A retrospective analysis of 56 children with hemophagocytic lymphohistiocytosis.

OBJECTIVE

The aim of this study was to investigate the etiological factors, clinical features, and prognostic factors in children with hemophagocytic lymphohistiocytosis (HLH).

METHODS

Fifty-six children with HLH in Wuhan Union Hospital, People's Republic of China, were retrospectively analyzed in recent years. We reviewed the medical records of 56 HLH children hospitalized from 2000 to 2013 to identify the possible prognostic factors.

RESULTS

In more than half of the cases (64.29%), the etiological factor was found to be infection. Clinical characteristics such as prolonged fever (100.00%), hepatosplenomegaly (95.24%), and pancytopenia (100.00%) were observed. Characteristic laboratory values presented with increased ferritin (64.29%), triglycerides (78.57%), transaminases (80.95%), bilirubin (67.54%), lactate dehydrogenase (95.23%), and decreased fibrinogen (61.90%), natrium (40.48%), and potassium (30.94%). Bone marrow aspiration showed hemophagocytosis in 48 cases (85.71%). Forty-two patients were treated according to HLH-2004 protocol. Out of the 42 patients, 19 cases acquired remission and 13 cases died. Ten cases lost follow-up. High lactate dehydrogenase (>2,000 U/L), high bilirubin (>2 mg/mL), and younger age (<2 years) at the time of diagnosis were adverse prognostic factors.

CONCLUSIONS

HLH is a life-threatening syndrome caused by complicated etiology. The level of lactate dehydrogenase and bilirubin accompanied with younger age were adverse factors.