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Impaired natural killer activity in lymphohistiocytosis syndrome
Abstract
In six patients with well-documented lymphohistiocytosis syndrome, natural killer activity was found to be profoundly impaired and could not be increased by incubation in vitro with interferon. This abnormality was not found in parents of the affected children. A clear correlation with the activity of the disease was observed, although a delay of a few weeks (possibly reflecting the life span of NK cells in blood) was seen in the disappearance of NK activity after the onset of the disease and its reappearance after remission. No absolute correlation was observed between NK activity and percentage of leukocytes detected by the Leu-7 monoclonal antibody. Our findings indicate that testing NK activity is useful for the diagnosis of lymphohistiocytosis syndrome and can be used as an index of activity of the disease, among other major clinical and biologic signs of this syndrome. Reversal of the NK activity defect (rather than detection of Leu-7 positive cells) appears to be a good criterion of complete remission.
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“The cytolytic function of NK cells is diminished in some forms of cytokine storm, which can lead to prolonged antigenic stimulation and difficulty resolving inflammation.”
From Cytokine storm (2020) · cites this paper as [17]
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“This causes the defect in the NK and T-cell cytotoxicity that has been recognized for long [15,16],with three causativegene defects known today; PRF [9], UNC13D [40], and STX11 [41].”
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“A hallmark of HLH, in genetic as well as in acquired cases, is impaired or absent function of natural killer (NK) cells and cytotoxic T lymphocytes (CTL) measured as lysis of K652 cells in a standard 4h chromium release assay [5, 17, 88, 102, 112].”
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Zanza, C., Romenskaya, T., Manetti, A. C., Franceschi, F., Russa, R. L., Bertozzi, G., Maiese, A., Savioli, G., Volonnino, G., & Longhitano, Y. (2022) · Medicina · doi:10.3390/medicina58020144 · Cited by 413
“Natural killer (NK) cells are the main cells responsible for cytokine storm in primary HLH, in which a defect in their cytolytic function leads to prolonged antigenic stimulation and incapability of resolving inflammation [6,14,15].”
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“…a pronounced inflammatory response with hypercyt- okinaemia and hyperferritinaemia, in association with a deficient lymphocyte cytotoxic activity (Perez et al, 1984; Esumi et al, 1989; Komp et al, 1989; Henter et al, 1991c; Schneider et al, 2002; Janka & Schneider, 2004; Horne et al, 2005a).”
BackgroundSpectrum of Perforin Gene Mutations in Familial Hemophagocytic Lymphohistiocytosis
Ericson, K. G., Fadeel, B., Nilsson-Ardnor, S., Söderhäll, C., Samuelsson, A., Janka, G., Schneider, M., Gürgey, A., Yalman, N., Révész, T., et al. (2001) · The American Journal of Human Genetics · doi:10.1086/318796 · Cited by 249
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“From 1984 onwards, evidence of impaired cellular cytotoxicity in HLH has been repeatedly confirmed (Perez et al, 1984; AricoÁ et al, 1988; Kataoka et al, 1990).”
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Self-citationControl of primary osteosarcoma with chemotherapy
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“Dysregulated NK cells fail to trigger apoptosis of the expanded effector T cell clones and APCs; resulting in a smoldering inflammatory response [17,20].”
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“In particular, demonstration of frequent association with common pathogens, together with evidence of impaired natural killer cytotoxic activity, provided the rationale for considering HLH as a selective immune deficiency.”
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Chan, L., Karimi, N., Morovati, S., Alizadeh, K., Kakish, J. E., Vanderkamp, S., Fazel, F., Napoleoni, C., Alizadeh, K., Mehrani, Y., et al. (2021) · Viruses · doi:10.3390/v13112318 · Cited by 102
“Some of the many roles of innate leukocytes in the development of CSS include the abundant production of cytokines by activated macrophages [15], the diminished cytolytic effect of NK cells leading to longer antigenic stimulation of B and T cells [13], along with the secretion of cytokines and formation of neutrophil…”
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Chinnici, A., Beneforti, L., Pegoraro, F., Trambusti, I., Tondo, A., Favre, C., Coniglio, M. L., & Sieni, E. (2023) · Frontiers in Immunology · doi:10.3389/fimmu.2023.1210041 · Cited by 99
“The initial identi fi cation of a biological marker refers to the impairment of natural killer (NK) cell cytotoxic activity, which was observed in affected patients, compared with healthy relatives, in the 1980s (5 – 8).”
