الملخص
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.
الملخص
Epstein-Barr virus (EBV)infection is highly prevalent in general population,serum EBV antibody can be detected in about 90% of the adults.Forty years of researches have demonstrated that EBV infection is closely related to a variety of hematological diseases,such as infectious mononucleosis, EBV-related lymphoproliferative disorders,lymphoma and hemophagocytic syndrome.This paper reviews EBV-associated hematological diseases and its pathogenic mechanism.
الملخص
<p><b>OBJECTIVE</b>To investigate the outcomes, survival status, and the prognostic factors of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) patients.</p><p><b>METHODS</b>A retrospective study was carried out to analyze the clinical data of 61 EBV-HLH cases, from January 2008 to July 2014. Prognostic factors were analyzed through COX model (single factor and multiple factors).</p><p><b>RESULTS</b>A total of 246 patients with HLH were diagnosed, among which 102 cases were with EBV infection (including 61 EBV-HLH, 36 lymphoma associated HLH, 5 primary HLH. Among the 61 cases, 40 were male and 21 were female, with a ration of 1.9:1. The median age was 28 years (range, 12-78). 1, 3, 6 and 12-month overall survival rates of 61 EBV-HLH were 65.6%, 47.5%, 32.4%, and 25.0%, respectively. The median follow-up time was 3 (0.5-28) months. 12 patients didn't use etoposide within 4 weeks after diagnosis, while HLH-94 protocol was used in 33 patients and HLH-2004 protocol was used in 16 patients. Response rates of theses three groups were 33.3%, 51.5%, and 43.8%, respectively (P=0.401). There was statistically difference between the group without etoposide and the HLH-94/ HLH-2004 group in the overall survival rate (P=0.033). Serum albumin level (P=0.033) and whether EBV could became negative (P=0.010) were independent predictors for EBV-HLH.</p><p><b>CONCLUSION</b>EBV-HLH patients have severe clinical feature and poor prognosis. Early application of immune chemotherapy based on etoposide can improve survival. Serum albumin level and whether EBV can become negative are independent prognostic factors for survival.</p>