., Casella, J. F., Shulkin, B. L., Shalaby-Rana, E. I., Strife, C. F., Miller, J. H., et al. (2009) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2009.06.060 · Cited by 138 · see citing works
Reactive Species in Sickle Cell Disease
ASLAN, M., THORNLEY‐BROWN, D., & FREEMAN, B. A. (2000) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.2000.tb06201.x · Cited by 136 · see citing works
Hemodynamic Predictors of Mortality in Adults with Sickle Cell Disease
Mehari, A., Alam, S., Tian, X., Cuttica, M. J., Barnett, C. F., Miles, G., Xu, D., Seamon, C., Adams-Graves, P., Castro, O. L., et al. (2013) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201207-1222oc · Cited by 135 · see citing works
Self-citationYeo, T. W., Lampah, D. A., Tjitra, E., Gitawati, R., Kenangalem, E., Piera, K., Granger, D. L., Lopansri, B. K., Weinberg, J. B., Price, R. N., et al. (2009) · The Journal of Infectious Diseases · doi:10.1086/644641 · Cited by 132 · see citing works
Gladwin, M. T., Barst, R. J., Gibbs, J. S. R., Hildesheim, M., Sachdev, V., Nouraie, M., Hassell, K. L., Little, J. A., Schraufnagel, D. E., Krishnamurti, L., et al. (2014) · PLoS ONE · doi:10.1371/journal.pone.0099489 · Cited by 132 · see citing works
Self-citationThe sickle erythrocyte in double jeopardy: autoxidation and iron decompartmentalization.
Hebbel, R. P. (1990) · PubMed · Cited by 132 · see citing works
The Painful Crisis of Homozygous Sickle Cell Disease
Baum, K. F. (1987) · Archives of Internal Medicine · doi:10.1001/archinte.1987.00370070045007 · Cited by 124 · see citing works
Derchi, G., Galanello, R., Bina, P., Cappellini, M. D., Piga, A. G., Lai, M.-E., Quarta, A., Casu, G., Perrotta, S., Pinto, V. M., et al. (2013) · Circulation · doi:10.1161/circulationaha.113.002124 · Cited by 122 · see citing works
Association between hemolysis and albuminuria in adults with sickle cell anemia
Day, T. G., Drašar, E., Fulford, T. J., Sharpe, C. C., & Thein, S. L. (2011) · Haematologica · doi:10.3324/haematol.2011.050336 · Cited by 122 · see citing works
Impaired nitric oxide-mediated vasodilation in transgenic sickle mouse
Kaul, D. K., Liu, X.-D., Fabry, M. E., & Nagel, R. L. (2000) · American Journal of Physiology-Heart and Circulatory Physiology · doi:10.1152/ajpheart.2000.278.6.h1799 · Cited by 122 · see citing works
Inhaled Nitric Oxide Enables Artificial Blood Transfusion Without Hypertension
Yu, B., Raher, M. J., Volpato, G. P., Bloch, K. D., Ichinose, F., & Zapol, W. M. (2008) · Circulation · doi:10.1161/circulationaha.107.729137 · Cited by 120 · see citing works
Saraf, S. L., Zhang, X., Kanias, T., Lash, J. P., Molokie, R. E., Oza, B., Lai, C., Rowe, J. H., Gowhari, M., Hassan, J., et al. (2014) · British Journal of Haematology · doi:10.1111/bjh.12690 · Cited by 120 · see citing works
Self-citationThe effect of fetal hemoglobin on the survival characteristics of sickle cells
Franco, R. S., Yasin, Z., Palascak, M. B., Ciraolo, P., Joiner, C. H., & Rucknagel, D. L. (2006) · Blood · doi:10.1182/blood-2005-09-008318 · Cited by 119 · see citing works
Repka, T. L., & Hebbel, R. (1991) · Blood · doi:10.1182/blood.v78.10.2753.2753 · Cited by 118 · see citing works
Erythroid DAMPs drive inflammation in SCD
Gladwin, M. T., & Ofori-Acquah, S. F. (2014) · Blood · doi:10.1182/blood-2014-03-563874 · Cited by 113 · see citing works
Self-citationBallas, S. K., & Marcolina, M. J. (2005) · Transfusion · doi:10.1111/j.1537-2995.2006.00679.x · Cited by 113 · see citing works
Fabry, M. E., & Nagel, R. L. (1982) · PubMed · Cited by 111 · see citing works
Baldwin, C. T., Nolan, V. G., Wyszynski, D. F., Ma, Q., Paola Sebastiani, B., Embury, S. H., Bisbee, A. B., Farrell, J. J., Farrer, L. A., & Steinberg, M. H. (2005) · Blood · doi:10.1182/blood-2005-02-0548 · Cited by 110 · see citing works
Self-citationEffect of fetal hemoglobin on microvascular regulation in sickle transgenic-knockout mice
Kaul, D. K., Liu, X.-D., Chang, H.-Y., Nagel, R. L., & Fabry, M. E. (2004) · Journal of Clinical Investigation · doi:10.1172/jci21633 · Cited by 108 · see citing works
Haptoglobin Preserves Vascular Nitric Oxide Signaling during Hemolysis
Schaer, C. A., Deuel, J., Schildknecht, D., Mahmoudi, L., García‐Rubio, I., Owczarek, C. M., Schauer, S., Kissner, R., Banerjee, U., Palmer, A. F., et al. (2015) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201510-2058oc · Cited by 108 · see citing works
Darbari, D. S., Onyekwere, O. C., Nouraie, M. S., Minniti, C. P., Luchtman‐Jones, L., RANA, S. R., Sable, C., Ensing, G. J., Dham, N., Campbell, A., et al. (2011) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2011.07.018 · Cited by 105 · see citing works
Self-citationHsu, L. L.-Y., Miller, S. T., Wright, E. C., Kutlar, A., McKie, V. C., Wang, W. C., Pegelow, C. H., Driscoll, C. E., Hurlet, A., Woods, G. M., et al. (2003) · Journal of Pediatric Hematology/Oncology · doi:10.1097/00043426-200308000-00007 · Cited by 104 · see citing works
Prevelance and clinical correlates of glomerulopathy in children with sickle cell disease
Wigfall, D. R., Ware, R. E., Burchinal, M., Kinney, T. R., & Foreman, J. W. (2000) · The Journal of Pediatrics · doi:10.1016/s0022-3476(00)48828-1 · Cited by 101 · see citing works
Johnson, M. C., Kirkham, F. J., Redline, S. S., ROSEN, C. L., Yan, Y., Roberts, I. A. G., Gruenwald, J., Marek, J., & DeBaun, M. R. (2010) · Blood · doi:10.1182/blood-2009-06-227447 · Cited by 100 · see citing works
Iron, Inflammation, and Early Death in Adults With Sickle Cell Disease
van Beers, E. J., Yang, Y., Raghavachari, N., Tian, X., Allen, D. T., Nichols, J. S., Mendelsohn, L. G., Nekhai, S. A., Gordeuk, V. R., Taylor, J. G., et al. (2014) · Circulation Research · doi:10.1161/circresaha.116.304577 · Cited by 100 · see citing works
Self-citationPulmonary Hypertension in Children and Adolescents with Sickle Cell Disease
Onyekwere, O. C., Campbell, A., Teshome, M., Onyeagoro, S., Sylvan, C., Akintilo, A., Hutchinson, S. K., Ensing, G. J., Gaskin, P. R. A., Kato, G. J., et al. (2007) · Pediatric Cardiology · doi:10.1007/s00246-007-9018-x · Cited by 99 · see citing works
Self-citationThe metabolism of hemoglobin and bile pigment in hemolytic disease
Crosby, W. H. (1955) · The American Journal of Medicine · doi:10.1016/0002-9343(55)90208-4 · Cited by 98 · see citing works
Alpha-thalassemia is related to prolonged survival in sickle cell anemia
Mears, J., Lachman, H., Labie, D., & Nagel, R. L. (1983) · Blood · doi:10.1182/blood.v62.2.286.bloodjournal622286 · Cited by 93 · see citing works
Zorca, S. M., Freeman, L. A., Hildesheim, M. E., Allen, D. T., Remaley, A. T., Taylor, J. G., & Kato, G. J. (2010) · British Journal of Haematology · doi:10.1111/j.1365-2141.2010.08109.x · Cited by 93 · see citing works
Self-citationIntracellular polymerization of sickle hemoglobin. Effects of cell heterogeneity.
