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重型β地中海贫血患者造血干细胞移植后肝铁过载与肝纤维化:一项初步研究。

Hepatic iron overload and fibrosis in patients with beta thalassemia major after hematopoietic stem cell transplantation: A pilot study.

作者信息

Ghavamzadeh Ardeshir, Mirzania Mehrzad, Kamalian Naser, Sedighi Nahid, Azimi Parisima

机构信息

Professor of Medicine, Hematology, Oncology and Stem Cell Transplantation Research Center, Tehran University of Medical Sciences, Tehran, Iran.

Hematologist and Medical Oncologist, Cancer Research Center, Cancer Institute, Tehran University of medical Sciences, Tehran, Iran.

出版信息

Int J Hematol Oncol Stem Cell Res. 2015 Apr 1;9(2):55-9.

Abstract

Currently, hematopoietic stem cell transplantation (HSCT) is the only curative option for patients with beta-thalassemia major, but liver iron overload in these patients will not decrease and hepatic fibrosis may still progress despite successful HSCT. Liver biopsy samples were taken from 14 patients (Out of 25 patients) who underwent HSCT. All patients met three criteria: negative HCV antibody, liver fibrosis in samples before HSCT and lack of regular treatment for iron overload after HSCT (Because patients did not consent to phlebotomy or they had not regular follow-up). We evaluated liver fibrosis and liver iron overload by a semi quantitative method, Perls' Prussian blue staining, before and after HSCT. HSCT was successful in all the patients. Liver iron overload did not change after transplant (P=0.61), but hepatic fibrosis progressed after transplant (P=0.01). In patients with beta thalassemia major who previously had some degree of liver fibrosis, HSCT alone cannot reduce liver iron overload and liver fibrosis will increase. We recommend that regardless of the amount of iron overload in patients with beta thalassemia major that have shown some degree of fibrosis in their liver biopsy before transplantation, appropriate steps should be taken to reduce iron overload as soon as possible after successful transplantation.

摘要

目前,造血干细胞移植(HSCT)是重型β地中海贫血患者唯一的治愈选择,但尽管HSCT成功,这些患者的肝脏铁过载并不会减轻,肝纤维化仍可能进展。从接受HSCT的25例患者中的14例获取肝活检样本。所有患者均符合三项标准:HCV抗体阴性、HSCT前样本存在肝纤维化以及HSCT后缺乏铁过载的常规治疗(因为患者不同意放血或未进行定期随访)。我们在HSCT前后通过半定量方法(Perls普鲁士蓝染色)评估肝纤维化和肝脏铁过载情况。所有患者的HSCT均成功。移植后肝脏铁过载未改变(P = 0.61),但移植后肝纤维化进展(P = 0.01)。在之前有一定程度肝纤维化的重型β地中海贫血患者中,单纯HSCT不能降低肝脏铁过载,且肝纤维化会加重。我们建议,对于移植前肝活检已显示一定程度纤维化的重型β地中海贫血患者,无论其铁过载程度如何,成功移植后应尽快采取适当措施降低铁过载。

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