结核分枝杆菌感染相关噬血细胞性淋巴组织细胞增生症1例及国内15例临床分析

Mycobacterium Tuberculosis Infection-associated Hemophagocytic Lymphohistiocytosis:A Case Report and Review of 15 Cases Reported in China

目的总结与探讨结核分枝杆菌感染相关噬血细胞性淋巴组织细胞增生症的临床特征及诊治方法。方法总结皖南医学院弋矶山医院呼吸科确诊病例1例的临床特征及诊治经过、转归,同时检索万方数据库和中国期刊全文数据库发表的相关文献(检索日期2013年8月)。结果共检索病例15例,有较完整的病例资料9例,平均年龄(31.2±15.83)岁,男7例,女2例,多数患者无基础疾病史。结核菌感染组织:肺部6例、肝脏2例、脾脏1例、淋巴结2例、胸膜3例、腹膜2例、脑膜1例,两个及两个以上部位感染5例。主要临床表现为发热、肝脾肿大、淋巴结肿大。实验室检查以肝功能损害、血细胞三系减少及骨髓涂片见噬血现象多见。治疗及转归:接受免疫治疗5例,其中接受丙种球蛋白治疗3例、糖皮质激素5例,抗结核治疗14例。15例患者中治愈3例、好转6例、死亡6例。结论结核菌感染相关噬血细胞性淋巴组织细胞增生症缺乏特异性指标,早期诊断、早期使用抗结核药物和免疫抑制治疗是治疗有效的关键。

Objective To summarize the clinical manifestations, diagnosis and treatment of mycobacteriumtuberculosis infection-associated hemophagocytic lymphohistiocytosis. Methods One proven diagnosed case of mycobacterium tuberculosis infection-associated hemophagocytic lymphohistiocytosis was analyzed. Related publications of case reports and articles fromWanfang Data and ChinaHospital Knowledge Database were also reviewed. Results A total of 15 cases were reported, in which 9 cases with complete clinical data. There were 7 males and 2 females, with a mean age of 31. 2 ±15. 83 years. The infected tissues were as follows： lung（ 6 cases） , liver（ 2 cases） , spleen（ 1 case） , lymph node（ 2 cases） , pleural （ 3 cases） , peritoneum（ 2 cases） , meninges（ 1 case） , two or more sites infection（ 5 cases） . The most clinical manifestations were fever, hepatosplenomegaly and enlarged lymph glands. The common laboratory abnormalities were liver dysfunction, pancytopaenia and haemophagocytosis in bone marrow. Treatment and outcomes： immunotherapy （ 5 cases, 3 cases with intravenous immunoglobulin and 5 cases with steroid therapy） and anti-tuberculosis （ 14 cases） . 3 cases were cured, 6 cases improved and 6 died.Conclusions There were no specific indexes of mycobacterium tuberculosis infection-associated hemophagocytic lymphohistiocytosis. Early proven diagnosis and administration of anti-tuberculosis and immunotherapy is essentials of effective therapy.