Noguchi, C. T., Torchia, D. A., & Schechter, A. N. (1983) · Journal of Clinical Investigation · doi:10.1172/jci111055 · Cited by 92 · see citing works
Quinn, C. T., Lee, N. J., Shull, E. P., Ahmad, N., Rogers, Z. R., & Buchanan, G. R. (2007) · Blood · doi:10.1182/blood-2007-07-100719 · Cited by 90 · see citing works
Guasch, A., Zayas, C. F., Eckman, J. R., Muralidharan, K., Zhang, W., & Elsas, L. J. (1999) · Journal of the American Society of Nephrology · doi:10.1681/asn.v1051014 · Cited by 90 · see citing works
O-Thalassemia Reduces the Hemolytic Rate in Homozygous Sickle-Cell Disease
Ceulaer, K. D., Higgs, D. R., Weatherall, D. J., Hayes, R., Serjeant, B. E., & Serjeant, G. R. (1983) · New England Journal of Medicine · doi:10.1056/nejm198307213090320 · Cited by 89 · see citing works
MRI abnormalities of the brain in one‐year‐old children with sickle cell anemia
Wang, W. C., Pavlakis, S. G., Helton, K. J., McKinstry, R. C., Casella, J. F., Adams, R. J., & Rees, R. C. (2008) · Pediatric Blood & Cancer · doi:10.1002/pbc.21612 · Cited by 86 · see citing works
Prevalence and clinical correlates of microalbuminuria in children with sickle cell disease
Becton, L. J., Kalpatthi, R. V., Rackoff, E., Disco, D., Orak, J. K., Jackson, S. M., & Shatat, I. F. (2010) · Pediatric Nephrology · doi:10.1007/s00467-010-1536-8 · Cited by 85 · see citing works
High levels of placenta growth factor in sickle cell disease promote pulmonary hypertension
Sundaram, N., Tailor, A., Mendelsohn, L. G., Wansapura, J. P., Wang, X., Higashimoto, T., Pauciulo, M. W., Gottliebson, W. M., Kalra, V. K., Nichols, W. C., et al. (2010) · Blood · doi:10.1182/blood-2009-09-244830 · Cited by 85 · see citing works
Self-citationExcess of heme induces tissue factor-dependent activation of coagulation in mice
Sparkenbaugh, E., Chantrathammachart, P., Wang, S., Jonas, W., Kirchhofer, D., Gailani, D., Gruber, A., Kasthuri, R. S., Key, N. S., Mackman, N., et al. (2015) · Haematologica · doi:10.3324/haematol.2014.114728 · Cited by 85 · see citing works
Gordeuk, V. R., Minniti, C. P., Nouraie, M. S., Campbell, A., RANA, S. R., Luchtman‐Jones, L., Sable, C., Dham, N., Ensing, G. J., Prchal, J. T., et al. (2010) · Haematologica · doi:10.3324/haematol.2010.030767 · Cited by 84 · see citing works
Self-citationVasavda, N., Menzel, S., Kondaveeti, S., Maytham, E. G., Awogbade, M., Bannister, S., Cunningham, J., Eichholz, A., Daniel, Y., Okpala, I. E., et al. (2007) · British Journal of Haematology · doi:10.1111/j.1365-2141.2007.06643.x · Cited by 83 · see citing works
Bean, C. J., Boulet, S. L., Ellingsen, D., Pyle, M., Barron‐Casella, E. A., Casella, J. F., Payne, A. B., Driggers, J., Trau, H. A., Yang, G., et al. (2012) · Blood · doi:10.1182/blood-2011-06-361642 · Cited by 83 · see citing works
Serghides, L., Kim, H., Lu, Z., Kain, D. C., Miller, C., Francis, R. C. E., Liles, W. C., Zapol, W. M., & Kain, K. C. (2011) · PLoS ONE · doi:10.1371/journal.pone.0027714 · Cited by 82 · see citing works
Almeida, C. B., de Souza, L. E. B., Leonardo, F. C., Costa, F. T. M., WERNECK, C. C., Covas, D. T., Costa, F. F., & Conran, N. (2015) · Blood · doi:10.1182/blood-2014-12-616250 · Cited by 82 · see citing works
Yuditskaya, S., Tumblin, A. R., Hoehn, G., Wang, G., Drake, S. K., Xu, X., Ying, S., Amy, H., Remaley, A. T., Shen, R., et al. (2008) · Blood · doi:10.1182/blood-2008-03-142604 · Cited by 80 · see citing works
Self-citationPrevalence and clinical correlates of glomerulopathy in children with sickle cell disease
Wigfall, D. R., Ware, R. E., Burchinal, M., Kinney, T. R., & Foreman, J. W. (2000) · The Journal of Pediatrics · doi:10.1067/mpd.2000.105998 · Cited by 80 · see citing works
Sickle cell anemia mice develop a unique cardiomyopathy with restrictive physiology
Bakeer, N., James, J., Roy, S., Wansapura, J., Shanmukhappa, S. K., Lorenz, J. N., Osinska, H., Backer, K., Huby, A.-C., Shrestha, A., et al. (2016) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1600311113 · Cited by 79 · see citing works
Fetal haemoglobin and early manifestations of homozygous sickle cell disease.
