Clinical characteristics of lymphoma associated hemophagocytic syndrome
LI Yongqian1 CHEN Sushan1 CHEN Manna1 CAI Songhao1 YE Junbin1 YANG Qimei1 DU Zepeng2 HONG Shaojie1
1.Department of Hematology, Shantou Central Hospital, Guangdong Province, Shantou 515031, China;
2.Department of Pathology, Shantou Central Hospital, Guangdong Province, Shantou 515031, China
Abstract：Objective To investigate the clinical characteristics of lymphoma associated hemophagocytic syndrome. Methods The clinical data of 18 patients with lymphoma associated hemophagocytic syndrome admitted to Shantou Central Hospital from January 2012 to June 2017 was analyzed retrospectively. Results Among 18 patients, there were 13 cases of patients with T cell lymphoma, and the main clinical stages were moderate and advanced stages. There was no significant difference of pathological types of lymphoma in different gender (χ2 = 0.628, P ＞ 0.05). The clinical stage of lymphoma was positively correlated with the age of onset (r = 0.765, P ＜ 0.05). The older the patients were, the later the clinical stage of lymphoma was. The related gene detection of lymphoma associated hemophagocytic syndrome showed that there were 9 cases of gene mutation, among which, 6 cases had UNC13D mutations. According to hemophagocytic syndrome-94 regimen, all patients were given glucocorticoid and Etoposide to improve hemophagocytic symptoms and then gradually transferred to chemotherapy. 13 of 18 patients died. EB virus infection and severe thrombocytopenia were the major causes of death of lymphoma related hemophagocytic syndrome. Conclusion Lymphoma related hemophagocytic syndrome has a wide variety of clinical manifestations and high mortality. Most patients have EB virus infection. Early diagnosis and treatment is the key.
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2019, Vol. 16 
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