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Case Report: Pure Red Cell Aplasia due to Angioimmunoblastic T-Cell Lymphoma

机译:病例报告:血管免疫母细胞性T细胞淋巴瘤引起的纯红细胞发育不良

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摘要

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.
机译:纯红细胞发育不全(PRCA)是一种罕见的骨髓衰竭,其特征是进行性正常血红细胞贫血和网状血细胞减少症,无白细胞减少症和血小板减少症。它可能与多种血液学疾病有关,但与血管免疫母细胞性T细胞淋巴瘤(AITL)很少相关。我们报告了一名与AITL相关的PRCA的72岁女性的病例。该患者出现严重贫血(血红蛋白2.6 g / dL),网织红细胞计数低至0.7%。直接和间接库姆斯试验均为阳性。胸部,腹部和骨盆的CT扫描显示多发性淋巴腺病。宫颈淋巴结活检与AITL兼容。骨髓活检显示,AITL累及了髓质,严重的红系发育不全,骨髓与红系的比率为19.70。患者开始CHOP治疗,经过6个周期的PET扫描证实完全缓解。

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