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Allen, M., Fusco, C. D., Legrand, F., Clementi, R., Conter, V., Danesino, C., Janka, G., & Aricò, M. (2001) · PubMed · Cited by 84
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Abnormality in the NK-cell population is prolonged in severe COVID-19 patients
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“In particular, demonstration of frequent association with common pathogens, together with evidence of impaired natural killer cytotoxic activity, provided the rationale for considering HLH as a selective immune deficiency.”
BackgroundFamilial Hemophagocytic Lymphohistiocytosis
Degar, B. A. (2015) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2015.06.008 · Cited by 55
“In the setting of active HLH, the functional activity of NK cells is usually impaired.(27) An inability to reconstitute NK functional activity in vitro using modified assay conditions, and the persistence of depressed NK cell function after treatment of HLH, are associated with FHL.”
BackgroundHemophagocytic lymphohistiocytosis in adults
Bhatt, N. S., Oshrine, B. R., & Talano, J. (2018) · Leukemia & lymphoma/Leukemia and lymphoma · doi:10.1080/10428194.2018.1482543 · Cited by 53
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“activity, provided the rationale for considering HLH as a selective immune deficiency.(11-13) Starting from the original report by Farquhar et al.”
BackgroundRapid diagnosis of FHL3 by flow cytometric detection of intraplatelet Munc13-4 protein
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Genetics and pathophysiology of haemophagocytic lymphohistiocytosis
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Treatment of Hemophagocytic Lymphohistiocytosis With Alemtuzumab in Systemic Lupus Erythematosus
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“Poorly functioning natural killer (NK) cells fail to induce apoptosis of activated cells resulting in a persistent inflammatory state characterized by increased interleukin 1 (IL-1), IL-6, and tumor necrosis factor > production.(2) Hemophagocytic lymphohistiocytosis is subdivided into primary and secondary forms.”
BackgroundAcute encephalopathy as a primary manifestation of haemophagocytic lymphohistiocytosis
Kieslich, M., Vecchi, M., Driever, P. H., Laverda, A. M., Schwabe, D., & Jacobi, G. (2001) · Developmental Medicine & Child Neurology · doi:10.1017/s0012162201001001 · Cited by 34
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Heterogeneity of Immune Markers in Hemophagocytic Lymphohistiocytosis
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Hemophagocytic Lymphohistiocytosis Complicating Influenza A Infection
Mou, S. S., Nakagawa, T. A., Riemer, E. C., McLean, T. W., Hines, M. H., & Shetty, A. K. (2006) · PEDIATRICS · doi:10.1542/peds.2005-1861 · Cited by 31
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Enterovirus‐associated haemophagocytic syndrome in a neonate
Barre, V., Marret, S., Mendel, I., Lesesve, J., & Fessard, C. (1998) · Acta Paediatrica · doi:10.1111/j.1651-2227.1998.tb01482.x · Cited by 30
“NK cell activity was noted to be severely impaired (3, 5, 6), but NK absolute cell number remained normal (5), or decreased (3) as in our case.”
BackgroundInfluentialNatural Killer Cells in Relation to Disease and Treatment
Pedersen, B. K. (1985) · Allergy · doi:10.1111/j.1398-9995.1985.tb00881.x · Cited by 29
Familial Hemophagocytic Lymphohistiocytosis: Diagnostic Problems and Differential Diagnosis
Janka, C. E. (1989) · Pediatric Hematology and Oncology · doi:10.3109/08880018909034290 · Cited by 28
Familial Hemophagocytic Lymphohistiocytosis: Too Little Cell Death Can Seriously Damage Your Health
Fadeel, B., Orrenius, S., & Henter, J. (2001) · Leukemia & lymphoma/Leukemia and lymphoma · doi:10.3109/10428190109097672 · Cited by 27
Langerhans cell histiocytosis and other disorders of monocyte-histiocyte lineage
Egeler, R. M., & Nesbit, M. E. (1995) · Critical Reviews in Oncology/Hematology · doi:10.1016/1040-8428(94)00117-c · Cited by 26
Jenkins, R. W., Clarke, C. J., Lucas, J. T., Shabbir, M., Wu, B. X., Simbari, F., Mueller, J., Hannun, Y. A., Lazarchick, J. J., & Shirai, K. (2013) · American Journal of Hematology · doi:10.1002/ajh.23535 · Cited by 24
“The pathophysiologic progression of HLH begins with a predisposing immunodysfunction, which leads to impaired attenuation of immune cell activation, leading to hypercytokinemia [7,8].”
