Mortality in sickle cell patients on hydroxyurea therapy
Mortality in sickle cell patients on hydroxyurea therapy
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DOI：
10.1182/blood-2004-01-0322
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发表时间：
2005-01-15
期刊：
影响因子：
20.3
通讯作者：
Kutlar, A
中科院分区：
文献类型：
--
作者：
Bakanay, SM;Dainer, E;Kutlar, A
羟基尿素(HU)的疗效及其在降低镰状细胞患者死亡率中的作用已经确定。然而，仍有许多患者在服用HU期间死于这种疾病的并发症。在我们中心接受HU治疗的226名患者中，38人死亡(34人死于镰状细胞相关原因)。急性胸部综合征(ACS)是最常见的死亡原因(35%)。死亡患者和存活患者在平均HU剂量、基线胎儿血红蛋白(Hb F)或最大Hb F反应方面没有显著差异。然而，死亡的患者在HU发生时明显年龄更大，贫血更严重，更有可能有BAN或CAM单倍型。他们的血清尿素氮(BUN)和肌酐水平也显著高于对照组。在接受HU治疗期间死亡的镰状细胞患者可能代表了老年患者的一个亚群。可能有更严重的疾病和更严重的器官损伤。这类患者需要早期识别，并及时送往HU机构。(C)2005年，由美国血液病学会提供。
The efficacy of hydroxyurea (HU) and its role in the reduction in mortality in sickle cell patients has been established. Nevertheless, many patients still die of complications of this disease while on HU. Of the 226 patients treated with HU at our center, 38 died (34 of sickle cell-related causes). Acute chest syndrome (ACS) was the most common (35%) cause of death. Deceased and surviving patients did not differ significantly in average HU dose, baseline fetal hemoglobin (Hb F), or maximum Hb F response. However, the deceased patients were significantly older when HU was instituted, were more anemic, and more likely to have BAN or CAM haplotypes. They also had significantly higher serum blood-urea-nitrogen (BUN) and creatinine levels. Sickle cell patients who die while on HU therapy may represent a subgroup of older patients. possibly with more severe disease and more severe organ damage. Such patients need early identification and prompt HU institution. (C) 2005 by The American Society of Hematology.