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.
- Fever of unknown origin (FUO)
- Hemophagocytic lymphohistiocytosis (HLH)
- Soluble CD25