BackgroundEnterovirus-associated haemophagocytic syndrome in a neonate
Marret, S. (1998) · Acta Paediatrica · doi:10.1080/08035259850157138 · Cited by 22
Treatment of familial erythrophagocytic lymphohistiocytosis with cyclosporine A
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Electrical stimulation of the brain for relief of intractable pain due to cancer
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“Defective T lymphocyte and natural killer (NK) cell cytotoxicity is consistently observed [3–5].”
STEM-CELL TRANSPLANTATION FOR INHERITED IMMUNODEFICIENCY DISORDERS
Horwitz, M. E. (2000) · Pediatric Clinics of North America · doi:10.1016/s0031-3955(05)70276-5 · Cited by 19
BURGIO, G. R., Aricò, M., Marconi, M., Lanfranchi, A., Caselli, D., & Ugazio, A. G. (1990) · British Journal of Haematology · doi:10.1111/j.1365-2141.1990.00086.x-i1 · Cited by 19
Stéphan, J. L. (1995) · European Journal of Pediatrics · doi:10.1007/bf02079059 · Cited by 19
The role of BMT in childhood histiocytoses
Caselli, D., Aricò, M., & Party, O. B. O. T. E. P. W. (2008) · Bone Marrow Transplantation · doi:10.1038/bmt.2008.46 · Cited by 18
“17 In particular, demonstration of frequent association with common pathogens and evidence of impaired natural killer cytotoxic activity provided the rationale for considering HLH as a selective immune deficiency”
BackgroundCancer family syndrome: Marker studies
Markowitz, J. F., Aiges, H. W., Cunningham–Rundles, S., Kahn, E., Teichberg, S., Fisher, S. E., & Daum, F. (1986) · Gastroenterology · doi:10.1016/0016-5085(86)90626-8 · Cited by 18
Bone Marrow Transplantation in Hemophagocytic Lymphohistiocytosis
Dürken, M., Finckenstein, F. G., & Janka, G. (2001) · Leukemia & lymphoma/Leukemia and lymphoma · doi:10.3109/10428190109057957 · Cited by 17
Wagner, R., Morgan, G., & Ströbel, S. (1995) · Clinical & Experimental Immunology · doi:10.1111/j.1365-2249.1995.tb05535.x · Cited by 16
Komp, D. M., Buckley, P. J., McNamara, J., & van Hoff, J. (1989) · Pediatric Hematology and Oncology · doi:10.3109/08880018909034295 · Cited by 16
Do COVID-19 Infections Result in a Different Form of Secondary Hemophagocytic Lymphohistiocytosis
Chu, R., van Eeden, C., Suresh, S., Sligl, W., Osman, M. S., & Tervaert, J. W. C. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22062967 · Cited by 15
Unusual immunophenotype displayed by histiocytes in haemophagocytic lymphohistiocystosis.
Herlin, T., Pallesen, G., Kristensen, T., & Clausen, N. (1987) · Journal of Clinical Pathology · doi:10.1136/jcp.40.12.1413 · Cited by 15
Peripheral neuropathy associated with erythrophagocytic lymphohistiocytosis.
Boutin, B., Routon, M. C., Rocchiccioli, F., Mayer, M., Leverger, G., Robain, O., Ponsot, G., & Arthuis, M. (1988) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.51.2.291 · Cited by 13
Familial haemophagocytic lymphohistiocytosis. A report of three cases with unusual lung involvement
Popper, H., Zenz, W., Mache, C. J., & Öhlinger, W. (1994) · Histopathology · doi:10.1111/j.1365-2559.1994.tb00005.x · Cited by 13
History of Hemophagocytic Lymphohistiocytosis
Janka, G. (2024) · Advances in experimental medicine and biology · doi:10.1007/978-3-031-59815-9_2 · Cited by 12
FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS: AN AUTOPSY STUDY
Kakkar, N., Vasishta, R. K., Ak, B., Marwaha, R. K., & Thapa, B. R. (2003) · Fetal and Pediatric Pathology · doi:10.1080/15227950307733 · Cited by 12
Mache, C. J., Slavc, I., Schmid, C., Höefler, G., Urban, C., Schwinger, W., Winter, E., Hulla, W., Zenz, W., & Holter, W. (1994) · Annals of Hematology · doi:10.1007/bf01698488 · Cited by 11
“probably due to a lack of biologic control [2, 29, 37].”
