重症结核病引起噬血细胞性淋巴组织细胞增多症一例并文献复习

A case of hemophagocytic lymphohistocytosis syndrome caused by severe tuberculosis and literature review

目的分析1例重症结核病引起噬血细胞性淋巴组织细胞增多症（简称噬血细胞综合征）患者的临床特点，以利于早期诊断噬血细胞综合征，减少误诊。方法分析2011年7月收治的1例重症结核病引起噬血细胞综合征患者的临床资料及诊治经过，并结合文献报道对结核病合并噬血细胞综合征进行分析。结果患者男，16岁，临床表现为发热、咳嗽、腹胀和水肿。实验室检查发现全血细胞减少，白细胞为3．0×10^9／L，血红蛋白为98g/L，血小板为34×10^9／L；肝功能损害，丙氨酸转氨酶为51．5U／L，天冬氨酸转氨酶为211U／L，总胆红素为20μmol／L，直接胆红素为17．6μmol／L；凝血功能障碍，凝血酶和凝血酶原时间延长，纤维蛋白原为0．56g／L；铁蛋白升高（662μg/L），骨髓涂片有吞噬现象，痰抗酸杆菌检测阳性。采用乙胺丁醇、链霉素和对氨基水杨酸钠进行抗结核治疗，同时给予氢化泼尼松、人免疫球蛋白和胸腺肽仪。等治疗，2个月后患者的体温、凝血功能和肝功能恢复正常，痰菌转阴。结论结核病是噬血细胞综合征的病因之一，对噬血细胞综合征患者应进行结核病筛查，并尽早抗结核治疗以改善预后。

Objective To investigate the clinical features of a case of hemophagocytic lymphohistoeytosis syndrome （abbreviated as hemophagocytic syndrome, HPS） caused by severe tuberculosis and therefore to improve early diagnosis of this condition. Methods The clinical features and process of diagnosis and treatment of a case with HPS caused by severe tuberculosis in July 2011 were analyzed, and the reported literatures of the disease were reviewed. Results The patient was a 16-year-old male. The primary manifestations were fever, cough, abdominal distention and edema. Laboratory analysis indicated pancytopenia （WBC 3.0 x 10^9/L, Hb 98 g/L, PLT 34 x 109/L）, liver dysfunction （ALT 51.5 U/L, AST 211 U/L, TBIL 20μmol/L, DBIL 17.6 μmol/L）, coagulation abnormalities （ extention of TT and APTF, FIB 0. 56 g/L） , high level of ferritin （662μg/L） , and hemophagocytosis in bone marrow. Sputum smear was positive for tubercule bacillus. After 2 months of antituberculous therapy with ethambutol, streptomycin and sodium aminosalieylate, along with administration of prednisolone, human immunoglobulin, and thymic peptide c^1 , the patient＇ s body temperature, function of coagulation and liver abnormalities all returned to normal, and repeated sputum smears became negative. Conclusions Tuberculosis is a cause of reactive hemophagocytic syndrome. Patients with hemophagocytic symptom should be rigorously screened for tuberculosis, and antituberculous therapy should be initiated early to improve prognosis.