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外周T细胞淋巴瘤非特指型合并纯红细胞再生障碍1例报告并文献复习

Nonspecific peripheral T cell lymphoma combined with pure red cell aplasia:a case report and literature review
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摘要 外周T细胞淋巴瘤非特指型(PTCL-NOS)合并纯红细胞再生障碍(PRCA)国内报道较少。本文报道1例PTCL-NOS患者,以重度贫血为首发表现,伴有多部位淋巴结肿大。实验室检查证实红细胞减少、网织红细胞比例降低、骨髓红细胞增生明显减低,骨髓CD3^(+)CD8^(+)T淋巴细胞比例显著增高,淋巴结病理及免疫组化符合PTCL-NOS,TCRβ基因重排阳性,一线ECHOP方案化疗4个疗程后,患者肿大淋巴结显著缩小,全身CT评估部分缓解,血红蛋白升至正常,骨髓造血红系增生恢复正常,CD3^(+)CD8^(+)T淋巴细胞未检出,目前口服来那度胺维持治疗,预后尚好。 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 TCRβgene 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.
作者 张学亚 郑瑜瑜 范大铬 钟金发 吴诗馨 Zhang Xueya;Zheng Yuyu;Fan Dage;Zhong Jinfa;Wu Shixin(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;Department of Pathology,the Second Affiliated Hospital of Fujian Medical University,Quanzhou 362000,China)
出处 《中华血液学杂志》 北大核心 2025年第4期359-362,共4页 Chinese Journal of Hematology
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