Bailey, K. A., Morris, J., Thomas, P., & Serjeant, G. R. (1992) · Archives of Disease in Childhood · doi:10.1136/adc.67.4.517 · Cited by 78 · see citing works
Hhawker, H., Neilson, H., Hayes, R., & Serjeant, G. R. (1982) · British Journal of Haematology · doi:10.1111/j.1365-2141.1982.tb01887.x · Cited by 77 · see citing works
Autologous Transfusion of Stored Red Blood Cells Increases Pulmonary Artery Pressure
Berra, L. M., Pinciroli, R., Stowell, C. P., Wang, L., Yu, B., Fernandez, B. O., Feelisch, M., Mietto, C., Hod, E. A., Chipman, D., et al. (2014) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201405-0850oc · Cited by 72 · see citing works
Detterich, J. A., Kato, R. M., Rábai, M., Meiselman, H. J., Coates, T. D., & Wood, J. C. (2015) · Blood · doi:10.1182/blood-2014-12-614370 · Cited by 71 · see citing works
Hu, W., Jin, R. C., Zhang, J., You, T., Peng, Z., Ge, X., Bronson, R. T., Halperin, J. A., Loscalzo, J., & Qin, X. (2010) · Blood · doi:10.1182/blood-2010-01-267112 · Cited by 70 · see citing works
Effects of Aged Stored Autologous Red Blood Cells on Human Endothelial Function
Risbano, M. G., Kanias, T., Triulzi, D. J., Donadee, C., Barge, S., Badlam, J. B., Jain, S., Belanger, A., Kim‐Shapiro, D. B., & Gladwin, M. T. (2015) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201501-0145oc · Cited by 69 · see citing works
Self-citationCumming, V., King, L., Fraser, R. A., Serjeant, G. R., & Reid, M. E. (2008) · British Journal of Haematology · doi:10.1111/j.1365-2141.2008.07115.x · Cited by 69 · see citing works
Mukisi-Mukaza, M., Elbaz, A., Leborgne-Samuel, Y., Kélard, L., Turdu-Chicot, C. L., Christophe-Duchange, E., & Mérault, G. (2000) · Orthopedics · doi:10.3928/0147-7447-20000401-17 · Cited by 69 · see citing works
van Beers, E. J., Samsel, L., Mendelsohn, L. G., Saiyed, R., Fertrin, K. Y., Brantner, C. A., Daniels, M. P., Nichols, J., McCoy, J. P., & Kato, G. J. (2014) · American Journal of Hematology · doi:10.1002/ajh.23699 · Cited by 67 · see citing works
Self-citationHemoglobin and free radicals: implications for the development of a safe blood substitute
Alayash, A. I., & Cashon, R. E. (1995) · Molecular Medicine Today · doi:10.1016/s1357-4310(95)80089-1 · Cited by 66 · see citing works
Phase 1 Study of a Sulforaphane-Containing Broccoli Sprout Homogenate for Sickle Cell Disease
Doss, J. F., Jonassaint, J., Garrett, M. E., Ashley‐Koch, A. E., Telen, M. J., & Chi, J. (2016) · PLoS ONE · doi:10.1371/journal.pone.0152895 · Cited by 66 · see citing works
Haematological determinants of cardiac involvement in adults with sickle cell disease
Damy, T., Bodez, D., Habibi, A., Guellich, A., Rappeneau, S., Inamo, J., Guendouz, S., Gellen-Dautremer, J., Pissard, S., Loric, S., et al. (2016) · European Heart Journal · doi:10.1093/eurheartj/ehv555 · Cited by 65 · see citing works
Machado, R. F., Hildesheim, M. E., Mendelsohn, L. G., Remaley, A. T., Kato, G. J., & Gladwin, M. T. (2011) · British Journal of Haematology · doi:10.1111/j.1365-2141.2011.08777.x · Cited by 64 · see citing works
Self-citationLiem, R. I., Young, L. T., & Thompson, A. A. (2007) · Haematologica · doi:10.3324/haematol.11576 · Cited by 64 · see citing works
Gordeuk, V. R., Campbell, A., RANA, S. R., Nouraie, M. S., Niu, X., Minniti, C. P., Sable, C., Darbari, D. S., Dham, N., Onyekwere, O. C., et al. (2009) · Blood · doi:10.1182/blood-2009-04-218040 · Cited by 63 · see citing works
Self-citationVoskaridou, E., Tsetsos, G. A., Tsoutsias, A., Spyropoulou, E., Christoulas, D., & Terpos, E. (2007) · Haematologica · doi:10.3324/haematol.11136 · Cited by 63 · see citing works
Microparticles in sickle cell anaemia: promise and pitfalls
Hebbel, R. P., & Key, N. S. (2016) · British Journal of Haematology · doi:10.1111/bjh.14112 · Cited by 63 · see citing works
Mahadeo, K. M., Oyeku, S. O., Taragin, B. H., Rajpathak, S. N., Moody, K., Santizo, R. O., & Driscoll, M. C. (2011) · American Journal of Hematology · doi:10.1002/ajh.22103 · Cited by 62 · see citing works
Taylor, J. G., Ackah, D., Cobb, C., Orr, N., Percy, M. J., Sachdev, V., Machado, R. F., Castro, O. L., Kato, G. J., Chanock, S. J., et al. (2007) · American Journal of Hematology · doi:10.1002/ajh.21035 · Cited by 60 · see citing works
Self-citationVinchi, F., & Tolosano, E. (2013) · Oxidative Medicine and Cellular Longevity · doi:10.1155/2013/396527 · Cited by 59 · see citing works
Cellular Adhesion and the Endothelium
Telen, M. J. (2014) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2013.11.010 · Cited by 59 · see citing works
Cellular Adhesion and the Endothelium
Kutlar, A., & Embury, S. H. (2014) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2013.11.007 · Cited by 58 · see citing works
Dham, N., Ensing, G. J., Minniti, C. P., Campbell, A., Arteta, M., RANA, S. R., Darbari, D. S., Nouraie, M. S., Onyekwere, O. C., Lasota, M., et al. (2009) · The American Journal of Cardiology · doi:10.1016/j.amjcard.2009.04.034 · Cited by 58 · see citing works
Self-citationCox, S. E., Makani, J., Soka, D., L’Esperence, V., Kija, E., Domínguez-Salas, P., Newton, C. R. J. C., Birch, A. A., Prentice, A. M., & Kirkham, F. J. (2014) · British Journal of Haematology · doi:10.1111/bjh.12791 · Cited by 57 · see citing works
Gall stones in Jamaican children with homozygous sickle cell disease.
