血源播散性肺结核继发噬血细胞综合征1例  

Hemophagocytic lymphohistiocytosis caused by hematogenous disseminated pulmonary tuberculosis:A case report

作　　者：李秋钰 梁瀛[1] 代妮妮 王玉湘 朱博韬 伍蕊[1] 朱红[1] 孙永昌[1] LI Qiu-yu;LIANG Ying;DAI Ni-ni;WANG Yu-xiang;ZHU Bo-tao;WU Rui;ZHU Hong;SUN Yong-chang(Department of Respiratory and Critical Care Medicine,2.Department of Pathology,Peking University Third Hospital,Beijing 100191,China)

机构地区：[1]北京大学第三医院呼吸与危重医学科,北京100191 [2]北京大学第三医院病理科,北京100191

出　　处：《北京大学学报（医学版）》2022年第6期1219-1223,共5页Journal of Peking University:Health Sciences

摘　　要：1病例资料患者男性,28岁,主因“发热2个月,鼻衄及双下肢出血性皮疹10天”入院。2个月前,患者无明显诱因出现发热,体温39℃,弛张热型,伴乏力,不伴咳嗽、咳痰、腹痛、腹泻、尿急、尿频、尿痛等不适。自行口服退热药(具体不详)后可短暂退热,但效果不佳,遂于当地医院就诊,予“头孢菌素”及“地塞米松”静脉输液(具体剂量不详)6 d,体温恢复正常,但停药后再次出现发热,体温波动于38.5~40℃,未再诊治。1个月余前就诊于外院,查血常规未见异常,胸片示“双肺纹理增多”,予莫西沙星口服2 d(具体剂量不详),患者体温波动于38.5~40℃。18 d前患者就诊于北京大学第三医院发热门诊,查血常规提示:白细胞(white blood cell,WBC)5.89×10^(9)/L,血红蛋白(hemoglobin,Hb)123.0 g/L和血小板计数(platelet count,PLT)103.0×10^(9)/L,中性粒细胞(neutrophile granulocyte,Neu)百分数80.8%;降钙素原(procalcitonin,PCT)1.09μg/L;抗核抗体谱:抗Jo-1抗体和抗SSB抗体呈弱阳性;胸部CT提示:“双肺多发小结节,细支气管炎?”(图1)。10 d前患者出现鼻衄、双下肢出血性皮疹,皮疹无瘙痒,伴双下肢轻度水肿,无皮肤破溃;患者仍持续高热,每日体温在38.5~40℃之间波动。患者再次就诊于北京大学第三医院,查血常规提示血红蛋白(81.0 g/L)和血小板计数(1.0×10^(9)/L)明显降低,为进一步诊治收入院。Hemophagocytic lymphohistiocytosis(HLH) was a life-threatening syndrome due to the uncontrolled immune activation of cytotoxic T lymphocytes, natural killer(NK) cells, and macrophages. HLH is characterized by primary and secondary causes, the early diagnosis and treatment of patients are closely related to the prognosis and clinical outcome of patients. The clinical presentation is variable but mostly includes prolonged fever, splenomegaly, coagulopathy, hypertriglyceridemia, and hemophagocytosis, none of them is specific and particular for HLH. Tuberculosis(TB) infection is one of the causes of HLH. HLH caused by TB is very rare clinically, but it has a high mortality. For patients with fever of unknown origin, HLH-related clinical manifestations sometimes present before the final diagnosis of TB, and HLH is associated with the most significant mortality rate. This article is mainly about a 28-year-old patient with HLH who suffered from severe TB infection. The patient attended a hospital with a history of 2 months of prolonged fever, 10 days booger and subcutaneous hemorrhage in lower limbs. Before this, he was in good health and denied any history of tuberculosis exposure. Combined with relevant laboratory test results(such as splenomegaly, hemoglobin, platelet count, and hypertriglyceridemia) and clinical manifestations(e.g. fever), the patient was diagnosed with hemophagocytic lymphohistiocytosis, but the etiology of HLH remained to be determined. To confirm the etiology, the patient was asked about the relevant medical history(intermittent low back pain) and was performed chest CT scan, bone marrow biopsy, and fundus photography. Finally, he was diagnosed with hemophagocytic lymphohistiocytosis caused by hematogenous disseminated pulmonary tuberculosis. In response to this, intravenous methylprednisolone and anti-tuberculosis treatment(isoniazid, pyrazinamide, moxifloxacin, and amikacin) were administered to the patient. After more than a month of treatment, the patient recovered from HLH caused by severe T

关 键 词：血源播散性肺结核 噬血细胞综合征 发热 肺部结节 

分 类 号：R521.1[医药卫生—内科学]