Hemophagocytic Lymphohistiocytosis (HLH) Diagnostic Criteria - 2004 vs 2009 At least three of the following: - Fever - Splenomegaly - Cytopenia affecting at least two cell lines: - Hemoglobin <9 g/L - Platelets < 100 b/L - Absolute neutrophil count < 1000 b/L - Hepatitis At least one of the following: - Ferritin elevation - Elevated soluble CD25 (soluble IL2-receptor) - Hemophagocytosis seen on tissue biopsy - Low/absent NK-cell activity Other supportive features (not required) - Hypertriglyceridemia - Hypofibrinogenemia - Hyponatremia Dr. Josh Farkas @PulmCrit #HLH #Hemophagocytic #Lymphohistiocytosis #Diagnosis #Criteria #Hematology