BackgroundVancomycin-induced DRESS with evidence of T-cell activation in a 22-month-old patient
Kitcharoensakkul, M., Ree, N., Bloomberg, G. R., Dehner, L. P., Heidingsfelder, J., White, A. J., & Cooper, M. A. (2012) · Annals of Allergy Asthma & Immunology · doi:10.1016/j.anai.2012.07.016 · Cited by 10
Genetic dissection of NK cell responses
Moussa, P., Marton, J., Vidal, S. M., & Fodil-Cornu, N. (2013) · Frontiers in Immunology · doi:10.3389/fimmu.2012.00425 · Cited by 10
“In this disease, cytotoxic T and NK cell activity was consistently low or absent (Perez et al., 1984; Arico et al., 1988; Eife et al., 1989; Egeler et al., 1996).”
BackgroundAdvances in the management of histiocytic disorders
Windebank, K. (2008) · Paediatrics and Child Health · doi:10.1016/j.paed.2007.12.004 · Cited by 9
Familial Hemophagocytic Lymphohistiocytosis: Clinical Features
Aricò, M., Caselli, D., & Burgio, G. R. (1989) · Pediatric Hematology and Oncology · doi:10.3109/08880018909034294 · Cited by 9
Shahlaee, A. H., & Arceci, R. J. (2006) · doi:10.1002/9780470987001.ch15 · Cited by 9
Aricò, M., & BURGIO, G. R. (1989) · Pediatric Hematology and Oncology · doi:10.3109/08880018909034298 · Cited by 7
New and Old Immunodeficiencies
Stiehm, E. (1993) · Pediatric Research · doi:10.1203/00006450-199304001-00002 · Cited by 25
Case Report: Home-based Management of Severe COVID-19 with Low-dose Tofacitinib
Sharma, A., & Ali, M. (2021) · American Journal of Tropical Medicine and Hygiene · doi:10.4269/ajtmh.21-0737 · Cited by 6
“In addition, the number and function of natural killer cells is reduced.(6) All three patients described here had hypoxemia and transaminitis suggestive of multiorgan dysfunction (Table 1).”
BackgroundNonneoplastic Histiocytic Proliferations of Lymph Nodes and Bone Marrow
Rezk, S. A., Sullivan, J. L., & Woda, B. A. (2011) · Elsevier eBooks · doi:10.1016/b978-0-7216-0040-6.00051-4 · Cited by 5
Degar, B. A., Fleming, M. D., & Rollins, B. J. (2009) · Elsevier eBooks · doi:10.1016/b978-1-4160-3431-5.00024-8 · Cited by 4
Natural products for treating cytokine storm-related diseases: Therapeutic effects and mechanisms.
Liu, M., Wang, Q., Xu, W., Wu, J., Xu, X., Yang, H., & Li, X. (2023) · Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie · doi:10.1016/j.biopha.2023.115555 · Cited by 3
CD8+ T Cell Biology in Cytokine Storm Syndromes
Sekine, T., Galgano, D., Casoni, G. P., Meeths, M., Cron, R. Q., & Bryceson, Y. T. (2024) · Advances in experimental medicine and biology · doi:10.1007/978-3-031-59815-9_10 · Cited by 3
Metadata
- Authors
- Perez, N., Virelizier, J.-L., Arenzana-Seisdedos, F., Fischer, A., & Griscelli, C.
- ORCID
- Fischer, Alain
- Venue
- The Journal of Pediatrics
- Publisher
- Elsevier BV
- Published
- 1984-04
- Volume
- 104
- Issue
- 4
- Pages
- 569-573
- ISSN
- 0022-3476 (ISSN-L)
- OpenAlex
- W2059566117
- Cited by
- 121
- References
- 26
- Citations by year
- 2026: 1 · 2025: 1 · 2024: 2 · 2023: 2 · 2022: 2 · 2021: 5 · 2020: 1 · 2019: 2 · 2018: 3 · 2017: 1 · 2016: 2 · 2015: 1 · 2014: 1 · 2013: 3 · 2012: 3
- FWCI
- 1.17
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Pediatrics (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
- Life Sciences › Immunology and Microbiology › Immunology › Immune Cell Function and Interaction
- Health Sciences › Medicine › Hematology › Autoimmune and Inflammatory Disorders Research
- Keywords
- natural killer cells, NK cell dysfunction, interferon, disease activity monitoring, remission, NK cell depletion
- MeSH terms
- Antibodies, Monoclonal, Cytotoxicity, Immunologic, Fluorescent Antibody Technique, Humans, Killer Cells, Natural, Leukocytes, Lymphatic Diseases
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