结外NK/T细胞淋巴瘤相关噬血细胞综合征1例

目的 探讨结外NK/T细胞淋巴瘤(extranodal natural killer/T-cell lymphoma,ENKTL)相关噬血细胞综合征临床特点及有效的治疗策略。方法 回顾性分析安徽医科大学第二附属医院血液科收治1例ENKTL相关噬血细胞综合征临床资料及治疗措施。结果 患者，女性，17岁，以噬血细胞综合征起病，经皮肤活检诊断ENKTL,患者同时合并EB病毒感染。予PD1单抗联合P-GemOx方案治疗4疗程后患者体温、血常规、纤维蛋白、甘油三酯、铁蛋白及骨髓噬血现象等噬血细胞综合征指标完全正常，通过疗效评估ENKTL达到部分缓解，EB病毒DNA定量明显下降。随后行单倍体异基因造血干细胞移植，EBV转阴，疾病持续缓解状态。结论 有明确活动性EB病毒感染的ENKTL相关噬血细胞综合征早期诊断和适当的免疫化疗加单倍体异基因造血干细胞移植对改善预后至关重要。本例患者的成功诊治为临床提供一定借鉴意义。

A case with extranodal NK/T cell lymphoma-related hemophagocyte syndrome

Objective To explore the clinical characteristics and effective strategies of extranodal natural killer/T-cell lymphoma (ENKTL)-related hemophagocytic sydrome. Methods The clinical data and therapeutic strategies of a case of ENKTL-related hemophagocytic sydrome admitted to the Department of Hematology of the Second Affiliated Hospital of Anhui Medical University were retrospectively analyzed. Results A 17-year-old female patient with hemophagocytic sydrome onset was diagnosed with ENKTL by skin biopsy. The patient was also complicatedly infected with Epstein-Barr (EB) virus. The patient's body temperature, blood routine, fibrin, triglyceride, ferritin, bone marrow hemophagy and other hemophagocytic sydrome indicators were completely normal after 4 courses of treatment with programmed death 1(PD1) monoclonal antibody combined with pegaspargase, gemcitabine, and oxaliplatin (P-GEMOX) regimen, ENKTL had partial remission through efficacy evaluation, and EB virus(EBV) DNA quantitatively decreased significantly. The haploid allogeneic hematopoietic stem cell transplantation was performed subsequently, EBV was negative, and the disease continued to be in remission state. Conclusion Early diagnosis on ENKTL-related hemophagocytic sydrome with definite active EB viral infection and appropriate immunochemotherapy plus haploid allogeneic hematopoietic stem cell transplantation are critical for improved prognosis. The successful diagnosis and treatment of the patient provides certain reference for clinical practice.