Prospective Studies Of Phlebotomy Therapy In Hereditary

血色沉着症患者构成了大量和愿意输血的血液来源。最近联邦法规的变化放宽了对血色素沉着病患者输血的限制，然而，目前还没有数据来指导献血中心的静脉采血治疗。我们建立并评估了一项在捐献者中心治疗血色素沉着症患者的计划，并使他们的血液可用于输血。无论受试者是否符合异基因捐献的标准，都可以免费进行静脉采血治疗。以12.5g/dL的Hb作为切取静脉的阈值，以MCV的降低作为指导治疗终点。130名受试者连续入选：74%为HFE基因C282Y突变纯合子，76%符合异基因献血标准，55%为既往献血者。在MCV达到基线以下3%的目标终点之前，平均每周或每两周进行20次静脉采血(范围7-99)，此时铁蛋白<30微克/L，转铁蛋白饱和度<30%。维持治疗期间保持MCV在这个水平所需的中位静脉采血间隔为10周。在1,402次捐献中，没有发生输血传播疾病试剂的血清转换事件。所有病毒剂检测呈阳性的受试者都有可推迟风险的既往病史。截至2003年9月，在美国国立卫生研究院临床中心收集的供异基因使用的红细胞中，有15%来自血色素沉着症捐献者。由于NIH血色病捐赠者的稳定贡献，在其他医院区域血液供应严重不足的情况下，在库存较低的时期，没有必要推迟手术或从外部供应商那里购买血液。我们的数据表明，血色素沉着症患者可以安全和显着地增加同种异体血液供应。提供不受费用或捐赠适宜性限制的静脉采血疗法可以改善获得护理的机会，并消除不完全风险披露的诱因。 目前的工作集中在通过分离的双重红细胞捐献在HH患者管理中的关键检查，优化利用红细胞指数的变化作为静脉采血治疗终点的指南，描述采血治疗对关节炎症状的影响，以及评估治疗期间血清非转铁蛋白结合铁水平的变化。

Persons with hemochromatosis constitute a plentiful and willing source of blood for transfusion. Recent changes in federal regulations have eased restrictions on the use of blood from hemochromatosis subjects for transfusion, however, no data exist to guide the performance of phlebotomy therapy in a blood donor center setting. We established and evaluated a program for treating persons with hemochromatosis in a donor center, and making their blood available for transfusion. Phlebotomy therapy was performed free of charge regardless of whether subjects met criteria for allogeneic donation. Hemoglobin of 12.5 g/dL was used as the threshold for performing phlebotomy and decreases in the MCV were used to guide the endpoints of therapy. One hundred thirty subjects were consecutively enrolled: 74% were homozygous for the C282Y mutation in the HFE gene, 76% met eligibility criteria for allogeneic donation, and 55% were previous blood donors. A median of 20 weekly or biweekly phlebotomies (range 7-99) were performed before the MCV reached the targeted endpoint of 3% below baseline, at which time the ferritin was less than 30 mcg/L and the transferrin saturation less than 30%. The median phlebotomy interval necessary to keep the MCV at this level during maintenance therapy was 10 weeks. No incident seroconversions for agents of transfusion-transmissible disease occurred during 1,402 donations. All subjects testing positive for viral agents gave a prior history of deferrable risk. As of September 2003, hemochromatosis donors were contributing 15% of the red cell units collected for allogeneic use in the NIH Clinical Center. Due to the steady contributions of NIH hemochromatosis donors, it was not necessary to postpone surgery or acquire blood from outside suppliers during periods of low inventory, at a time when regional blood supplies were at critically low levels in other hospitals. Our data demonstrate that hemochromatosis subjects can safely and significantly augment the allogeneic blood supply. Provision of phlebotomy therapy unrestricted by considerations of cost or suitability for donation can improve access to care and remove incentives for incomplete risk disclosure. Current efforts are focused on critical examination of the role of double red cell donation by apheresis in the management of HH subjects, on optimizing the use of changes in red cell indices as a guide to the endpoints of phlebotomy therapy, on delineating the effect of phlebotomy therapy on arthritic symptoms, and on assessing changes in serum non-transferrin bound iron levels during therapy.