噬血细胞综合征合并消化道出血患者的临床分析

OBJECTIVE: To explore the clinical features of patients with hemophagocytic lymphohistiocytosis (HLH) complicated with gastrointestinal bleeding. METHODS: The clinical data of 52 patients with HLH diagnosed from January 2015 to February 2017 were analyzed retrospectively. RESULTS: ①Of the 52 patients[including 36 cases of EBV related (69.2%), 5 cases of lymphoma related (9.6%), 3 cases of primary HLH (5.8%), 3 cases of rheumatism related (5.8%), 2 cases of tuberculosis related (3.8%), 3 cases of unknown causes (3.8%)], 32 cases were male (61.5%) and 20 cases female (38.5%) with a median age of 26 (6–64) years old. ②The overall survival rates at 1, 3, 6 and 12 months were 74.7%, 53.8%, 32.9% and 23.3% respectively. ③Multivariate analysis (logistic regression) showed thrombocytopenia (P=0.036) and other sites of hemorrhage (P=0.030) were risk factors of poor prognosis, and allogeneic hematopoietic stem cell transplantation (allo-HSCT, P=0.026) was the influence of good prognostic factor. CONCLUSION: HLH combined with gastrointestinal bleeding was an entity of disease with poor prognosis. Thrombocytopenia and other sites of bleeding had a significant negative impact on patients, allo-HSCT produced a significant positive impact on patients.