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患有高胎儿血红蛋白(Hb F)的镰状细胞贫血患者中的粒细胞-巨噬细胞集落刺激因子(GM-CSF)

GM-CSF in sickle cell anemia patients with elevated Hb F.

作者信息

Haider M Z, Raghupathy R, Azizieh F, Abdelsalam R, D'Souza T M, Adekile A D

机构信息

Department of Pediatrics, Faculty of Medicine, Kuwait University, Safat, Kuwait.

出版信息

Acta Haematol. 2000;102(3):140-3. doi: 10.1159/000040988.

DOI:10.1159/000040988
PMID:10692677
Abstract

We estimated plasma GM-CSF levels in a group of 28 steady-state sickle cell anemia (SS) patients in Kuwait, using an ELISA technique. There were 24 age-matched Hb AA controls, 14 of whom were healthy while 10 were acutely ill at the time of the study. Five SS patients were also studied during 6 episodes of painful crisis. Among the SS patients, 82.1% were homozygous for the Saudi Arabia/India (SAI) haplotype with Hb F ranging from 15 to 35% and total Hb from 8.5 to 11 g/dl. Three patients (siblings) were SAI/Benin compound heterozygotes with Hb F of 9-23% and total Hb >10 g/dl. One patient each was homozygous for the Benin or the Bantu haplotype; they had Hb F <2% and total Hb of 6.6 and 7.2 g/dl, respectively. Four (14. 3%) steady-state SS patients had detectable plasma GM-CSF ranging from 75 to 1,817.6 pg/ml. These included the 2 patients with Hb F <2. 0% and 2 with the SAI/Benin compound heterozygotes with Hb F of 11 and 9%, respectively. Four (66.7%) SS patients in crisis, 6 (42.9%) healthy controls and 6 (60%) acutely ill controls had detectable plasma GM-CSF. A clearcut association of GM-CSF with Hb F level or degree of anemia in steady-state SS patients could not be established. The appearance of GM-CSF in the plasma of patients in crisis and also among control subjects raises the possibility that other factors are involved in the production of this cytokine in the subjects studied.

摘要

我们采用酶联免疫吸附测定(ELISA)技术,对科威特的一组28例稳态镰状细胞贫血(SS)患者的血浆粒细胞-巨噬细胞集落刺激因子(GM-CSF)水平进行了评估。有24例年龄匹配的血红蛋白AA(Hb AA)对照者,其中14例健康,10例在研究时患有急性疾病。5例SS患者还在6次疼痛危象发作期间接受了研究。在SS患者中,82.1%为沙特阿拉伯/印度(SAI)单倍型纯合子,Hb F范围为15%至35%,总血红蛋白范围为8.5至11 g/dl。3例患者(兄弟姐妹)为SAI/贝宁复合杂合子,Hb F为9%至23%,总血红蛋白>10 g/dl。各有1例患者为贝宁或班图单倍型纯合子;他们的Hb F<2%,总血红蛋白分别为6.6 g/dl和7.2 g/dl。4例(14.3%)稳态SS患者的血浆GM-CSF可检测到,范围为75至1817.6 pg/ml。这些患者包括2例Hb F<2.0%的患者以及2例分别为Hb F为11%和9%的SAI/贝宁复合杂合子患者。4例(66.7%)处于危象的SS患者、6例(42.9%)健康对照者和6例(60%)急性病对照者的血浆GM-CSF可检测到。在稳态SS患者中,无法确定GM-CSF与Hb F水平或贫血程度之间存在明确关联。GM-CSF在处于危象的患者血浆中以及对照受试者中出现,这增加了在研究对象中其他因素参与该细胞因子产生的可能性。

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