Journal
MEDICAL ONCOLOGY
Volume 30, Issue 4, Pages -Publisher
HUMANA PRESS INC
DOI: 10.1007/s12032-013-0740-3
Keywords
Hemophagocytic lymphohistiocytosis (HLH); Fever of unknown origin (FUO); Cytopenia; Hyperferritinemia; Soluble CD25; Hypofibrinogenemia
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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.
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