Stimulation of F-cell production in patients with sickle-cell anemia treated with cytarabine or hydroxyurea.
Stimulation of F-cell production in patients with sickle-cell anemia treated with cytarabine or hydroxyurea.
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用阿糖胞苷或羟基脲治疗的镰状细胞性贫血患者可刺激 F 细胞产生。
DOI：
10.1056/nejm198512193132503
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发表时间：
1985
期刊：
影响因子：
--
通讯作者：
Stamatoyannopoulos,G
中科院分区：
文献类型：
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作者：
Veith,R;Galanello,R;Papayannopoulou,T;Stamatoyannopoulos,G
为了研究镰状细胞性贫血（血红蛋白 S 病）中胎儿血红蛋白的药物刺激机制，我们用不同剂量的阿糖胞苷（也称为 Ara-C）或羟基脲治疗了两名纯合子疾病患者，并评估了每种治疗对 F-网织红细胞产生和造血的影响。这些治疗以剂量相关的方式刺激 F 细胞的产生。增加 F 细胞产生的治疗也增加了患者的血细胞比容，并且仅导致白细胞轻微、短暂的减少。对红细胞生成的主要影响包括成熟红细胞的细胞减少（通过网织红细胞的测量来评估）或红系祖细胞区室（集落形成单位-红系和突发形成单位-红系）的减少。减少阶段之后是网织红细胞再生，在此期间发生了 F 网织红细胞绝对数量的大部分增加。较低剂量的阿糖胞苷或羟基脲导致网织红细胞再生波较小，并且对 F-网织红细胞产生的影响较小。这些结果表明，在接受细胞周期特异性化合物治疗的镰状细胞性贫血患者中，刺激胎儿血红蛋白的主要原因是药物治疗引发的红细胞再生。 （新英格兰医学杂志 1985 年；313：1571–5。）
To investigate the mechanism of pharmacologic stimulation of fetal hemoglobin in sickle-cell anemia (hemoglobin S disease), we treated two patients with homozygous disease with various doses of cytarabine (also known as Ara-C) or hydroxyurea and evaluated the effects of each treatment on F-reticulocyte production and on hemopoiesis. The treatments stimulated F-cell production in a dose-related fashion. Treatments that increased F-cell production also increased the patient's hematocrit and caused only minor, transient decreases in white cells. The main effect on erythropoiesis consisted of cytoreduction of the mature erythron (as assessed by measurements of reticulocytes) or a decrease in the compartment of erythroid progenitors (colony-forming units-erythroid and burst-forming units—erythroid). The reduction phase was followed by reticulocyte regeneration, during which most of the increase in the absolute numbers of F reticulocytes took place. Lower doses of cytarabine or hydroxyurea resulted in smaller waves of reticulocyte regeneration and lesser effects on F-reticulocyte production. These results suggest that the main cause of stimulation of fetal hemoglobin in patients with sickle-cell anemia treated with cell cycle—specific compounds is the erythroid regeneration triggered by the drug treatment. (N Engl J Med 1985; 313: 1571–5.)