Zhang X Y, Zheng Y Y, Fan D G, Zhong J F, Wu S X
Department of Hematology, the Second Affiliated Hospital of Fujian Medical University, Quanzhou 362000, China.
Department of Laboratory, the Second Affiliated Hospital of Fujian Medical University, Quanzhou 362000, China.
Zhonghua Xue Ye Xue Za Zhi. 2025 Apr 14;46(4):359-362. doi: 10.3760/cma.j.cn121090-20240809-00297.
Nonspecific peripheral T-cell lymphoma (PTCL-NOS) combined with pure red cell aplasia (PRCA) is reported by only few in China. This article described a patient with PTCL-NOS who presented with severe anemia as the initial symptom, accompanied by multiple lymphadenopathy. Laboratory tests confirmed decreased red blood cell count and reticulocyte proportion, markedly reduced bone marrow red blood cell proliferation, and markedly increased proportion of bone marrow CD3(+)CD8(+) T lymphocytes. On lymph node pathology examination, immunohistochemistry was consistent with PTCL-NOS, with positive TCRβ gene rearrangement. First-line chemotherapy with four courses of ECHOP regimen led to significant reductions in lymph node size, partial remission on whole-body computed tomography evaluation, normalization of hemoglobin and bone marrow hematopoietic erythroid proliferation, and absence of CD3(+)CD8(+) T lymphocytes in bone marrow. Currently, the patient remains to have good prognosis with maintenance oral lenalidomide.
在中国，非特异性外周T细胞淋巴瘤（PTCL-NOS）合并纯红细胞再生障碍性贫血（PRCA）的报道较少。本文描述了一名以严重贫血为首发症状、伴有多处淋巴结肿大的PTCL-NOS患者。实验室检查证实红细胞计数和网织红细胞比例降低，骨髓红细胞增殖明显减少，骨髓CD3(+)CD8(+) T淋巴细胞比例明显增加。淋巴结病理检查显示，免疫组化结果符合PTCL-NOS，TCRβ基因重排呈阳性。采用ECHOP方案进行4个疗程的一线化疗后，淋巴结大小显著缩小，全身计算机断层扫描评估显示部分缓解，血红蛋白和骨髓造血红系增殖恢复正常，骨髓中CD3(+)CD8(+) T淋巴细胞消失。目前，该患者口服来那度胺维持治疗，预后良好。
