Hemophagocytic Lymphohistiocytosis

Teaching Points

Definition: severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages
Presentation: fever, hepatosplenomegaly, lymphadenopathy, jaundice and a rash.
Diagnosis: Fulfillment of five out of the eight criteria below: fever (>100.4 °F, >38 °C), splenomegaly, cytopenias affecting at least two of three lineages, hypertriglyceridemia, ferritin ≥ 500 ng/ml, haemophagocytosis in the bone marrow, spleen or lymph nodes, low or absent natural killer cell activity, soluble CD25
Treatment: high dose corticosteroids, etoposide and cyclosporin

Didactic: Morning Report