关键词: 噬血细胞综合征, 病因, 治疗, 转归

Abstract: Objective To summarize the clinical features of patients with hemophagocytic syndrome (HPS). Methods The underlying diseases, clinical characteristics, laboratory findings, responses to therapy as well as their outcomes of 45 patients with HPS were analyzed retrospectively. Results The underlying diseases included virus infection(n=9), tuberculosis infection(n=2), rheumatic diseases(n=4), malignancies(n=22), unknown origin(n=8). HPS was clinically characterized by high fever(93.3%), hepatomegaly (77.8%), splenomegaly(80%), lymphadenopathy(55.6%), skin rash(24.4%), gastrointestinal hemorrhage(22.2%), renal (35.6%) and central nervous systern involvement(15.6%), five patients presented with disseminated intravascular coagulation(DIC)(11.1%). Laboratory data mainly manifested with cytopenia(100%), liver dysfunction(77.8%), hypofibrinogenemia(62.8%), hypertriglyceridemia (71.1%) , serum ferritin>500μg/L(87.5%), low NK-cell activity(92.9%) and hemophagocytosis in bone marrow(100%). Based on treating underlying diseases and use of corticosteroids and immunosuppressive agents in combination with intravenous immunoglobulins(IVIG) therapy, 14 patients recovered, 19 patients died in the hospital, and other 12 cases give up treatment because of exacerbation of the disease. Conclusion　There are various underlying diseases and clinical manifestations for HPS. HPS is always extremely dangerous situation with high mortality. Underlying diseases treatments, corticosteroids, immunodepressant and IVIG may improve the prognosis of HPS.

Key words: hemophagocytic syndrome, underlying diseases, treatment, prognosis