OBJECTIVETo investigate the clinical features of hemophagocytic syndrome (HPS) and to improve its recognition, early diagnosis and to reduce misdiagnosis.

METHODSA retrospective study was carried out to analyze the underlying diseases, clinical characteristics, laboratory findings and outcomes in 41 patients with HPS.

RESULTSHPS was clinically characterized by prolonged fever (100%), hepatomegaly (97.6%), splenomegaly (95.1%), and other features including lymph adenopathy (65.9%), respiratory symptoms (53.7%), hydrops of multiple serous cavity (26.8%), jaundice (26.8%), central nervous system involvement (14.6%), alimentary tract hemorrhage (12.2%) and skin rash (12.2%). Laboratory data indicated that liver dysfunction was the most prominent feature (100%) mainly manifested with elevated liver enzymes and hypoalbuminemia, and the others were hemophagocytosis in bone marrow (92.7%), pancytopenia 70.7%), coagulation abnormalities (52.4%), DIC, hypertriglyceridemia and refractory hyponatremia. The underlying disease of infection (IAHS) was most common (63.4%), in which EBV-AHS was predominant, making up to 69.2%. Fourteen patients died, 11 of them with IAHS (nine were EBV-AHS) and the other 3 non-IAHS (one of them was malignant lymphoma). The case-fatality rate was increased with the elevated levels of LDH and AST, the correlation coefficient was 0.486 and 0.516 (P < 0.05), respectively. Logistic regression analysis showed that age < 3 years old, levels of LDH > 2000 U/L and AST level > 200 U/L were independent prognostic factors (P value was 0.031, 0.002 and 0.001, respectively).

CONCLUSIONThere are various underlying diseases and clinical manifestations for HPS. EBV-AHS is the extremely dangerous situation with high mortality. Age, levels of LDH and AST are the death-associated risk factors. Repeat bone marrow examinations are helpful for diagnosis in time.