Expression patterns of fetal hemoglobin in sickle cell erythrocytes are both patient- and treatment-specific during childhood.
Expression patterns of fetal hemoglobin in sickle cell erythrocytes are both patient- and treatment-specific during childhood.
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DOI：
10.1002/pbc.22643
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发表时间：
2011-01
影响因子：
3.2
通讯作者：
Miller, Jeffery L.
中科院分区：
文献类型：
--
作者：
Meier, Emily Riehm;Byrnes, Colleen;Weissman, Maxine;Noel, Pierre;Luban, Naomi L. C.;Miller, Jeffery L.
镰状细胞病（SCD）患儿治疗相关的胎儿血红蛋白（HbF）表达模式尚未得到充分描述。本研究的目的是比较接受羟基脲（HU）、慢性输血（Tx）或不接受慢性治疗（Ctrl）的儿童SCD患者外周血中HbF的表达谱（HbF和f细胞）。收集年龄在1个月至21岁之间的SCD患者的外周血样本，并用抗hbf和抗hba抗体进行免疫染色。用这种双染色法枚举含有HbF （f细胞）的红细胞。采用HPLC法测定HbF。将44例≤4岁患者的血液与年龄较大的儿童进行比较（50例，17例HU， 17例Tx）。在年龄较大的儿童中，Tx组HbF和f细胞的百分比均较对照组显著降低（HbF为5.4±4.2%比11.0±7.2%,P = 0.003； f细胞为30.2±16.3%比43.8±20.4%,P = 0.0071）。HU组f细胞的分布明显增加（56.3±17.1%比43.8±20.4%,P = 0.016）， HbF的增加较弱（14.7±6.4%比11.0±7.2%,P = 0.051）。各组HbF与f细胞分布呈正相关（P < 0.0001）。在个体患者的系列样本中，注意到HbF和f细胞分布的相对静态模式。小儿SCD患者外周血中HbF转换和沉默的模式不同。此后，红细胞HbF表达水平和分布维持在患者和治疗特异性模式下，这可能有助于预测对HbF调节治疗的需求或反应。
Treatment-associated fetal hemoglobin (HbF) expression patterns in children with sickle cell disease (SCD) have not been fully described. The objective of this study was to compare HbF expression profiles (HbF and F-cells) in the peripheral blood of pediatric SCD patients receiving hydroxyurea (HU), chronic transfusions (Tx) or no chronic therapy (Ctrl). Peripheral blood samples were collected from SCD patients between 1 month and 21 years of age and immunostained with anti-HbF and anti-HbA antibodies. Erythrocytes containing HbF (F-cells) were enumerated with this dual staining method. HbF was measured using chromatography (HPLC). Blood from 44 Ctrl patients ≤4 years of age was compared with that from older children (50 Ctrl, 17 HU, 17 Tx). Among the older children, the percentage of both HbF and F-cells in the Tx group was significantly decreased compared to the control (HbF 5.4 ± 4.2% vs. 11.0 ± 7.2%, P = 0.003; F-cells 30.2 ± 16.3% vs. 43.8 ± 20.4%, P = 0.0071). While the distribution of F-cells was significantly increased in the HU group (56.3 ± 17.1% vs. 43.8 ± 20.4%, P = 0.016), the increase in HbF was less robust (14.7 ± 6.4% vs. 11.0 ± 7.2%, P = 0.051). Positive correlations of HbF and F-cell distributions were noted in all groups (P < 0.0001 for all groups). In serial samples from individual patients, relatively static patterns of HbF and F-cell distribution were noted. Pediatric SCD patients possess distinct patterns of HbF switching and silencing in peripheral blood erythrocytes. Thereafter, erythrocyte HbF expression level and distribution are maintained with both patient- and treatment-specific patterns that may be useful for predicting the need or response to HbF-modulating therapy.
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影响因子：
158.5
作者：
PLATT, OS;BRAMBILLA, DJ;KLUG, PP
通讯作者：
KLUG, PP
影响因子：
158.5
作者：
NOGUCHI, CT;RODGERS, GP;SCHECHTER, AN
通讯作者：
SCHECHTER, AN
影响因子：
5.1
作者：
EMOND, AM;COLLIS, R;SERJEANT, GR
通讯作者：
SERJEANT, GR
影响因子：
5.1
作者：
STEVENS, MCG;HAYES, RJ;SERJEANT, GR
通讯作者：
SERJEANT, GR
影响因子：
20.3
作者：
Franco, Robert S.;Yasin, Zahida;Rucknagel, Donald L.
通讯作者：
Rucknagel, Donald L.