噬血细胞综合征：1例淋巴瘤诊治的曲折经历  

Hemophagocytic lymphohistiocytosis： tortuous experience in the diagnosis and treatment of one lymphoma

作　　者：张天喜[1] 叶鹏[1] 唐文台[1] 赵开飞[1] 刘安平[1] 喻安永[1] Zhang Tianxi;Ye Peng;Tang Wentai;Zhao Kaifei;Liu Anping;Yu Anyong(Department of Emergency,Affiliated Hospital of Zunyi Medical College,Zunyi 563003,Guizhou,China)

机构地区：[1]遵义医学院附属医院急诊科,贵州遵义563003

出　　处：《中华危重病急救医学》2018年第8期807-809,共3页Chinese Critical Care Medicine

基　　金：国家自然科学基金（81560217）

摘　　要：噬血细胞综合征（HLH）病因复杂,常诊断困难,原因不明时常以血液系统恶性肿瘤多见,受浸润器官穿刺活检能帮助明确病因.由于穿刺技术及穿刺部位的不同可能影响结果,多角度穿刺可提高成功率,但相应会增加风险.遵义医学院附属医院收治1例病因不明HLH患者,在积极对症支持治疗的同时,分别予以查找病毒感染依据、自身免疫性疾病相关检测、血培养和骨髓穿刺涂片及脾脏穿刺活检等寻找病原学依据.在行脾穿刺活检后患者脾脏出血不能控制,紧急行脾脏切除止血抢救生命,但患者最终因低蛋白、肺水肿、呼吸衰竭抢救无效死亡.骨髓穿刺涂片及脾脏穿刺活检均未见肿瘤浸润依据,脾切除后脾脏病理切片加免疫组化显示弥漫性大B细胞淋巴瘤（DLBCL）,确定为恶性肿瘤相关性噬血细胞综合征（M-HLH）.由于肿瘤相关的肿大脾脏穿刺活检出血风险大,故应严格掌握穿刺指征及准备急救预案.通过对该例患者的临床特点及诊治经过进行分析,以期提高临床医师对HLH及淋巴瘤的认识. The etiology of hemophagocytic lymphohistiocytosis （HLH） is complicated and difficult to diagnose, unexplained HLH often with hematological malignancies. Invasive biopsy can help to find etiology, the results may be affected by the technique and the location of the puncture site. Multiangle puncture can improve the success rate, but the corresponding risk increases. A patient with HLH was admitted to Affiliated Hospital of Zunyi Medical College. The etiology was unknown. Active symptomatic support treatment was conducted, at the same time, finding the evidence of viral infection, autoimmune disease related detection, blood culture, bone marrow puncture smear and spleen biopsy were performed respectively to find the pathogen basis. Spleen hemorrhage was not being controlled after spleen biopsy in patients, and emergency splenectomy was adopted to stop bleeding for saving lives. Finally, the patients died of low protein, pulmonary edema and respiratory failure. The bone marrow puncture and spleen biopsy failed to provide the basis for tumor invasion, while the spleen pathological slices plus immunohistochemical indicate diffuse large B cell lymphoma （DLBCL） after splenectomy, which was identified as malignant tumor-associated hemophagocytic syndrome. Underscoring the high risk of bleeding after tumor-associated splenomegaly puncture and the importance of having emergency plans. Through analyzing the clinical characteristics, diagnosis and treatment of this patient, we hope to improve the clinicians＇ understanding of HLH and lymphoma.

关 键 词：噬血细胞综合征 淋巴瘤 诊断 脾脏穿刺活检 脾切除 病理检查 

分 类 号：R733.1[医药卫生—肿瘤] R55[医药卫生—临床医学]