Webb, D. K. H., Darby, J. S., Dunn, D. T., Terry, S. I., & Serjeant, G. R. (1989) · Archives of Disease in Childhood · doi:10.1136/adc.64.5.693 · Cited by 57 · see citing works
Nébor, D., Broquère, C., Brudey, K., Mougenel, D., Tarer, V., Connes, P., Élion, J., & Romana, M. (2010) · Blood Cells Molecules and Diseases · doi:10.1016/j.bcmd.2010.06.003 · Cited by 57 · see citing works
Landburg, P. P., Teerlink, T., Biemond, B. J., Brandjes, D. P. M., Muskiet, F. A. J., Duits, A. J., & Schnog, J. B. (2010) · Blood Cells Molecules and Diseases · doi:10.1016/j.bcmd.2010.02.005 · Cited by 57 · see citing works
Kassa, T., Jana, S., Strader, M. B., Meng, F., Jia, Y., Wilson, M. T., & Alayash, A. I. (2015) · Journal of Biological Chemistry · doi:10.1074/jbc.m115.651257 · Cited by 57 · see citing works
Laboratory and echocardiography markers in sickle cell patients with leg ulcers
Minniti, C. P., Taylor, J. G., Hildesheim, M. E., Oneal, P. A., Wilson, J., Castro, O. L., Gordeuk, V. R., & Kato, G. J. (2011) · American Journal of Hematology · doi:10.1002/ajh.22065 · Cited by 56 · see citing works
Self-citationQuinn, C. T., Smith, E. P., Arbabi, S., Khera, P. K., Lindsell, C. J., Niss, O., Joiner, C. H., Franco, R. S., & Cohen, R. M. (2016) · American Journal of Hematology · doi:10.1002/ajh.24562 · Cited by 55 · see citing works
Vascular dysfunction in a murine model of severe hemolysis
Frei, A. C., Guo, Y. H., Jones, D. W., Pritchard, K. A., Fagan, K. A., Hogg, N., & Wandersee, N. J. (2008) · Blood · doi:10.1182/blood-2007-12-126714 · Cited by 54 · see citing works
Placenta growth factor in sickle cell disease: association with hemolysis and inflammation
Brittain, J. E., Hulkower, B., Jones, S., Strayhorn, D., Castro, L. D., Telen, M. J., Orringer, E. P., Hinderliter, A. L., & Ataga, K. I. (2009) · Blood · doi:10.1182/blood-2009-04-217950 · Cited by 54 · see citing works
Genetic determinants of haemolysis in sickle cell anaemia
Milton, J. N., Rooks, H., Drašar, E., McCabe, E. L., Baldwin, C. T., Melista, E., Gordeuk, V. R., Nouraie, M. S., Kato, G. R., Minniti, C. P., et al. (2013) · British Journal of Haematology · doi:10.1111/bjh.12245 · Cited by 51 · see citing works
Self-citationVercellotti, G. M., Zhang, P., Nguyen, J., Abdulla, F. A., Chen, C., Nguyen, P., Nowotny, C., Steer, C. J., Smith, A., & Belcher, J. D. (2016) · Molecular Medicine · doi:10.2119/molmed.2016.00063 · Cited by 51 · see citing works
Joly, P., Garnier, N., Kébaïli, K., Renoux, C., Dony, A., Cheikh, N., Renard, C., Ceraulo, A., Cuzzubbo, D., Pondarré, C., et al. (2015) · European Journal Of Haematology · doi:10.1111/ejh.12607 · Cited by 50 · see citing works
Increased Pulmonary Pressures and Myocardial Wall Stress in Children with Severe Malaria
Janka, J., Koita, O., Traoré, B., Traoré, J. M., Mzayek, F., Sachdev, V., Wang, X., Sanogo, K. M., Sangaré, L., Mendelsohn, L. G., et al. (2010) · The Journal of Infectious Diseases · doi:10.1086/655225 · Cited by 50 · see citing works
Self-citationThe Influence of Fetal Hemoglobin on the Clinical Expression of Sickle Cell Anemia a
Powars, D., Chan, L. S., & Schroeder, W. (1989) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.1989.tb24174.x · Cited by 49 · see citing works
Heimlich, J. B., Speed, J. S., O'Connor, P. M., Pollock, J. S., Townes, T. M., Meiler, S. E. L., Kutlar, A., & Pollock, D. M. (2015) · British Journal of Pharmacology · doi:10.1111/bph.13380 · Cited by 49 · see citing works
Belcher, J. D., Marker, P. H., Geiger, P. G., Girotti, A. W., Steinberg, M. H., Hebbel, R. P., & Vercellotti, G. M. (1999) · Journal of Laboratory and Clinical Medicine · doi:10.1016/s0022-2143(99)90191-9 · Cited by 47 · see citing works
Self-citationAlbuminuria correlates with hemolysis and NAG and KIM-1 in patients with sickle cell anemia
Hamideh, D., Raj, V. M. S., Harrington, T. J., Li, H., Margolles, E., Amole, F., Garcia-Buitrago, M. T., Ruiz, P., B, G. Z., & Alvarez, O. A. (2014) · Pediatric Nephrology · doi:10.1007/s00467-014-2821-8 · Cited by 46 · see citing works
Wang, X., Mendelsohn, L. G., Rogers, H. M., Leitman, S. F., Raghavachari, N., Yang, Y., Yau, Y. Y., Tallack, M. R., Perkins, A. C., Taylor, J. G., et al. (2014) · Blood · doi:10.1182/blood-2013-11-539718 · Cited by 45 · see citing works
Self-citationYu, B., Lei, C., Baron, D. M., Steinbicker, A. U., Bloch, K. D., & Zapol, W. M. (2012) · Transfusion · doi:10.1111/j.1537-2995.2011.03473.x · Cited by 44 · see citing works
Prevalence and predictors of microalbuminuria in Jamaican children with sickle cell disease
King, L., MooSang, M., Miller, M., & Reid, M. E. (2011) · Archives of Disease in Childhood · doi:10.1136/archdischild-2011-300628 · Cited by 43 · see citing works
Ataga, K. I., Derebail, V. K., Caughey, M. C., Elsherif, L., Shen, J. H., Jones, S., Maitra, P., Pollock, D. M., Cai, J., Archer, D. R., et al. (2016) · PLoS ONE · doi:10.1371/journal.pone.0162652 · Cited by 42 · see citing works
Caughey, M. C., Poole, C., Ataga, K. I., & Hinderliter, A. L. (2015) · British Journal of Haematology · doi:10.1111/bjh.13447 · Cited by 41 · see citing works
Insight onto the Pathophysiology and Clinical Complications of Thalassemia Intermedia
Cappellini, M. D., Musallam, K. M., & Taher, A. T. (2009) · Hemoglobin · doi:10.3109/03630260903351528 · Cited by 38 · see citing works
Red Cell Life Span in Sickle Cell Trait
Barbedo, M. M. R., & McCurdy, P. R. (2009) · Acta Haematologica · doi:10.1159/000208316 · Cited by 38 · see citing works
Lei, C., Yu, B., Shahid, M., Beloiartsev, A., Bloch, K. D., & Zapol, W. M. (2012) · Anesthesiology · doi:10.1097/aln.0b013e318272d866 · Cited by 36 · see citing works
Wali, Y. A. M. S., Al‐Lamki, Z., Hussein, S., Bererhi, H., Kumar, D., Wasifuddin, S. M., Zachariah, M., & Ghosh, K. (2002) · Pediatric Hematology and Oncology · doi:10.1080/08880010290097314 · Cited by 35 · see citing works
Mikobi, T. M., Lukusa-Tshilobo, P., Aloni, M. N., Lelo, G. M., Akilimali, P. Z., Muyembe-Tamfum, J. J., Race, V., Matthijs, G., & Mwamba, J. M. M. (2015) · PLoS ONE · doi:10.1371/journal.pone.0123568 · Cited by 34 · see citing works
Sickle cell retinopathy in Jamaican children: a search for prognostic factors.
