伴有自身免疫性溶血性贫血及纯红系再生障碍性贫血血管免疫母细.docVIP

伴有自身免疫性溶血性贫血及纯红系再生障碍性贫血血管免疫母细 　 作者：王福旭,张学军,潘崚,乔淑凯,郭晓玲,董作仁 【摘要】 血管免疫母细胞性T细胞淋巴瘤（AITL）是一种外周T细胞淋巴瘤，常合并自身免疫现象，如免疫相关性血细胞减少症，是NHL中的少见类型。为了研究AITL的临床特征，病理表现和有效的治疗方法，对1例37岁男性患者进行了血常规检查、骨髓检测、单个核细胞的流式细胞术检测、Coombs试验、血清学检测、CT和免疫组织化学测定等。结果查明，患者有广泛淋巴结肿大、肝脾肿大，颈部淋巴结活检表明为血管免疫母细胞性T细胞淋巴瘤；患者重度贫血，网织红细胞降低，Coombs实验阳性，骨髓红系增生低下，提示并发温抗体型自身免疫性溶血性贫血（AIHA）和纯红系再生障碍性贫血（PRCA）；经过CHOPE方案化疗后，合并的AIHA和PRCA以及AITL浸润症状均消失。结论：成功地确诊了合并有AIHA和PRCA的AITL，淋巴结活检和骨髓检测意义大，CHOPE化疗方案对此种AITL有一定治疗效果。 【关键词】 血管免疫母细胞性T细胞淋巴瘤 Angioimmunoblastic Tcell Lymphoma with Autoimmune Hemolytic Anemia and Pure Red Cell Aplasia Abstract Angioimmunoblastic Tcell lymphoma (AILT) is a peripheral Tcell lymphoma often complicated autoimmune phenomena such as autoimmune cytopenia, and is a truly rare type of NHL. In order to investigate the clinical features, pathological manifestation of this lymphoma, and to explore its therapy protocol, a 37years old patient with AILT was investigated. The routine blood examination, bone marrow smear, lymphonodus biopsy, Coombs test, flow cytometry for bone marrow mononuclear cells, serological test, immunochemistry method etc were performed for this patient. The results showed that the systemic lymphadenectasis and hepatosplenomegaly were seen in patient, the cervical lymphonode biopsy revealed AITL. The hematoglobin level and number of reticulocytes were very low. Coombs test was positive. Simultaneously, the bone marrow aspirate revealed erythroid aplasia. The warm type autoimmune hemolytic anemia (AIHA) and pure red cell aplasia (PRCA) were coexisted. After one course of chemotherapy with CHOPE, infiltration sign of AITL patient with AIHA and PRCA disappeared. In conclusion, the AITL patient complicated with AIHA and PRCA was successfully diagnosed, the lymphonode biopsy and bone marrow smear showed more significant, the chemotherapy protocol of CHOPE can give some effect to cure such angioimmunoblastic T cell lymphoma. Key words angioimmunoblastic Tcell lymphoma；warm autoimmune hemolytic