Infection- And Malignancy-Associated Hemophagocytic Syndromes

Janka, Gritta; Imashuku, Shinsaku; Elinder, Göran; Schneider, Marion; Henter, Jan‐Inge

doi:10.1016/s0889-8588(05)70521-9

Cited by 392 publications

(283 citation statements)

References 40 publications

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“…These findings also support the distinctive role of cytokine storm in the pathophysiology of the infection, and TH1 response and lack of viral clearance are shown to be the key factors of the pathophysiology resulting the cytokine storm in primary and some sHPS including macrophage activating syndrome (MAS) in systemic Juvenile rheumatoid arthritis. Similar final mechanism may occur in the patients with CCHF [14,17,18]. As previously described in primary familial HLH, these unfortunate responses are under the genetic control or associated with perforin deficiency and impaired natural killer cell function [11,20].…”

Section: Discussionmentioning

confidence: 54%

“…On the other hand, it can be associated with variety of bacterial, viral, fungal, and parasitic infections, and malignancy and collagen vascular disorders, which is called secondary HPS or reactive HPS. Secondary hemophagocytosis may resolve with the treatment of the underlying disease but may also be as fatal as primary HPS [11,[17][18][19][20][21]. Also, in CCHF and other hemorrhagic virus infections the primary cause of demise is acquired HPS and recently it was named ''virus associated HPS'' (VAHPS), which was proposed to be a distinct clinical entity [19].…”

Section: Discussionmentioning

confidence: 99%

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Crimean‐Congo hemorrhagic fever: Five patients with hemophagocytic syndrome

Fışgın

Tanyel

et al. 2007

American J Hematol

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Three pediatric and two adult Turkish patients with Crimean Congo Hemorrhagic Fever (CCHF) induced hemophagocytic syndrome (HPS) were admitted to Ondokuz Mayis University Hospital, which is in the Middle Black Sea Region of Turkey. All of them had remarkable hemophagocytosis in the bone marrow with severe bleeding symptoms along with the other known clinical and laboratory findings of CCHF. We would like to present these patients and to discuss the pathophysiology and the effect of acquired HPS on the severity of the disease. Am. J. Hematol., 2008. © 2007 Wiley‐Liss, Inc.

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Section: Discussionmentioning

confidence: 54%

Section: Discussionmentioning

confidence: 99%

Crimean‐Congo hemorrhagic fever: Five patients with hemophagocytic syndrome

Fışgın

Tanyel

et al. 2007

American J Hematol

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“…The primary form of the disease occurs due to genetic mutations and found in young children [3]. Another form of the disease occurs secondary to conditions like infections, malignancies, autoimmune diseases or immunosuppression [4]. Most common infectious etiologies include EBV among virus and VL among parasites [5].…”

Section: Casementioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis Associated with Visceral Leishmaniasis: Varied Presentation

Ranjan

Kumar

Ganguly

et al. 2015

Indian J Hematol Blood Transfus

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Visceral leishmaniasis (VL) is endemic in many parts of India. Rarely, it may be complicated by hemophagocytic lymphohistiocytosis (HLH) that has varied presentation and course. We describe two cases of VL complicated by HLH that were markedly different in clinical presentation, course and management. First case presented with Fever of unknown origin whereas second case had fever with severe bleeding manifestations. VL was diagnosed by bone marrow aspiration and serum rk39 immunodiagnostic test respectively in these cases. HLH was diagnosed by HLH 2004 diagnostic criteria. VL was treated by intravenous amphotericin B in both cases. HLH was managed by treating primary disease in the first case whereas steroid was given for management in the second case. High index of suspicion is crucial for early diagnosis of HLH to reduce morbidity and mortality.

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“…2 Secondary or reactive HPS (R-HPS) is associated with infections, malignancies and autoimmune disorders. 3 We describe a case of R-HPS with life-threatening gastrointestinal (GI) tract bleeding after autologous peripheral stem cell transplantation (APSCT) for multiple myeloma (MM), which resolved after high-dose intravenous immunoglobulin (IVIG) therapy.…”

mentioning

confidence: 99%

“…1 The prognosis is poor, with an overall mortality rate of 15-60% despite prompt aggressive treatment. 1,3,4 The pathogenesis of HPS is not fully understood. One proposed mechanism is uncontrolled T-lymphocyte and macrophage activation leading to massive cytokine release and abnormal immune responses resulting in widespread hemophagocytosis.…”

mentioning

confidence: 99%