Table 2.

HLH diagnostic criteria.

HLH diagnostic criteria
Clinical	Laboratory
Unremitting fever	Fall in ESR, but high CRP
Bruising, purpura and mucosal bleeding	Pancytopenia (early stages neutrophelia)
Hepatosplenomegaly	Hyperferritinemia (>10,000)
Jaundice	High liver enzymes and LDH
CNS involvement (seizures, disorientation)	High triglycerides
Multiple organ failure	Low albumin
Lymphadenopathy	Low fibrinogen and elevated D-dimer, prolonged PT and PTT.
	Bone marrow hemophagocytosis (negative in 15%)

CNS, Central nervous system; CRP, C-reactive protein; ESR, Erythrocyte sedimentation rate; LDH, Lactate dehydrogenase; PT, Prothrombin time; PTT, Partial thromboplastin time.