Talbot, J. F., Bird, A. C., Rabb, L., Maude, G. H., & Serjeant, G. R. (1983) · British Journal of Ophthalmology · doi:10.1136/bjo.67.11.782 · Cited by 34 · see citing works
Nonhematopoietic Nrf2 dominantly impedes adult progression of sickle cell anemia in mice
Ghosh, S., Ihunnah, C. A., Hazra, R., Walker, A. L., Hansen, J. M., Archer, D. R., Owusu-Ansah, A. T., & Ofori-Acquah, S. F. (2016) · JCI Insight · doi:10.1172/jci.insight.81090 · Cited by 32 · see citing works
Belisário, A. R., Nogueira, F. L., Rodrigues, R. S., de Toledo, N. E., Cattabriga, A. L. M., Velloso‐Rodrigues, C., Duarte, F. O. C., Silva, C. M., & Viana, M. B. (2014) · Blood Cells Molecules and Diseases · doi:10.1016/j.bcmd.2014.08.001 · Cited by 30 · see citing works
Hypoxia/reoxygenation stress increases markers of vaso-occlusive crisis in sickle SAD mice
Aufradet, É., DeSouza, G., Bourgeaux, V., Bessaad, A., Campion, Y., Canet‐Soulas, E., Pialoux, V., Chirico, E. N., Chevrier, A., Godfrin, Y., et al. (2013) · Clinical Hemorheology and Microcirculation · doi:10.3233/ch-131735 · Cited by 27 · see citing works
Adenosine deaminase–adenosine pathway in hemolysis-associated pulmonary hypertension
Tofovic, S. P., Jackson, E. K., & Rafikova, O. (2009) · Medical Hypotheses · doi:10.1016/j.mehy.2008.12.043 · Cited by 27 · see citing works
Forrest, S. J., Kim, A., Carbonella, J., & Pashankar, F. (2011) · Pediatric Blood & Cancer · doi:10.1002/pbc.23338 · Cited by 26 · see citing works
Echocardiographic findings in patients with sickle cell disease
Naoman, S. G., Nouraie, M. S., Castro, O. L., Nwokolo, C., Fadojutimi-Akinsiku, M., Diaz, S. F., Willie-Carnegie, G., Andrews, N., & Gordeuk, V. R. (2009) · Annals of Hematology · doi:10.1007/s00277-009-0764-0 · Cited by 25 · see citing works
HETEROCELLULAR HEREDITARY PERSISTENCE OF FETAL HÆMOGLOBIN AND HOMOZYGOUS SICKLE-CELL DISEASE
Serjeant, G., Serjeant, B. E., & Mason, K. P. (1977) · The Lancet · doi:10.1016/s0140-6736(77)92976-2 · Cited by 24 · see citing works
Pulmonary Hypertension in Sickle Cell Disease
Gladwin, M. T. (2009) · CRC Press eBooks · doi:10.3109/9781420094763-19 · Cited by 22 · see citing works
Self-citationCaughey, M. C., Hinderliter, A. L., Jones, S., Shah, S. P., & Ataga, K. I. (2012) · The American Journal of Cardiology · doi:10.1016/j.amjcard.2011.11.067 · Cited by 21 · see citing works
Limitations of Hb F as a Phenotypic Modifier in Sickle Cell Disease: Study of Kuwaiti Arab Patients
Adekile, A. D. (2011) · Hemoglobin · doi:10.3109/03630269.2011.617230 · Cited by 19 · see citing works
van Beers, E. J., Nur, E., Schaefer‐Prokop, C. M., Gillavry, M. R. M., van Esser, J. W. J., Brandjes, D. P. M., Kappers‐Klunne, M. C., Duits, A. J., Muskiet, F. A. J., Schnog, J. B., et al. (2008) · American Journal of Hematology · doi:10.1002/ajh.21272 · Cited by 19 · see citing works
A novel hemoglobin-binding peptide reduces cell-free hemoglobin in murine hemolytic anemia
Hanson, M. S., Xu, H., Flewelen, T. C., Holzhauer, S., Retherford, D. M., Jones, D. W., Frei, A. C., Pritchard, K. A., Hillery, C. A., Hogg, N., et al. (2012) · American Journal of Physiology-Heart and Circulatory Physiology · doi:10.1152/ajpheart.00500.2012 · Cited by 16 · see citing works
Influence of alpha thalassaemia on the retinopathy of homozygous sickle cell disease.
Fox, P. D., Higgs, D. R., & Serjeant, G. R. (1993) · British Journal of Ophthalmology · doi:10.1136/bjo.77.2.89 · Cited by 16 · see citing works
Lorch, D. G., Spevack, D. M., & Little, J. A. (2011) · Acta Haematologica · doi:10.1159/000323464 · Cited by 14 · see citing works
TRV: A physiological biomarker in sickle cell disease
Kato, G. J. (2011) · Pediatric Blood & Cancer · doi:10.1002/pbc.23399 · Cited by 14 · see citing works
Self-citationAtaga, K. I., Hinderliter, A. L., Brittain, J. E., Jones, S., Xu, H., Cai, J., Kim, S., Pritchard, K. A., & Hillery, C. A. (2014) · Hematology · doi:10.1179/1607845414y.0000000171 · Cited by 14 · see citing works
Nguyen, K., Tian, X., Alam, S., Mehari, A., Leung, S., Seamon, C., Allen, D. T., Minniti, C. P., Sachdev, V., Arai, A. E., et al. (2015) · Haematologica · doi:10.3324/haematol.2015.125229 · Cited by 14 · see citing works
Self-citationUpadhya, B., Stacey, R. B., Ntim, W. O., Knovich, M. A., & Pu, M. (2014) · Acta Haematologica · doi:10.1159/000357393 · Cited by 13 · see citing works
Alpha thalassaemia and the macular vasculature in homozygous sickle cell disease.
