Successful treatment of diamond-blackfan anemia with metoclopramide
Successful treatment of diamond-blackfan anemia with metoclopramide
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DOI：
10.1002/ajh.20278
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发表时间：
2005-04-01
影响因子：
12.8
通讯作者：
Eto, Y
中科院分区：
文献类型：
--
作者：
Akiyama, M;Yanagisawa, T;Eto, Y
钻石-黑粉贫血(DBA)是一种先天性贫血，其特征是网织红细胞数量低，骨髓中含有血红蛋白的细胞缺失或严重减少，巨核细胞和粒细胞分化正常。虽然贫血最初可能对皮质类固醇治疗有反应，但许多患者需要终生输血红细胞(RBC)，导致感染并发症和铁超载。甲氧氯普胺最近被用于治疗DBA。甲氧氯普胺治疗诱导催乳素从脑下垂体释放，并刺激红细胞生成。出于这些原因，我们使用甲氧氯普胺治疗了一名20岁的男性DBA患者，该患者对低剂量和高剂量皮质类固醇、环孢素A和他克莫司(FK506)无效。他的血红蛋白和红细胞压积缓慢上升，8个月来一直保持无症状和不依赖输血。在出现治疗相关并发症之前，应考虑甲氧氯普胺治疗难治性DBA。(C)2005年Wiley-Liss，Inc.
Diamond-Blackfan anemia (DBA) is a congenital anemia characterized by a low reticulocyte count, the absence or severe reduction of hemoglobin-containing cells in the bone marrow, and normal megakaryocytic and granulocytic differentiation. Although the anemia may initially respond to corticosteroid therapy, many patients require lifelong red blood cell (RBC) transfusion, leading to infectious complications and iron overload. Metoclopramide has recently been used to treat DBA. Treatment with metoclopramide induces the release of prolactin from the pituitary and stimulates erythropoiesis. For these reasons, we used metoclopramide to treat a 20-year-old man with DBA refractory to low and high doses of corticosteroids, cyclosporin A, and tacrolimus (FK506). The hemoglobin and hematocrit slowly increased, and he has remained asymptomatic and transfusion-independent for 8 months. Metoclopramide therapy should be considered in with refractory DBA before treatment-related complications develop. (c) 2005 Wiley-Liss, Inc.