Mizobe Takamitsu, Tsukada Junichi, Higashi Takehiro, Iwashige Atsushi, Ota Takanori, Kawano Ichiro, Kubota Ayumu, Matsuura Ai, Morimoto Hiroaki, Ogawa Ryosuke, Toda Yoko, Tanaka Yoshiya
First Department of Internal Medicine, University of Occupational and Environmental Health.
Rinsho Ketsueki. 2005 Mar;46(3):211-6.
A 71-year-old woman was admitted in December 2002 because of lymphadenopathy, hepatosplenomegaly and pleural effusion. She had severe anemia with hemoglobin 5.9 g/dl and a reticulocyte count of 1% per hundred. Direct/indirect Coombs tests and anti-double stranded DNA antibody were positive, her serum CH50 level was reduced and an increase in serum LDH isoenzyme 3 was observed. Bone marrow aspiration showed an almost total absence of erythroblasts and no pathological cell proliferation. The diagnosis of angioimmunoblastic T-cell lymphoma (AILT) was made based on the lymph node histological findings. Proliferation of arborizing small vessels with hyperplastic endothelium and infiltration of atypical T-lymphocytes were observed. After combination chemotherapy (THP-COP), remission was achieved in both the pure red cell aplasia (PRCA) and AILT. Remission was also accompanied by normalization of the Coombs tests, suggesting that autoimmune mechanisms in AILT may contribute to the development of PRCA.
一名71岁女性于2002年12月因淋巴结病、肝脾肿大和胸腔积液入院。她患有严重贫血，血红蛋白为5.9 g/dl，网织红细胞计数为每百个1%。直接/间接抗人球蛋白试验和抗双链DNA抗体呈阳性，血清CH50水平降低，血清乳酸脱氢酶同工酶3升高。骨髓穿刺显示几乎完全没有成红细胞，也没有病理性细胞增殖。根据淋巴结组织学检查结果诊断为血管免疫母细胞性T细胞淋巴瘤（AILT）。观察到有内皮细胞增生的树枝状小血管增殖和非典型T淋巴细胞浸润。联合化疗（THP-COP）后，纯红细胞再生障碍性贫血（PRCA）和AILT均实现缓解。缓解还伴随着抗人球蛋白试验的正常化，这表明AILT中的自身免疫机制可能促成了PRCA的发生。
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