Thymectomy in Red Cell Aplasia - A Case Report

Arthur dos Santos Sena, Adolpho de Barros e Silva de Oliveira, Maria Carolina Santos Malafaia Ferreira, Duílio Cabral da Costa Neto, César Freire de Melo Vasconcelos, Wolfgang William Schmidt Aguiar
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Abstract

1.1. Background: Pure Red Cell Aplasia (PRCA) is a very rare syndrome responsible for severe anemia due to absence or significant reduction of erythroid precursors in the bone marrow. It’s etiology is variable, ranging from a congenital disease to an acquired one. Secondary acquired PRCA may be associated with several conditions, including Thymic Tumors. 1.2. Case Presentation: A 42-year-old male presented to the Internal Medicine department with constitutional symptoms: non-intentional weight-loss, palpitations, somnolence. Clinical investigation showed a severe normocytic normochromic anemia, with significant reticulocytopenia. Due to clinical status, several blood transfusions were needed. Further investigation with bone marrow biopsy showed intact precursors for platelets and leukocytes, with absence of erythroid precursors; the primary diagnostic hypothesis was Pure Red Cell Aplasia, with no etiology well defined, however. In order to rule out other conditions, imaging scans were performed; the chest CT showed a large mediastinal expansive formation, compatible with Thymoma. Patient was submitted to Thymectomy by the Thoracic Surgery team, with posterior improvement of clinical and hemodynamic status. Even so, for definitive remission of the PRCA, a bone marrow transplant was needed. The patient was successfully treated and remains healthy so far.
胸腺切除术治疗红细胞发育不全1例
1.1. 背景:纯红细胞不全(PRCA)是一种非常罕见的综合征,由于骨髓中红细胞前体缺失或显著减少而导致严重贫血。它的病因多种多样,从先天性疾病到后天疾病都有。继发性获得性PRCA可能与几种情况有关,包括胸腺肿瘤。病例介绍:一名42岁男性,以非故意体重减轻、心悸、嗜睡等体质症状就诊内科。临床表现为严重的正红细胞正色性贫血,伴明显的网状红细胞减少。由于临床状况,需要多次输血。进一步骨髓活检显示血小板和白细胞前体完整,红细胞前体缺失;最初的诊断假设是纯红细胞发育不全,但病因不明。为了排除其他情况,进行了成像扫描;胸部CT示纵隔大片扩张,与胸腺瘤相符。患者在临床和血流动力学状况改善后,由胸外科小组提交胸腺切除术。即便如此,为了彻底缓解PRCA,还需要进行骨髓移植。病人得到了成功的治疗,到目前为止仍然健康。
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