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[铁粒幼细胞贫血:57例患者的临床与血液学研究]

[Sideroblastic anemia: clinical and hematological study on 57 patients].

作者信息

Vetsch W, Pugin P, Miescher P A

出版信息

Schweiz Med Wochenschr. 1979 Sep 29;109(37):1398.

PMID:482917
Abstract

Sideroblastic anemias (SA) are a heterogeneous group of hematologic disorders marked by a defect of heme synthesis, disturbance of iron metabolism and the presence of ringed sideroblasts. 57 cases of SA, including 23 primary and 34 secondary forms, are discussed. In the group of secondary SA 13 patients were alcoholics, 3 had received chloramphenicol, 2 had lead poisoning, 2 had received busulfan and 2 had immune hemolytic anemia. In some cases serum ferritin was found to be excessively high. The caryotype of one patient presented deletion of the long arm on chromosome 20. None of the patients with primary SA responded to vitamin treatment, and 3 developed a myeloproliferative syndrome.

摘要

铁粒幼细胞贫血(SA)是一组异质性血液系统疾病,其特征为血红素合成缺陷、铁代谢紊乱以及环形铁粒幼细胞的存在。本文讨论了57例SA患者,其中包括23例原发性和34例继发性。在继发性SA组中,13例患者为酗酒者,3例曾使用氯霉素,2例有铅中毒,2例曾使用白消安,2例有免疫性溶血性贫血。部分病例血清铁蛋白过高。1例患者的核型显示20号染色体长臂缺失。原发性SA患者均对维生素治疗无反应,3例发展为骨髓增殖综合征。

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