中国癌症杂志 ›› 2021, Vol. 31 ›› Issue (2): 121-125.doi: 10.19401/j.cnki.1007-3639.2021.02.006

• 论著 • 上一篇    下一篇

外周T细胞淋巴瘤合并自身免疫性溶血性贫血的临床特点分析

何合胜 1 ,杨玉琼 1 ,刘银华 2 ,靳小可 1 ,徐又海 1 ,姚军萍 1 ,刘善浩 1 ,严家炜 1 ,黄东平 1   

  1. 1. 皖南医学院第一附属医院血液科,安徽 芜湖 241000 ;
    2. 皖南医学院第一附属医院病理科,安徽 芜湖 241000
  • 出版日期:2021-02-28 发布日期:2021-03-02
  • 通信作者: 黄东平 E-mail: yjshdp@163.com
  • 基金资助:
    安徽省重点研究与开发计划(201904a07020036)。

Characteristic analysis of peripheral T-cell lymphoma associated with autoimmune hemolytic anemia

HE Hesheng 1 , YANG Yuqiong 1 , LIU Yinhua 2 , JIN Xiaoke 1 , XU Youhai 1 , YAO Junping 1 , LIU Shanhao 1 , YAN Jiawei 1 , HUANG Dongping #br#   

  1. 1. Department of Hematology, the First Affiliated Hospital of Wannan Medical College, Wuhu 241000, Anhui Province, China; 2. Department of Pathology, the First Affiliated Hospital of Wannan Medical College, Wuhu 241000, Anhui Province, China
  • Published:2021-02-28 Online:2021-03-02
  • Contact: HUANG Dongping E-mail: yjshdp@163.com

摘要: 背景与目的:与自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)相关的淋巴瘤病理学类型多见于惰性B细胞淋巴瘤,而很少见于弥漫性大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)及外周T细胞淋巴瘤(peripheral T-cell lymphoma,PTCL)。探索PTCL合并AIHA的临床特点,以进一步提高对该病的认识。方法:回顾性分析2012年2月—2018年11月皖南医学院第一附属医院收治的7例PTCL合并自身免疫性溶血性贫血患者的临床资料,分析其临床及实验室特征、治疗及转归。结果:7例患者中男性5例,女性2例;中位发病年龄66(51~89)岁。血红蛋白(hemoglobin,Hb)中位数为62(32~91)g/L,网织红细胞比例中位数为4.4%(1.6%~16.0%),总胆红素中位数为39.9(6.3~51.5)μmoL/L,直接胆红素中位数为12.5(4.29~18.70)μmoL/L,LDH中位数为379(142~1 012)U/L。Coombs试验全部为阳性。7例患者中Ⅲ期6例,Ⅳ期1例;国际预后指数(International Prognostic Index,IPI)评分为2分者2例,3分3例,4分1例,5分1例。4例患者联合化疗及后续激素治疗,2例仅予以化疗,1例患者单用激素;至随访结束时为止,3例患者存活,3例患者死亡,1例失访,可随访的患者中位生存期为57(2~73)个月。结论:PTCL合并AIHA临床非常少见,治疗上应根据患者的发病特点、治疗效果采取个性化的治疗方案,预后主要取决于淋巴瘤的缓解程度。

关键词: 自身免疫性溶血性贫血, T细胞淋巴瘤, 糖皮质激素, 化疗

Abstract: Background and purpose: The pathological type of lymphoma associated with autoimmune hemolytic anemia (AIHA) is more common in indolent B lymphoma than in diffuse large B-cell lymphoma (DLBCL) or peripheral T-cell lymphoma (PTCL). This study aimed to explore the clinical features of PTCL with AIHA so as to improve the learning of this disease. Methods: Data of 7 patients with PTCL complicated by AIHA treated at the First Affiliated Hospital of Wannan Medical College from Feb. 2012 to Nov. 2018 were reviewed retrospectively. The clinical and laboratory features, treatment and follow-up results were analyzed. Results:Seven cases of PTCL patients complicated by AIHA were collected, with 5 male patients and 2 female patients, aged from 51 to 89, with a median age of 66 years. The median level of hemoglobin (Hb) was 62 (32-91) g/L, and the median ratio of reticulocytes was 4.4% (1.6%-16.0%). Of the 7 patients, 6 presented with stage Ⅲ and 1 with stage Ⅳ PTCL. The International Prognostic Index (IPI) was 2 for 2 patients, was 3 for 3 patients, was 4 for 1 patient, and was 5 for 1 patient. Four patients received prednisone during chemotherapy, 2 patients received chemotherapy only, and 1 patient received prednisone only. Among the 7 patients, 3 died. The median overall survival (OS) was 57 months. Conclusion: AIHA is a rather unusual presentation in patients with PTCL. Prognosis depends largely on the response status of PTCL. The treatment schedule is quite varied with no consensus on first-line therapy regimen. More efforts are still needed to treat this rare disease.

Key words: Autoimmune hemolytic anemia, T-cell lymphoma, Glucocorticoid, Chemotherapy