组织细胞坏死性淋巴结炎合并嗜血细胞综合征一例报道并文献复习  

Histiocytic Necrotizing Lymphadenitis Complicated with Hemophagocytic Syndrome: a Case Report and Literature Review

作　　者：程小龙 蔡青[2] 赵东宝[2] 胡珍丽[3] 吉连梅[2] 韩一平[1,3] 

机构地区：[1]第二军医大学附属长海医院全科规培基地,上海市200433 [2]第二军医大学附属长海医院风湿免疫科,上海市200433 [3]第二军医大学附属长海医院呼吸内科,上海市200433

出　　处：《中国全科医学》2017年第35期4447-4453,共7页Chinese General Practice

摘　　要：组织细胞坏死性淋巴结炎病因及发病机制未明,以顽固性发热、区域性淋巴结肿大伴或不伴压痛和一过性白细胞计数减少为特征。该病临床表现、实验室及影像学检查均无特异性,且相对少见,临床极易误诊为淋巴瘤、系统性红斑狼疮等疾病,确诊主要根据组织病理检查及免疫组织化学检查。本文报道1例患者表现为持续高热、颈部淋巴结肿大伴压痛、消瘦患者,抗生素治疗无效,^(18)F-FDG正电子发射断层显像/计算机断层成像(PET-CT)检查示全身多发淋巴结肿大,最大标准摄取值(SUV_(max))高达20.4,高度怀疑恶性淋巴瘤。该患者经颈部淋巴结活检证实为组织细胞坏死性淋巴结炎,且骨髓细胞学检查提示嗜血细胞增多骨髓象,最终诊断为组织细胞坏死性淋巴结炎合并嗜血细胞综合征。The etiology and pathogenesis of histiocytic necrotizing lymphadenitis( HNL) is still unknown now. It is mainly manifested as refractory fever, regional lymphadenopathy with or without tenderness and transient leucopenia. As its clinical manifestations, laboratory and imaging findings have no specific characteristics, it is easily be misdiagnosed as lymphoma,systemic lupus erythematosus or other kinds of diseases. Histopathological examination and immunohistochemistry are the main methods to confirm the final diagnosis. Here we reported the diagnostic procedure of a case of HNL with hemophagocytic syndrome. The patient manifested as persistent high fever,neck lymph nodes swollen combined with tenderness,weight loss,and nonresponse to antibiotic treatment. Moreover,^(18) F-FDG PET-CT examination showed generalized lymphadenopathy,SUV_(max) up to 20. 4,so he was highly suspected with malignant lymphoma. However, the neck lymph node biopsy confirmed it was HNL,and bone marrow cytology prompted hemophagocytic cells increased. The above data made this case a final diagnosis as HNL complicated with hemophagocytic syndrome.

关 键 词：组织细胞坏死性淋巴结炎 嗜血细胞综合征 正电子发射断层显像术 病理学 鉴别诊断 

分 类 号：R551.2[医药卫生—血液循环系统疾病]