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Subcutaneous bortezomib is highly effective for pure red cell aplasia after ABO-incompatible haematopoietic stem cell transplantation

机译:皮下注射硼替佐米对不兼容ABO的造血干细胞移植后的纯红细胞发育不良非常有效

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摘要

Pure red cell aplasia (PRCA) is an uncommon complication of ABO-incompatible haematopoietic stem cell transplantation. It is characterised by anaemia, reticulocytopenia and absence of erythroid precursors in a morphologically normal-appearing bone marrow (Sawada et al, 2009). Most cases of PRCA resolve spontaneously within weeks to months. While a small subset of patients has a protracted disease course requiring continued red blood cell (RBC) transfusions, there is no approved standard of care for PRCA. Tapering of immunosuppressives (including steroids and calcineurin inhibitors), plasma exchange, ritux-imab and anti-thymocyte globulin have all been employed with varying success rates (Booth et al, 2013). Here we describe a case of PRCA after ABO-incompatible transplant that responded remarkably to treatment with subcutaneous administration of the proteasome inhibitor bortezomib.
机译:纯红细胞发育不良(PRCA)是ABO不相容的造血干细胞移植的罕见并发症。它的特征是贫血,网状细胞减少和形态正常的骨髓中不存在类红细胞前体(Sawada等,2009)。大多数PRCA病例会在数周至数月内自发消退。尽管一小部分患者的病程较长,需要持续输注红细胞(RBC),但尚无批准的PRCA护理标准。逐渐减少的免疫抑制剂(包括类固醇和钙调神经磷酸酶抑制剂),血浆置换,利妥昔单抗和抗胸腺细胞球蛋白的使用均取得了不同的成功率(Booth等人,2013)。在这里,我们描述了ABO不兼容移植后的PRCA病例,该病例对皮下注射蛋白酶体抑制剂硼替佐米的治疗反应显着。

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