Clinical features and prognostic analysis of T-cell lymphoma with hemophagocytic syndrome

Objective To investigate the clinical characteristics and survival analysis of T-cell lymphoma with hemophagocytic syndrome (HPS).Methods The clinical features,laboratory tests and survival of 30 patients of T-cell lymphoma with HPS were analyzed comparing with 50 random patients of T-cell lymphoma without HPS (control group),and the data were obtained from department of hematology,Ruijin Hospital,Shanghai Jiaotong University School of Medicine from January 2006 to December 2011.Results 30 cases of T-cell lymphoma with HPS manifested for high fever [100.00 ％ (30/30)],splenomegaly [96.67 ％ (29/30)],more than two-line reduction in blood cells [93.33 ％ (28/30)],bone marrow with bloodthirsty phenomenon [86.67 ％ (26/30)],LDH increased [100.00 ％ (30/30)],hypertriglyceridemia [46.67 ％ (14/30)],low fibrin hyperlipidemia [60.00 ％ (18/30)],ferritin increased [93.33 ％ (28/30)],liver damaged [86.67 ％ (26/30)] from patient group,which were higher than those of control group,the differences were statistically significant (x2 =23.11,22.50,36.05,64.20,21.82,5.31,16.54,26.82,46.43,all P ＜ 0.05).But the incidence of superficial lymph nodes enlargement was not high,only 33.33 ％,which made the diagnosis of primary disease difficult.Combined chemotherapy of CHOP or etoposide plus dexamethasone could make the symptoms and laboratory indicators improved,but the median survival time was only 20 days.Compared with the control group,there were significant differences on the survival curves (x2 =35.05,P ＜ 0.0001).Conclusion In T-cell lymphoma with HPS,the clinical manifestations are complex,usually with multi-organ dysfunction,aggressive course,and poor prognosis.The pathogenesis and treatment of HPS should be further studied. Key words: Lymphohistiocytisis, hemophagocytic;  Lymphoma, T-cell;  Clinical features; Treatment;  Prognosis