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一名患有胸腺增生、低丙种球蛋白血症和成人T细胞白血病/淋巴瘤的患者出现纯红细胞再生障碍。

Pure Red Cell Aplasia in a Patient with Thymic Hyperplasia, Hypogammaglobulinemia and Adult T-cell Leukemia/Lymphoma.

作者信息

Kubota Yasushi, Sano Haruhiko, Takeda Yuji, Yamaguchi Kyosuke, Nakamura Hideaki, Kai Keita, Kidoguchi Keisuke, Kusaba Kana, Yokoo Masako, Ando Toshihiko, Sueoka Eisaburo, Kimura Shinya

机构信息

Division of Hematology, Respiratory Medicine and Oncology, Department of Internal Medicine, Faculty of Medicine, Saga University, Japan.

Department of Transfusion Medicine, Saga University Hospital, Japan.

出版信息

Intern Med. 2025 May 1;64(9):1393-1398. doi: 10.2169/internalmedicine.4443-24. Epub 2024 Sep 18.

DOI:10.2169/internalmedicine.4443-24
PMID:39293973
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12120213/
Abstract

Acquired pure red cell aplasia (PRCA), caused by thymic hyperplasia, is extremely rare. We herein report a previously healthy 41-year-old man who presented with severe anemia, lymphadenopathy, an upper mediastinal mass, and hypogammaglobulinemia. The patient was eventually diagnosed with PRCA and adult T-cell leukemia/lymphoma (ATLL). The mediastinal mass was pathologically diagnosed as thymic hyperplasia without clear ATLL invasion. Although his anemia improved rapidly after thymectomy, PRCA recurred approximately 500 days later and was accompanied by ATLL exacerbation. The findings in this patient suggest that the Good's syndrome-like symptoms (thymic hyperplasia and hypogammaglobulinemia) in this patient and PRCA may have been paraneoplastic syndromes caused by ATLL.

摘要

由胸腺增生引起的获得性纯红细胞再生障碍性贫血(PRCA)极为罕见。我们在此报告一名既往健康的41岁男性,他出现了严重贫血、淋巴结病、上纵隔肿块和低丙种球蛋白血症。该患者最终被诊断为PRCA和成人T细胞白血病/淋巴瘤(ATLL)。纵隔肿块经病理诊断为胸腺增生,无明确的ATLL浸润。尽管胸腺切除术后他的贫血迅速改善,但PRCA在大约500天后复发,并伴有ATLL病情加重。该患者的这些发现提示,该患者出现的类似古德综合征的症状(胸腺增生和低丙种球蛋白血症)及PRCA可能是由ATLL引起的副肿瘤综合征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/003d/12120213/98c3533c3829/1349-7235-64-1393-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/003d/12120213/f1cec6273078/1349-7235-64-1393-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/003d/12120213/59c389ed7c6b/1349-7235-64-1393-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/003d/12120213/d7bc0174315a/1349-7235-64-1393-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/003d/12120213/6c2a0593a427/1349-7235-64-1393-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/003d/12120213/98c3533c3829/1349-7235-64-1393-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/003d/12120213/f1cec6273078/1349-7235-64-1393-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/003d/12120213/59c389ed7c6b/1349-7235-64-1393-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/003d/12120213/d7bc0174315a/1349-7235-64-1393-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/003d/12120213/6c2a0593a427/1349-7235-64-1393-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/003d/12120213/98c3533c3829/1349-7235-64-1393-g005.jpg

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本文引用的文献

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T cell clonal expansion and STAT3 mutations: a characteristic feature of acquired chronic T cell-mediated pure red cell aplasia.T 细胞克隆扩增和 STAT3 突变:获得性慢性 T 细胞介导的纯红细胞再生障碍性贫血的特征性特征。
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True thymic hyperplasia causing pure red cell aplasia: a case report.
真性胸腺增生致纯红细胞再生障碍:病例报告。
Interact Cardiovasc Thorac Surg. 2022 Mar 31;34(4):697-699. doi: 10.1093/icvts/ivab301.
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High frequency of STAT3 gene mutations in T-cell receptor (TCR)γδ-type T-cell large granular lymphocytic leukaemia: implications for molecular diagnostics.T细胞受体(TCR)γδ型T细胞大颗粒淋巴细胞白血病中STAT3基因突变的高频率:对分子诊断的意义
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