Abstract:

Objective To investigate the clinical characteristics of adult patients with hemophagocytic syndrome (HPS). Methods The clinical and experimental data of 19 adult patients with HPS from Department of Hematology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, were collected retrospectively from January 2005 to January 2009.ResultsAll patients presented high fever, hepatosplenomegaly, hematostatic abnormality, and cytopenia at first visit. Ten cases (52.6%) were diagnosed as non-Hodgkin's lymphoma; 2 (10.5%), as infection; 1 (5.3%), as acute lymphoblastic leukemia; and 6 (31.6%) without known etiology. Fourteen cases were dead during follow-up and one was still in follow-up (6 months till now). Four cases lost follow-up. The median survival time was 3 months (range 2-6 months). Conclusion HPS is an uncommon fatal disease, and rarely occurred in adult population. The clinical presentation is complex, usually with multi-organ dysfunction, aggressive course, and poor prognosis. The pathogenesis and treatment of HPS should be further studied.

Key words: hemophagocytic syndrome, pathogenesis, treatment, prognosis