How I treat hemophagocytic lymphohistiocytosis

Jordan, Michael B.; Allen, Carl E.; Weitzman, Sheila; Filipovich, Alexandra H.; McClain, Kenneth L.

doi:10.1182/blood-2011-03-278127

Cited by 940 publications

(1,176 citation statements)

References 94 publications

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“…HLH is a heterogeneous group of diseases that are characterized by uncontrolled proliferation of activated macrophages and T cells with overproduction of proinflammatory cytokines [1,2]. Activated CD8 + T cells are frequently observed during the acute phase of HLH.…”

Section: Discussionmentioning

confidence: 99%

“…Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal disease that is characterized by marked systemic inflammation and unregulated activation of macrophages and T cells [1,2]. Patients with HLH may present with fever, cytopenia, hepatosplenomegaly, liver dysfunction, coagulation abnormalities, and hemophagocytosis [1,2].…”

Section: Introductionmentioning

confidence: 99%

“…Patients with HLH may present with fever, cytopenia, hepatosplenomegaly, liver dysfunction, coagulation abnormalities, and hemophagocytosis [1,2]. HLH is comprised of primary and secondary forms.…”

Section: Introductionmentioning

confidence: 99%

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Down-regulation of CD5 expression on activated CD8+ T cells in familial hemophagocytic lymphohistiocytosis with perforin gene mutations

et al. 2013

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Hemophagocytic lymphohistiocytosis (HLH) is characterized by uncontrolled activation of T cells and macrophages with overproduction of cytokines. Familial HLH type2 (FHL2) is the most common form of primary HLH and is caused by mutations in PRF1. We have recently described a significant increase in the subpopulation of CD8 + T cells with clonal expansion and CD5 down-regulation in Epstein-Barr virus associated-HLH, which represented a valuable tool for its diagnosis. However, this unusual phenotype of CD8 + T cells has not been investigated fully in patients with FHL2. We performed immunophenotypic analysis of peripheral blood and measured serum proinflammatory cytokines in 5 patients with FHL2. All patients showed significantly increased subpopulations of activated CD8 + T cells with down-regulation of CD5, which were negligible among normal controls. Analysis of T-cell receptor Vβ repertoire suggested the reactive and oligoclonal expansion of these cells. The proportion of the subset declined after successful treatment concomitant with reduction in the serum levels of cytokines in all patients except one who continued to have a high proportion of the subset and died. These findings suggest that down-regulation of CD5 on activated CD8 +

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Down-regulation of CD5 expression on activated CD8+ T cells in familial hemophagocytic lymphohistiocytosis with perforin gene mutations

et al. 2013

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“…What distinguishes HLH from other syndromes of immune activation, immunodeficiencies, and inflammatory states is the coexistence of immune dysregulation with unchecked inflammation. 115 Clinically patients will present with fever, hepatosplenomegaly, lymphadenopathy, jaundice (in more than 2/3 cases), and often maculopapular rash. Of central nervous system manifestations (seen in nearly half the cases), encephalopathy, meningismus, and seizures are the most commonly reported.…”

Section: Hemophagocytic Lymphohistiocytosis (Hlh)mentioning

confidence: 99%

Approach to Clinical Syndrome of Jaundice and Encephalopathy in Tropics

Anand

Garg

2015

Journal of Clinical and Experimental Hepatology

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“…Neurological manifestations are seen in nearly half of the cases: encephalopathy, meningismus, and seizures are the most common. 36,37 Laboratory abnormalities include cytopenias, hyperbilirubinemia, hypertriglyceridemia and hyperferritinemia. Serum fibrinogen levels are typically low.…”

Section: Correct Answers: a B C And Ementioning

confidence: 99%