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Reticulocyte parameters and hemoglobin F production in sickle cell disease patients undergoing hydroxyurea therapy

机译:接受羟基脲治疗的镰状细胞病患者的网状细胞参数和血红蛋白F产生

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摘要

Hemoglobin F (HbF) is an effective inhibitor of HbS polymerization. Hydroxyurea (HU) is used to increase HbF synthesis and improve the clinical course of sickle cell disease (SCD) patients. We studied a series of laboratory parameters concerning HbF production and reticulocyte response, and compared data between two groups: 1) 13 SCD patients treated with HU, and 2) 33 untreated SCD patients. Higher values of Hb concentration, mean cell volume (MCV), mean cell hemoglobin (MCH), mean reticulocyte volume (MRV), HbF concentration, percentage of F‐cells, and amount of HbF/F‐cells were observed in the treated group of patients. There was no correlation between Hb and HbF elevations. The reticulocyte count, immature reticulocyte count, mean fluorescence index (MFI), and neutrophil count were significantly lower in treated patients. Taken together, these findings suggest that a decreased hemolytic process occurred in patients undergoing HU treatment. There was a significant correlation between MCV and HbF, between MRV and HbF, and between MRV and F‐cell in patients taking HU. These data indicate that macroreticulocytes correspond to F‐reticulocytes, and that an increase in MRV in SCD patients using HU may be an indirect signal of F‐cell production. The concentration of HbF/F‐cells was higher in patients treated with HU, but this increase apparently was independent of F‐cell production. Reticulocyte (RTC) parameters, as assessed by hematological analyzers, may be useful for following erythropoietic changes in patients receiving HU, and can indirectly indicate HbF and F‐cell production induced by HU therapy. J. Clin. Lab. Anal. 17:66–72, 2003. © 2003 Wiley‐Liss, Inc.
机译:血红蛋白F(HbF)是HbS聚合的有效抑制剂。羟基脲(HU)用于增加HbF合成并改善镰状细胞病(SCD)患者的临床病程。我们研究了有关HbF产生和网织红细胞反应的一系列实验室参数,并比较了两组之间的数据:1)13例接受HU治疗的SCD患者,以及2)33例未经治疗的SCD患者。在治疗组中观察到更高的Hb浓度值,平均细胞体积(MCV),平均细胞血红蛋白(MCH),平均网织细胞体积(MRV),HbF浓度,F细胞百分比和HbF / F细胞数量的患者。 Hb和HbF升高之间没有相关性。在治疗的患者中,网织红细胞计数,未成熟网织红细胞计数,平均荧光指数(MFI)和中性粒细胞计数显着降低。综上所述,这些发现表明接受HU治疗的患者发生的溶血过程减少。在接受HU治疗的患者中,MCV与HbF,MRV与HbF之间以及MRV与F细胞之间存在显着相关性。这些数据表明,大网状细胞对应于F网状细胞,使用HU的SCD患者MRV的增加可能是F细胞产生的间接信号。 HU治疗的患者中HbF / F细胞的浓度较高,但这种增加显然与F细胞的产生无关。血液分析仪评估的网织红细胞(RTC)参数可能有助于跟踪接受HU的患者的红细胞生成变化,并可以间接表明HU治疗诱导的HbF和F细胞生成。 J.临床实验室肛门17:66-72,2003.©2003 Wiley-Liss,Inc.

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