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羟基脲对镰状细胞贫血未成熟网织红细胞分数的影响。

Effect of hydroxyurea on immature reticulocyte fraction in sickle cell anemia.

作者信息

Bagdasaryan Robert, Glasser Lewis, Quillen Karen, Chaves Fernando, Xu Dongsheng

机构信息

Department of Pathology and Laboratory Medicine, Rhode Island Hospital, Providence, Rhode Island, USA.

出版信息

Lab Hematol. 2007;13(3):93-7. doi: 10.1532/LH96.07008.

DOI:10.1532/LH96.07008
PMID:17984040
Abstract

Immature reticulocyte fraction (IRF) is a good indicator of bone marrow erythropoiesis in response to hemolysis or tissue hypoxia and is markedly increased in sickle cell disease (SS). We compared IRF changes in SS patients with those who were treated with hydroxyurea (SS-HU), and those who had concurrent alpha globin gene deletion (SS-(- deletion). Forty-two patients including 16 SS, 16 SS-HU, and 10 SS-alpha-deletion patients were studied. Significant decreases (P <.01) in reticulocyte indices including IRF, the reticulocyte percentage, and absolute reticulocyte count (ARC) were observed in SS-alpha-deletion compared to SS patients. On the other hand, although the reticulocyte percentage (P <.01) and ARC (P <.01) were significantly decreased in SS-HU compared with SS patients, the IRF was persistently elevated in both groups (P = .4), suggesting continuous bone marrow stimulation in SS-HU patients in response to tissue hypoxia. The possible underlying physiological mechanisms are discussed.

摘要

未成熟网织红细胞分数(IRF)是骨髓红细胞生成对溶血或组织缺氧反应的良好指标,在镰状细胞病(SS)中显著升高。我们比较了SS患者与接受羟基脲治疗的患者(SS-HU)以及同时存在α珠蛋白基因缺失的患者(SS-α缺失)的IRF变化。研究了42例患者,包括16例SS患者、16例SS-HU患者和10例SS-α缺失患者。与SS患者相比,SS-α缺失患者的网织红细胞指数包括IRF、网织红细胞百分比和绝对网织红细胞计数(ARC)显著降低(P<.01)。另一方面,虽然与SS患者相比,SS-HU患者的网织红细胞百分比(P<.01)和ARC(P<.01)显著降低,但两组的IRF持续升高(P =.4),提示SS-HU患者因组织缺氧而持续受到骨髓刺激。文中讨论了可能的潜在生理机制。

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