The DOI matches its Crossref record; 26 of the 26 references it cites were found in a registry or an index.
Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by the activation of the mononuclear phagocytic system. The diagnosis of HLH in adults is challenging not only because the majority of the reported data are from pediatric patients, but also because HLH occurs in many disease entities. This study reports the clinical and laboratory findings and prognostic factors of adult HLH in a large cohort managed at a single medical center from 2003 to 2014. Seventy‐three patients met the HLH‐2004 diagnostic criteria. The median age was 51 years (range, 18–82 years); 41 (56.2%) were male. Patients manifested fever, cytopenias, and elevated ferritin in >85% of cases. Likely causes of HLH were as follows: 30 (41.1%) infections, 21 (28.8%) malignancies, 5 (6.8%) attributed to autoimmune disorders, 1 (1.4%) primary immunodeficiency, 2 (2.7%) post solid organ transplantation, and 13 (17.8%) idiopathic. The median overall survival was 7.67 months. Patients with malignancy‐associated HLH had a markedly worse survival compared with patients with non–malignancy‐associated HLH (median overall survival 1.13 vs. 46.53 months, respectively; P < 0.0001). In a multivariable analysis, malignancy (hazard ratio = 12.22; 95% CI: 2.53–59.02; P = 0.002) correlated with poor survival. Ferritin >50,000 µg/L correlated with 30‐day mortality. Survival after a diagnosis of HLH is dismal, especially among those with malignancy‐associated HLH. The development of a registry for adults with HLH would improve our understanding of this syndrome, validate diagnostic criteria, and help develop effective treatment strategies. Am. J. Hematol. 90:220–224, 2015. © 2014 Wiley Periodicals, Inc.
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HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis
Henter, J., Horne, A., Aricó, M., Egeler, R. M., Filipovich, A. H., Imashuku, S., Ladisch, S., McClain, K., Webb, D., Winiarski, J., et al. (2007) · Pediatric Blood & Cancer · doi:10.1002/pbc.21039 · Cited by 5,386
Adult haemophagocytic syndrome
Ramos-Casals, M., Brito-Zerón, P., López-Guillermo, A., Khamashta, M. A., & Bosch, X. (2014) · The Lancet · doi:10.1016/s0140-6736(13)61048-x · Cited by 1,442
Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis
Allen, C. E., Yu, X., Kozinetz, C. A., & McClain, K. L. (2008) · Pediatric Blood & Cancer · doi:10.1002/pbc.21423 · Cited by 634
Familial and Acquired Hemophagocytic Lymphohistiocytosis
Janka, G. E. (2012) · Annual Review of Medicine · doi:10.1146/annurev-med-041610-134208 · Cited by 549
Hemophagocytic lymphohistiocytosis: review of etiologies and management
George, M. (2014) · Journal of Blood Medicine · doi:10.2147/jbm.s46255 · Cited by 488
Incidence in Sweden and Clinical Features of Familial Hemophagocytic Lymphohistiocytosis
HENTER, J., ELINDER, G., SÖDER, O., & ÖST, Å. (1991) · Acta Paediatrica · doi:10.1111/j.1651-2227.1991.tb11878.x · Cited by 448
Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH
Zhang, K., Jordan, M. B., Marsh, R. A., Johnson, J. A., Kissell, D., Meller, J., Villanueva, J., Risma, K. A., Wei, Q., Klein, P. S., et al. (2011) · Blood · doi:10.1182/blood-2011-07-370148 · Cited by 428
Fukaya, S., Yasuda, S., Hashimoto, T., Oku, K., Kataoka, H., Horita, T., Atsumi, T., & Koike, T. (2008) · Rheumatology · doi:10.1093/rheumatology/ken342 · Cited by 337
Reactive Hemophagocytic Syndrome in Adults: A Retrospective Analysis of 162 Patients
Rivière, S., Galicier, L., Coppo, P., Marzac, C., Aumont, C., Lambotte, O., & Fardet, L. (2014) · The American Journal of Medicine · doi:10.1016/j.amjmed.2014.04.034 · Cited by 324
Prognostic Factors and Outcomes of Adults With Hemophagocytic Lymphohistiocytosis
Parikh, S. A., Kapoor, P., Letendre, L., Kumar, S., & Wolanskyj, A. P. (2014) · Mayo Clinic Proceedings · doi:10.1016/j.mayocp.2013.12.012 · Cited by 323
Cited by
Recommendations for the management of hemophagocytic lymphohistiocytosis in adults
La Rosée, P., Horne, A., Hines, M., von Bahr Greenwood, T., Machowicz, R., Berliner, N., Birndt, S., Gil-Herrera, J., Girschikofsky, M., Jordan, M. B., et al. (2019) · Blood · doi:10.1182/blood.2018894618 · Cited by 1,108
A consensus review on malignancy‐associated hemophagocytic lymphohistiocytosis in adults
Daver, N., McClain, K., Allen, C. E., Parikh, S. A., Otrock, Z., Rojas‐Hernandez, C., Blechacz, B., Wang, S., Minkov, M., Jordan, M. B., et al. (2017) · Cancer · doi:10.1002/cncr.30826 · Cited by 258
Self-citationWang, A., Pope, S. D., Weinstein, J. S., Yu, S., Zhang, C., Booth, C. J., & Medzhitov, R. (2019) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1820704116 · Cited by 67
Metadata
- Authors
- Otrock, Z. K., & Eby, C. S.
- Venue
- American Journal of Hematology
- Publisher
- Wiley
- Published
- 2015-01-16
- Volume
- 90
- Issue
- 3
- Pages
- 220-224
- ISSN
- 0361-8609 (ISSN-L)
- 1096-8652
- OpenAlex
- W1652620147
- Cited by
- 338
- References
- 26
- FWCI
- 14.08
- Type
- journal article
- Language
- en
- Open access
- closed
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