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遗传性血色素沉着症患者通过红细胞单采术比放血疗法更快达到转铁蛋白饱和度的安全范围。

Patients with hereditary hemochromatosis reach safe range of transferrin saturation sooner with erythrocytaphereses than with phlebotomies.

机构信息

Department of Internal Medicine, Division of Gastroenterology and Hepatology, Maastricht University Medical Centre+, Maastricht, The Netherlands.

Department of Transfusion Medicine, Sanquin Blood Supply, Maastricht, The Netherlands.

出版信息

J Clin Apher. 2022 Feb;37(1):100-105. doi: 10.1002/jca.21956. Epub 2021 Dec 13.

Abstract

INTRODUCTION

For the maintenance treatment of patients with hereditary hemochromatosis (HH), it is advised to keep the transferrin saturation (TSAT) <70% to prevent formation of non-transferrin-bound iron and labile plasma iron. The period of the initial iron depletion may last up to 1 year or longer and during this period, the patient is exposed to elevated TSAT levels. Therapeutic erythrocytapheresis (TE) is a modality which has proven to reduce treatment duration of patients with iron overload from HH. In this study, we investigated the time to reach TSAT <70% for both treatment modalities.

METHODS

From a previous randomized controlled trial comparing erythrocytaphereses with phlebotomies (PBMs), we performed an analysis in a subgroup of patients who presented with TSAT >70%. Mann-Whitney U tests were performed to compare the number of treatments and the number of weeks to reach the interim goal of a persistent level of <70% for TSAT between TE and PBM.

RESULTS

The period to reach TSAT levels of <70% was statistically significant shorter for the TE group compared to the PBM treatment group (median treatment procedures [IQR] 2.0 (5) vs 16.0 (23), P-value: <.001, and median treatment duration [IQR]: 5.5 (11) vs 19.0 (29) weeks, P-value: .007).

CONCLUSION

Patients with HH reach a safe TSAT <70% significantly sooner and with less treatment procedures with TE compared to PBM.

摘要

简介

对于遗传性血色素沉着症(HH)患者的维持治疗,建议将转铁蛋白饱和度(TSAT)<70%,以防止非转铁蛋白结合铁和不稳定血浆铁的形成。初始铁耗竭期可能持续长达 1 年或更长时间,在此期间,患者的 TSAT 水平升高。治疗性红细胞单采术(TE)已被证明可缩短 HH 患者铁过载的治疗时间。在这项研究中,我们研究了两种治疗方法达到 TSAT<70%的时间。

方法

从先前一项比较红细胞单采术与放血疗法(PBM)的随机对照试验中,我们对 TSAT>70%的患者进行了亚组分析。采用 Mann-Whitney U 检验比较 TE 和 PBM 两组达到持续<70%TSAT 中间目标的治疗次数和达到该目标的周数。

结果

TE 组达到 TSAT<70%的时间明显短于 PBM 治疗组(中位数治疗程序[IQR]2.0[5]vs 16.0[23],P 值:<.001,中位数治疗持续时间[IQR]:5.5[11]vs 19.0[29]周,P 值:.007)。

结论

与 PBM 相比,HH 患者接受 TE 治疗可更快、更安全地达到 TSAT<70%,且所需治疗程序更少。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eea1/9299622/3ae0592ba734/JCA-37-100-g001.jpg

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