Condon, P. I., Marsh, R. J., Maude, G. H., Higgs, D. R., Weatherall, D. J., & Serjeant, G. R. (1983) · British Journal of Ophthalmology · doi:10.1136/bjo.67.11.779 · Cited by 9 · see citing works
Diop, S. N., Diop, D., Seck, M., Guèye, Y. B., Faye, A., Dièye, T. N., Fall, A. O. T., Diallo, A., Thiam, D., & Diakhaté, L. (2010) · PubMed · Cited by 9 · see citing works
Hamad, Z., Aljedai, A., Halwani, R., & Alsultan, A. S. (2013) · Annals of Saudi Medicine · doi:10.5144/0256-4947.2013.372 · Cited by 8 · see citing works
Anti‐haemolytic effect of senicapoc and decrease in NT‐proBNP in adults with sickle cell anaemia
Minniti, C. P., Wilson, J., Mendelsohn, L. G., Rigdon, G. C., Stocker, J. W., Remaley, A. T., & Kato, G. J. (2011) · British Journal of Haematology · doi:10.1111/j.1365-2141.2011.08757.x · Cited by 6 · see citing works
Self-citationPulmonary hypertension in sickle cell disease
Machado, R. (2009) · PVRI Review · doi:10.4103/0974-6013.44873 · Cited by 5 · see citing works
Flagged first, then by citations
Research works printed but not linked: 5
- [6]6. Barabino GA, Platt MO, Kaul DK. Sickle cell biome- chanics. Annu Rev Biomed Eng. 2010;12:345-367.
- [56]56. Lee MT, Rosenzweig EB, Cairo MS. Pulmonary hypertension in sickle cell disease. Clin Adv Hematol Oncol. 2007;5(8):645-653.
- [73]73. Forrest S, Kim A, Carbonella J, Pashankar F. Pro- teinuria is associated with elevated tricuspid regur- gitant jet velocity in children with sickle cell dis- ease. Pediatr Blood Cancer. 2012;58(6):937-940.
- [156]156. Powars DR, Chan L, Schroeder WA. The influ- ence of fetal hemoglobin on the clinical expres- sion of sickle cell anemia. Ann N Y Acad Sci. 1989;565:262-278.
- [183]183. Fox PD, Higgs DR, Serjeant GR. Influence of α-thalassemia on the retinopathy of homo- zygous sickle-cell disease. Br J Ophthalmol. 1993;77(2):89-90.
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Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019) · Annual Review of Pathology Mechanisms of Disease · doi:10.1146/annurev-pathmechdis-012418-012838 · Cited by 813 · see citing works
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Page, G. P., Kanias, T., Guo, Y., Lanteri, M. C., Zhang, X., Mast, A. E., Cable, R. G., Spencer, B. R., Kiss, J. E., Fang, F., et al. (2021) · Journal of Clinical Investigation · doi:10.1172/jci146077 · Cited by 97 · see citing works
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Xu, J. Z., Conrey, A. K., Frey, I., Gwaabe, E., Menapace, L. A., Tumburu, L., Lundt, M., Lequang, T., Li, Q., Glass, K. E., et al. (2022) · Blood · doi:10.1182/blood.2022015403 · Cited by 76 · see citing works
Park, S., Mattè, A., Jung, Y., Ryu, J., Anand, W., Han, E.-Y., Liu, M., Carbone, C., Melisi, D., Nagasawa, T., et al. (2020) · Blood · doi:10.1182/blood.2019002227 · Cited by 69 · see citing works
Stefanova, D., Raychev, A., Deville, J., Humphries, R., Campeau, S., Ruchala, P., Nemeth, E., Ganz, T., & Bulut, Y. (2018) · Infection and Immunity · doi:10.1128/iai.00253-18 · Cited by 68 · see citing works
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Vats, R., Kaminski, T. W., Brzóska, T., Leech, J. A., Tütüncüoğlu, E., Katoch, O., Jonassaint, J., Tejero, J., Novelli, E. M., Pradhan‐Sundd, T., et al. (2022) · Blood · doi:10.1182/blood.2021014552 · Cited by 66 · see citing works
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Prothrombotic aspects of sickle cell disease
Sparkenbaugh, E., & Pawliński, R. (2017) · Journal of Thrombosis and Haemostasis · doi:10.1111/jth.13717 · Cited by 62 · see citing works
Meegan, J. E., Shaver, C. M., Putz, N. D., Jesse, J. J., Landstreet, S. R., Lee, H. N. R., Sidorova, T. N., McNeil, J. B., Wynn, J. L., Cheung‐Flynn, J., et al. (2020) · PLoS ONE · doi:10.1371/journal.pone.0228727 · Cited by 60 · see citing works
The Multiple Facets of Iron Recycling
Slusarczyk, P., & Mleczko‐Sanecka, K. (2021) · Genes · doi:10.3390/genes12091364 · Cited by 60 · see citing works
Li, W., Guo, R., Song, Y., & Jiang, Z. (2021) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2020.613885 · Cited by 62 · see citing works
Allali, S., Rignault‐Bricard, R., de Montalembert, M., Taylor, M., Bouceba, T., Hermine, O., & Maciel, T. T. (2022) · Blood · doi:10.1182/blood.2021014894 · Cited by 61 · see citing works
Vinchi, F., Sparla, R., Passos, S., Sharma, R., Vance, S. Z., Zreid, H., Juaidi, H., Manwani, D. G., Yazdanbakhsh, K., Nandi, V., et al. (2021) · British Journal of Haematology · doi:10.1111/bjh.17361 · Cited by 57 · see citing works
Hemolysis and immune regulation
Zhong, H., & Yazdanbakhsh, K. (2018) · Current Opinion in Hematology · doi:10.1097/moh.0000000000000423 · Cited by 59 · see citing works
Heme scavenging reduces pulmonary endoplasmic reticulum stress, fibrosis, and emphysema
Aggarwal, S., Ahmad, I., Lam, A., Carlisle, M. A., Li, C., Wells, J. M., Raju, S. V., Athar, M., Rowe, S. M., Dransfield, M. T., et al. (2018) · JCI Insight · doi:10.1172/jci.insight.120694 · Cited by 57 · see citing works
Ferroptosis regulates hemolysis in stored murine and human red blood cells
D’Alessandro, A., Keele, G. R., Hay, A. M., Nemkov, T. G., Earley, E. J., Stephenson, D., Vincent, M. J., Deng, X., Stone, M., Dzieciątkowska, M., et al. (2024) · Blood · doi:10.1182/blood.2024026109 · Cited by 57 · see citing works
Cardiovascular complications in patients with sickle cell disease
