纯红细胞再生障碍性贫血为主要表现的血管免疫母细胞T细胞淋巴瘤

Angioimmunoblastic T -cell lymphoma patients characterized with pure red cell aplastic anemia

目的探讨血管免疫母细胞T细胞淋巴瘤（AITL）合并纯红细胞再生障碍性贫血（PRCA）患者的临床特点。方法通过对1例以PRCA为主要表现的AITL病例回顾，分析其临床表现、血清学指标、病理表现和诊疗过程。结果AITL合并PRCA患者可具有广泛浅表淋巴结肿大、肝脾肿大，有发热、贫血、出血等临床表现。实验室指标异常包括：全血细胞减少、网织红细胞比例降低、骨髓Coombs试验阳性等。病理及免疫组化符合AITL，常规CHOP方案治疗反应率低，总体预后差。结论AITL合并PRCA后，临床表现多变，病情凶险，预后不良，需提高警惕。

Objective To investigate the clinical features of angioimmunoblastic T - cell lymphoma （AITL） patients characterized with pure red cell aplastic anemia （PRCA）. Methods A retrospective review was conducted for an AITL patient with PRCA to analyze its clinical characteristics, serological indexes, pathological manifestations and the process of diagnosis and treatment. Results The patient might present enlargement of superficial lymph nodes in wide areas and hepatosplenomegaly, in addition to clinical manifestations such as fever, anemia and bleeding. The patient＇s abnormal la- boratory indexes included pancytopenia, a low percentage of reticulocytes, and a positive Coombs test. The pathological and immunohistochemical results were consistent with those of AITL patients. The response to conventional CHOP regimen was low, with poor overall prognosis. Conclusion The AITL with PRCA is in dangerous condition, with various clinical manifestations and poor prognosis.