病例报告：血管免疫母细胞性T细胞淋巴瘤所致纯红细胞再生障碍性贫血。
Case Report: Pure Red Cell Aplasia due to Angioimmunoblastic T-Cell Lymphoma.
作者信息
Vitorino Marina, Nunes Filipa, Costa Mariana, Porteiro Beatriz, Borges Alexys Reis, Machado João
机构信息
Department of Oncology, Hospital Professor Doutor Fernando Fonseca, Amadora, Portugal.
Department of Internal Medicine, Hospital Professor Doutor Fernando Fonseca, Amadora, Portugal.
出版信息
Case Rep Oncol. 2020 Feb 6;13(1):76-78. doi: 10.1159/000505533. eCollection 2020 Jan-Apr.
Pure red cell aplasia (PRCA) is a rare bone marrow failure characterized by a progressive normocytic anemia and reticulocytopenia without leukopenia and thrombocytopenia. It can be associated with various hematological disorders but exceedingly rarely with angioimmunoblastic T-cell lymphoma (AITL). We report the case of a 72-year-old woman with PRCA associated with AITL. The patient presented with severe anemia (hemoglobin 2.6 g/dL) and a low reticulocyte count 0.7%. Direct and indirect Coombs tests were positive. A CT scan of the chest, abdomen, and pelvis revealed multiple lymphadenopathies. A cervical lymph node biopsy was compatible with AITL. A bone marrow biopsy showed medullary involvement by AITL and a severe erythroid hypoplasia with a myeloid:erythroid ratio of 19.70. The patient was started on CHOP and after 6 cycles the PET scan confirmed complete remission.
纯红细胞再生障碍性贫血（PRCA）是一种罕见的骨髓衰竭疾病，其特征为进行性正细胞性贫血和网织红细胞减少，而无白细胞减少和血小板减少。它可与多种血液系统疾病相关，但极罕见与血管免疫母细胞性T细胞淋巴瘤（AITL）相关。我们报告一例72岁女性PRCA合并AITL的病例。该患者表现为严重贫血（血红蛋白2.6 g/dL）和低网织红细胞计数0.7%。直接和间接抗人球蛋白试验均为阳性。胸部、腹部和骨盆的CT扫描显示多处淋巴结肿大。颈部淋巴结活检符合AITL。骨髓活检显示AITL累及骨髓，伴有严重的红系造血低下，髓系与红系比例为19.70。患者开始接受CHOP方案治疗，6个周期后PET扫描证实完全缓解。
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