Gladwin, M. T. (2017) · Hematology · doi:10.1182/asheducation-2017.1.423 · Cited by 56 · see citing works
Self-citationYalamanoglu, A., Deuel, J., Hunt, R., Baek, J. H., Hassell, K. L., Redinius, K., Irwin, D. C., Schaer, D. J., & Buehler, P. W. (2018) · American Journal of Physiology-Lung Cellular and Molecular Physiology · doi:10.1152/ajplung.00269.2018 · Cited by 53 · see citing works
Liu, Y., Pal, M., Bao, W., Shi, P. A., Lobo, C. A., An, X., Manwani, D. G., Zhong, H., & Yazdanbakhsh, K. (2021) · Blood · doi:10.1182/blood.2021011629 · Cited by 53 · see citing works
Kucukal, E., Ilich, A., Key, N. S., Little, J. A., & Gurkan, U. A. (2018) · American Journal of Hematology · doi:10.1002/ajh.25159 · Cited by 52 · see citing works
Václavů, L., Meynart, B. N., Mutsaerts, H. J. M. M., Petersen, E. T., Majoie, C. B. L. M., VanBavel, E., Wood, J. C., Nederveen, A. J., & Biemond, B. J. (2018) · Haematologica · doi:10.3324/haematol.2018.206094 · Cited by 53 · see citing works
Current and novel therapies for the prevention of vaso-occlusive crisis in sickle cell disease
Osunkwo, I., Manwani, D. G., & Kanter, J. (2020) · Therapeutic Advances in Hematology · doi:10.1177/2040620720955000 · Cited by 52 · see citing works
HO-1hi patrolling monocytes protect against vaso-occlusion in sickle cell disease
Liu, Y., Jing, F., Yi, W., Mendelson, A., Shi, P., Walsh, R., Friedman, D. F., Minniti, C., Manwani, D., Chou, S. T., et al. (2018) · Blood · doi:10.1182/blood-2017-12-819870 · Cited by 51 · see citing works
Fetal hemoglobin in sickle cell anemia: The Arab‐Indian haplotype and new therapeutic agents
Habara, A. H., Mohammed, E. S. E., & Steinberg, M. H. (2017) · American Journal of Hematology · doi:10.1002/ajh.24872 · Cited by 51 · see citing works
Self-citationCardiovascular manifestations of sickle cell disease
Hammoudi, N., Lionnet, F., Redheuil, A. B., & Montalescot, G. (2019) · European Heart Journal · doi:10.1093/eurheartj/ehz217 · Cited by 52 · see citing works
Erythrocytes: Central Actors in Multiple Scenes of Atherosclerosis
Turpin, C., Catan, A., Meilhac, O., Bourdon, E., Canonne‐Hergaux, F., & Rondeau, P. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22115843 · Cited by 51 · see citing works
Liu, Y., Zhong, H., Bao, W., Mendelson, A., An, X., Shi, P. A., Chou, S. T., Manwani, D. G., & Yazdanbakhsh, K. (2019) · Blood · doi:10.1182/blood.2019000172 · Cited by 48 · see citing works
Alimohamadi, H., Smith, A. S., Nowak, R. B., Fowler, V. M., & Rangamani, P. (2020) · PLoS Computational Biology · doi:10.1371/journal.pcbi.1007890 · Cited by 48 · see citing works
Wonkam, A., Mnika, K., Bitoungui, V. J. N., Chemegni, B. C., Chimusa, E. R., Dandara, C., & Kengne, A. P. (2017) · British Journal of Haematology · doi:10.1111/bjh.15011 · Cited by 47 · see citing works
Merle, N. S., Boudhabhay, I., Léon, J., Frémeaux‐Bacchi, V., & Roumenina, L. T. (2019) · Transfusion Clinique et Biologique · doi:10.1016/j.tracli.2019.02.008 · Cited by 47 · see citing works
Yalçınkaya, A. S., Ünal, S., & Öztaş, Y. (2019) · Lipids in Health and Disease · doi:10.1186/s12944-019-1174-5 · Cited by 47 · see citing works
Immune mechanisms involved in sickle cell disease pathogenesis: current knowledge and perspectives
de Azevedo, J. T. C., & Malmegrim, K. C. R. (2020) · Immunology Letters · doi:10.1016/j.imlet.2020.04.012 · Cited by 46 · see citing works
Hazzazi, A., Ageeli, M. H., Alfaqih, A. M., Jaafari, A., Malhan, H. M., & Bakkar, M. (2020) · Journal of Applied Hematology · doi:10.4103/joah.joah_67_19 · Cited by 45 · see citing works
Hypercoagulable state in sickle cell disease
Faës, C., Sparkenbaugh, E., & Pawliński, R. (2018) · Clinical Hemorheology and Microcirculation · doi:10.3233/ch-189013 · Cited by 43 · see citing works
How I diagnose and treat venous thromboembolism in sickle cell disease
Shet, A. S., & Wun, T. (2018) · Blood · doi:10.1182/blood-2018-03-822593 · Cited by 45 · see citing works
Olatunya, O. S., Lanaro, C., Longhini, A. L. F., Franco‐Penteado, C. F., Fertrin, K. Y., Adekile, A. D., Saad, S. T. O., & Costa, F. F. (2019) · Annals of Hematology · doi:10.1007/s00277-019-03792-x · Cited by 43 · see citing works
Drug Therapies for the Management of Sickle Cell Disease
Rai, P., & Ataga, K. I. (2020) · F1000Research · doi:10.12688/f1000research.22433.1 · Cited by 43 · see citing works
Onalo, R., Cooper, P., Cilliers, A. M., Vorster, B. C., Nnebe‐Agumadu, U., Oluseyi, O. O., Onalo, V. D., Zubairu, Y., Ayodele‐Kehinde, A. U., Damilare, O. M., et al. (2020) · American Journal of Hematology · doi:10.1002/ajh.26028 · Cited by 43 · see citing works
Vinchi, F. (2021) · Antioxidants and Redox Signaling · doi:10.1089/ars.2020.8167 · Cited by 43 · see citing works
Veluswamy, S., Shah, P., Denton, C. C., Chalacheva, P., Khoo, M. C. K., & Coates, T. D. (2019) · Journal of Clinical Medicine · doi:10.3390/jcm8101690 · Cited by 41 · see citing works
Gentinetta, T., Belcher, J. D., Brügger‐Verdon, V., Adam, J., Ruthsatz, T., Bain, J., Schu, D. J., Ventrici, L., Edler, M., Lioe, H., et al. (2022) · Journal of Clinical Medicine · doi:10.3390/jcm11030630 · Cited by 40 · see citing works
Self-citationRevisiting anemia in sickle cell disease and finding the balance with therapeutic approaches
Xu, J. Z., & Thein, S. L. (2022) · Blood · doi:10.1182/blood.2021013873 · Cited by 39 · see citing works
