结外NK/T细胞淋巴瘤相关性噬血细胞综合征临床分析

Clinical Analysis of Extranodal NK / T-cell Lymphoma Associated Hemophagocytic Syndrome

目的探讨结外NK／T细胞淋巴瘤（ENTKL）合并噬血细胞综合征（HPS）（ENKTL—LAHS）的临床特征、诊断、治疗及预后。方法分析3例NK／T—LAHS患者的临床资料，并进行文献复习。结果3例ENK-TL—LAHS患者年龄均小于60岁。2例淋巴瘤初诊时具有多项不良预后因素。3例ENKTL—LAHS患者均处于淋巴瘤的治疗和进展阶段。临床表现为持续高热、2～3系血细胞进行性减低、脾肿大、肝功能异常、铁蛋白升高及高TG血症等。发生HPS后病情进展迅速，短期内合并多器官功能不全综合征或弥漫性血管内凝血而死亡。自合并HPS后生存期18—124d。结论ENKTL—LAHS多见于中青年，多发生在ENKTL的进展或复发阶段，其临床特点不典型，早期诊断困难，尚缺乏有效治疗手段，预后差。

Objective To explore the clinical features, diagnosis, treatment and prognosis of extranodal NK/T- cell lymphoma（ENKTL）associated hemophagocytie syndrome（HPS） （ENKTL-LAHS）. Methods The clinical data of 3 patients with ENKTL-LAHS were retrospectively analyzed, and the related literature was reviewed. Results All the 3 patients were under 60 years old. Two of them at newly diagnosis had more than two risk factors indicating poor prognosis. All the 3 patients developed HPS at the progression or recurrence of lymphoma, and had the following clinical features ： persistent high fever,2-3 hemocyte decrease, splenomegaly, abnormal liver function, increased ser- um ferritin and triglyceride. The disease progressed rapidly after onset of HPS,leading to death in a short period due to multiple organ dysfunction syndrome or disseminated intravaseular coagulation. The survival time of 3 patients after onset of HPS were 18-124 days. Conclusion ENKTL-LAHS appears most frequently at the progression or recur- rence of ENKTL, at the age less than 60 years old. Its clinical features are not typical, and the early diagnosis is difficult. The therapeutic response and prognosis of ENKTL-LAHS are extremely poor.