Hemophagocytic lymphohistiocytosis (HLH): a review of literature

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• 作者：Rohtesh S. Mehta (1)
  Roy E. Smith (1)
  
• 关键词：Hemophagocytic lymphohistiocytosis (HLH) ; Fever of unknown origin (FUO) ; Cytopenia ; Hyperferritinemia ; Soluble CD25 ; Hypofibrinogenemia
• 刊名：Medical Oncology
• 出版年：2013
• 出版时间：December 2013
• 年：2013
• 卷：30
• 期：4
• 全文大小：218 KB
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• 作者单位：Rohtesh S. Mehta (1)
  Roy E. Smith (1)
  
  1. Division of Hematology-Oncology, University of Pittsburgh Medical Center, UPMC Cancer Pavilion, Room 463, 5150 Centre Avenue, Pittsburgh, PA, 15232, USA
  
• ISSN：1559-131X

文摘

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease in children and an exceptionally rare occurrence in adults. It is categorized broadly into primary (familial) or the secondary types; the latter being associated most commonly with an underlying malignancy. HLH carries a high rate of mortality, and the treatment itself is associated with significant morbidity and risk of mortality. A high degree of suspicion for the diagnosis, early treatment and aggressive supportive care is critical for management. We present a comprehensive review of literature describing the clinical features, diagnosis, management and outcome of HLH.