Sil, R., & Chakraborti, A. S. (2025) · Frontiers in Chemistry · doi:10.3389/fchem.2025.1543455 · Cited by 42 · see citing works
Hare, G. M. T., Han, K., Leshchyshyn, Y., Mistry, N., Kei, T., Dai, S. Y., Tsui, A. K. Y., Pirani, R. A., Honavar, J., Patel, R. P., et al. (2018) · Canadian Journal of Anesthesia/Journal canadien d anesthésie · doi:10.1007/s12630-018-1140-0 · Cited by 39 · see citing works
Li, Y., Han, M., Wang, Y., Liu, Q., Zhao, W., Su, B., & Zhao, C. (2018) · Carbohydrate Polymers · doi:10.1016/j.carbpol.2018.08.118 · Cited by 39 · see citing works
Redinus, K., Baek, J. H., Yalamanoglu, A., Shin, H. K., Moldova, R., Harral, J. W., Swindle, D. C., Pak, D. I., Ferguson, S. K., Nuss, R., et al. (2019) · JCI Insight · doi:10.1172/jci.insight.127860 · Cited by 39 · see citing works
Farrell, A. T., Panepinto, J. A., Desai, A. A., Kassim, A. A., Lebensburger, J. D., Walters, M. C., Bauer, D. E., Blaylark, R. M., DiMichele, D., Gladwin, M. T., et al. (2019) · Blood Advances · doi:10.1182/bloodadvances.2019000883 · Cited by 40 · see citing works
Self-citationDeficient mitophagy pathways in sickle cell disease
Martino, S., Arlet, J., Odièvre, M., Jullien, V., Moras, M., Hattab, C., Lefèbvre, T., Gouya, L., Ostuni, M. A., Lefevre, S. D., et al. (2021) · British Journal of Haematology · doi:10.1111/bjh.17416 · Cited by 39 · see citing works
Hsp90 in Human Diseases: Molecular Mechanisms to Therapeutic Approaches
Sumi, M. P., & Ghosh, A. (2022) · Cells · doi:10.3390/cells11060976 · Cited by 39 · see citing works
Measuring Intracellular Viscosity in Conditions of Hypergravity
Woodcock, E. M., Girvan, P., Eckert, J., López‐Duarte, I., Kubánková, M., van Loon, J. J. W. A., Brooks, N. J., & Kuimova, M. K. (2019) · Biophysical Journal · doi:10.1016/j.bpj.2019.03.038 · Cited by 38 · see citing works
Bories, G. F. P., Yeudall, S., Serbulea, V., Fox, T. E., Isakson, B. E., & Leitinger, N. (2020) · Blood · doi:10.1182/blood.2020004964 · Cited by 38 · see citing works
Glaros, A. K., Razvi, R., Shah, N., & Zaidi, A. U. (2021) · Therapeutic Advances in Hematology · doi:10.1177/20406207211001136 · Cited by 40 · see citing works
Sickle cell disease as an accelerated aging syndrome
Idris, I. M., Botchwey, E. A., & Hyacinth, H. I. (2022) · Experimental Biology and Medicine · doi:10.1177/15353702211068522 · Cited by 37 · see citing works
Yan, J., Wang, Y., Song, X., Yan, X., Zhao, Y., Yu, L., & He, Z. (2022) · Small Methods · doi:10.1002/smtd.202200139 · Cited by 37 · see citing works
Lee, M. T., Kattan, M., Fennoy, I., Arpadi, S. M., Miller, R. L., Cremers, S., McMahon, D. J., Nieves, J. W., & Brittenham, G. M. (2018) · Blood Advances · doi:10.1182/bloodadvances.2017013979 · Cited by 36 · see citing works
Sickle Cell Disorders and Severe COVID-19 Outcomes: A Cohort Study
Clift, A. K., Saatci, D., Coupland, C. A. C., Dambha‐Miller, H., & Hippisley‐Cox, J. (2021) · Annals of Internal Medicine · doi:10.7326/m21-1375 · Cited by 36 · see citing works
Vallelian, F., Buzzi, R. M., Pfefferlé, M., Yalamanoglu, A., Dubach, I. L., Wassmer, A., Gentinetta, T., Hansen, K., Humar, R., Schulthess-Lutz, N., et al. (2022) · Cell Death and Differentiation · doi:10.1038/s41418-022-00932-1 · Cited by 33 · see citing works
The clinical relevance of heme detoxification by the macrophage heme oxygenase system
Yeudall, S., Upchurch, C. M., & Leitinger, N. (2024) · Frontiers in Immunology · doi:10.3389/fimmu.2024.1379967 · Cited by 36 · see citing works
New insights into the pathophysiology and development of novel therapies for sickle cell disease
Moerdler, S., & Manwani, D. G. (2018) · Hematology · doi:10.1182/asheducation-2018.1.493 · Cited by 35 · see citing works
- Authors
- Kato, G. J., Steinberg, M. H., & Gladwin, M. T.
- Affiliations
- University of Pittsburgh (US)
- Boston University (US)
- Corresponding author
- Gladwin, Mark T.
- Venue
- Journal of Clinical Investigation
- Published
- 2017-03-01
- Volume
- 127
- Issue
- 3
- Pages
- 750-760
- ISSN
- 0021-9738 (ISSN-L)
- 1558-8238
- PMC
- PMC5330745
- OpenAlex
- W2592339311
- Semantic Scholar
- 0c9beb4e3a557982862ac3ec381a89baab12ceae
- Cited by
- 748
- References
- 193
- Citations by year
- 2026: 90 · 2025: 81 · 2024: 76 · 2023: 65 · 2022: 116 · 2021: 81 · 2020: 87 · 2019: 76 · 2018: 53 · 2017: 21 · 2016: 1
- FWCI
- 9.15
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Journal of Clinical Investigation (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Patient-Centered Outcomes Research Institute
- University of Pittsburgh
- Hemophilia Center of Western Pennsylvania
- National Institutes of Health (HL098032)
- National Heart, Lung, and Blood Institute (R01HL087681, R01HL068970, P01HL103455, RC2HL101212, R01HL121386, U01HL107443, T32HL007501, R01HL125886, R01HL098032, T32HL110849)
- National Institute on Minority Health and Health Disparities (R01MD009162)
- Topics
- Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Hemoglobin structure and function
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Blood properties and coagulation
- Keywords
- intravascular hemolysis, sickle cell disease, free hemoglobin, nitric oxide, methemoglobin, heme, arginase-1, asymmetric dimethylarginine, adenine nucleotides, pulmonary hypertension, inflammation
- MeSH terms
- Anemia, Sickle Cell, Hemolysis, Humans, Methemoglobin, Nitric Oxide, Vascular Diseases, Vasomotor System
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