Table 2 A pathophysiologic view of HLH... | American Society of Hematology

Table 2

A pathophysiologic view of HLH patterns

Category 1: predisposing immunodeficiency	Category 2: significant immune activation	Category 3: abnormal immunopathology
Low or absent NK-cell function*	Fever*	Cytopenias*
Genetic defect of cytotoxicity*	Splenomegaly*/hepatomegaly	Decreased fibrinogen or increased triglycerides*
Family history of HLH	Elevated ferritin* (> 3000 ng/mL)	Hemophagocytosis*
Prior episode(s) of HLH or unexplained cytopenias	Elevated sCD25*	Hepatitis
Markers of impaired cytotoxicity: decreased expression of perforin, SAP, XIAP, or mobilization of CD107a	Elevated sCD163⁹³	CNS involvement

Category 1: predisposing immunodeficiency	Category 2: significant immune activation	Category 3: abnormal immunopathology
Low or absent NK-cell function*	Fever*	Cytopenias*
Genetic defect of cytotoxicity*	Splenomegaly*/hepatomegaly	Decreased fibrinogen or increased triglycerides*
Family history of HLH	Elevated ferritin* (> 3000 ng/mL)	Hemophagocytosis*
Prior episode(s) of HLH or unexplained cytopenias	Elevated sCD25*	Hepatitis
Markers of impaired cytotoxicity: decreased expression of perforin, SAP, XIAP, or mobilization of CD107a	Elevated sCD163⁹³	CNS involvement

SAP indicates SLAM-associated protein; and XIAP, X-linked inhibitor of apoptosis protein.

The HLH-2004 diagnostic criteria.