对吡哆醇有反应且游离红细胞原卟啉值低的获得性低色素性和小细胞性铁粒幼细胞贫血：特发性获得性铁粒幼细胞贫血（IASA）的一个可能亚组。

Acquired hypochromic and microcytic sideroblastic anaemia responsive to pyridoxine with low value of free erythrocyte protoporphyrin: a possible subgroup of idiopathic acquired sideroblastic anaemia (IASA).

作者信息

Takeda Y, Sawada H, Sawai H, Toi-Matsuda T, Tashima M, Okuma M, Watanabe S, Ohmori S, Kondo M

机构信息

Department of Internal Medicine, Faculty of Medicine, Kyoto University, Japan.

出版信息

Br J Haematol. 1995 May;90(1):207-9. doi: 10.1111/j.1365-2141.1995.tb03402.x.

DOI:10.1111/j.1365-2141.1995.tb03402.x

PMID:7786786

Abstract

Patients with idiopathic acquired sideroblastic anaemia (IASA) usually show macrocytic or normocytic anaemia and increased free erythrocyte protoporphyrin (FEP). The mean cell haemoglobin concentration is normal or slightly low. Here we report a pyridoxine-responsive IASA patient with microcytic and hypochromic anaemia and low FEL level; these features are usually seen in cases of hereditary sideroblastic anaemia. Microcytosis increased during therapy. There may be a subgroup of IASA with microcytic and hypochromic anaemia, low normal FEP and some response to pyridoxine like hereditary sideroblastic anaemia.

摘要

特发性获得性铁粒幼细胞贫血（IASA）患者通常表现为大细胞性或正细胞性贫血，游离红细胞原卟啉（FEP）升高。平均红细胞血红蛋白浓度正常或略低。在此，我们报告一例对吡哆醇有反应的IASA患者，其表现为小细胞低色素性贫血和低FEL水平；这些特征通常见于遗传性铁粒幼细胞贫血病例。治疗期间小红细胞症加重。可能存在一个IASA亚组，其具有小细胞低色素性贫血、FEP略低于正常水平且对吡哆醇有类似遗传性铁粒幼细胞贫血的反应。