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Secondary hemophagocytic syndrome in adults: a case series of 18 patients in a single institution and a review of literature

2010; Wiley; Volume: 29; Issue: 2 Linguagem: Inglês

10.1002/hon.960

1099-1069

Munira Shabbir, John T. Lucas, John Lazarchick, Keisuke Shirai,

Parvovirus B19 Infection Studies

Abstract Hemophagocytic lymphohistiocytosis (HLH) is rare in adults and is usually fatal without treatment. We present a consecutive series of 18 adults with HLH diagnosed at our institution between 2004 and 2009. All diagnoses were confirmed by pathology. The median age at diagnosis was 56 years (range: 18–73 years), with a male: female ratio of 2:1. Patients uniformly presented with fever. Fifty‐five per cent of the patients presented with evidence of hepatomegaly or splenomegaly. All of the patients had at least a bi‐ or trilineage cytopenia. Elevated liver enzymes, hyperferritinemia, hypertriglyceridemia and hyperfibrinogenemia were seen in 50, 100, 40 and 50% of patients, respectively. The presumed causes were as follows; haematological malignancies ( n = 4), post‐autologous stem cell transplant ( n = 2), infection ( n = 2), rheumatologic illness ( n = 2), sickle cell disease ( n = 1), post‐orthotopic liver transplant ( n = 1) and idiopathic ( n = 3). The median time from suspicion to diagnosis was 5 days (1–27 days). Corticosteroids and/or cyclosporine were the most frequently used treatment regimen. Other agents used were etoposide, IVIG, cyclophosphamide and chemotherapy. The mortality rate was 72%, with multi‐system organ failure being the most common cause of death. Median survival time from diagnosis was 35 days. Six patients are alive to date. In a univariate analysis, the presence of fever was the only factor that was statistically significant for predicting a poor prognosis (early mortality) ( p = 0.05). In conclusion, a high index of suspicion is the critical factor for early diagnosis. Early treatment with immunosuppressant is warranted, and a thorough diagnostic evaluation to identify the underlying cause should be undertaken. Copyright © 2010 John Wiley & Sons, Ltd.