Effectiveness of Erythrocytapheresis in Idiopathic Hemochromatosis. Report of 14 Cases

Conte, Dario; Mandelli, C.; Cesana, Marina; Ferrini, R; Marconi, Maurizio; Bianchi, Annamaria

doi:10.1177/039139888901200110

Cited by 19 publications

(9 citation statements)

References 8 publications

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“…Therefore, this is an eective procedure for quickly attaining a suitable decrease in iron overload [8]. Sometimes, it may take 2±3 years to decrease the excess iron deposition by manual phlebotomies, which therefore requires the performance of a large number of phlebotomies [4,18,19].…”

Section: Discussionmentioning

confidence: 99%

In hereditary hemochromatosis, red cell apheresis removes excess iron twice as fast as manual whole blood phlebotomy

Muncunill

Vaquer

Galmés

et al. 2002

J of Clinical Apheresis

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The current treatment of hereditary hemochromatosis (HH) consists of performing periodic manual whole blood phlebotomies. Erythroapheresis (EPH) is considered to be an alternative procedure if the classic treatment is contra-indicated. A prospective study of 13 consecutive cases of HH were included in a periodic EPH program with the aim of assessing the efficacy, feasibility, and tolerability of EPH in the treatment of HH by induction and maintenance. Iron depletion (ferritin <20 microg/l) was achieved in all patients after a mean of 6.7 +/- 2.9 months of treatment and a mean of 13.5 +/- 7.2 EPH sessions. The procedure was well tolerated and there were no complications. After a follow-up period of 10.5 +/- 6.6 months, only four patients have required further maintenance sessions beyond 6 months after completing the induction therapy. The efficacy, speed, tolerability, and more favorable schedule of an EPH program facilitate treatment of HH.

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Section: Discussionmentioning

confidence: 99%

In hereditary hemochromatosis, red cell apheresis removes excess iron twice as fast as manual whole blood phlebotomy

Muncunill

Vaquer

Galmés

et al. 2002

J of Clinical Apheresis

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“…SF is monitored in the same manner as previously described for therapeutic phlebotomy. 54,56,57 TE achieves iron depletion in persons with hemochromatosis in a shorter treatment period than therapeutic phlebotomy. 56 In a pilot study of 6 hemochromatosis patients treated with TE, the total number and the duration of treatments in the TE group was approximately 70% lower than that in historical control subjects managed with therapeutic phlebotomy.…”

Section: Erythrocytapheresismentioning

confidence: 99%

How I treat hemochromatosis

Adams

Barton

2010

Blood

159

167

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Hemochromatosis is a common genetic disorder in which iron may progressively accumulate in the liver, heart, and other organs. The primary goal of therapy is iron depletion to normalize body iron stores and to prevent or decrease organ dysfunction. The primary therapy to normalize iron stores is phlebotomy. In this opinion article, we discuss the indications for and monitoring of phlebotomy therapy to achieve iron depletion, maintenance therapy, dietary and pharmacologic maneuvers that could reduce iron absorption, and the role of voluntary blood donation. (Blood. 2010;116(3):317-325)

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“…As juvenile hemochromatosis is typically more severe, oral iron chelation or erythrocytapheresis may be required in such patients as alternative treatment options. Erythrocytapheresis has been shown to be a more effective method of iron removal [55] . A trial of oral iron chelation with deferasirox for the treatment of hemochromatosis showed an unfavorable safety profile [56] , but a case study reports on treatment of juvenile hemochromatosis patients with acutely decompensated heart failure with i.v.…”

Section: Managementmentioning

confidence: 99%

Pathogenesis, Diagnosis and Treatment of Hemochromatosis

Zoller¹,

Henninger

2016

Dig Dis

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Hemochromatosis is a common cause of chronic liver disease and HFE genotyping allows decisive and non-invasive diagnosis. Molecular and clinical genetic studies have led to the identification of genes other than HFE in patients with inherited diseases associated with increased hepatic iron storage that can cause hemochromatosis, which adds complexity to a diagnostic approach to patients with suspected hemochromatosis. Despite major advances in genetics, hepatic iron quantification by non-invasive methods therefore remains the key to the diagnosis of hemochromatosis. Although associated with homozygosity for the C282Y polymorphism in the HFE gene in >80% of patients, hemochromatosis is a complex genetic disease with strong environmental disease modifiers. Testing for mutations in the non-HFE hemochromatosis genes transferrin receptor 2, hemojuvelin, HAMP and SLC40A1 is complex, costly and time-consuming. Demonstration of hepatic iron overload by liver biopsy or MRI is therefore required before such complex tests are carried out. The pathogenesis of chronic liver disease in hemochromatosis is mainly attributed to the redox potential of tissue iron, and only the more recent studies have focused on the toxic properties of circulating iron. Considering the fact that an increased saturation of transferrin and high iron in plasma are the hallmark of all hemochromatosis forms, an alternative view would be that toxic iron in the circulation is involved in the pathogenesis of hemochromatosis. Recent studies have shown an increased concentration of redox-active iron in plasma in patients with increased transferrin saturation. This finding supports the hypothesis that tissue iron may be the ‘smoking gun' of iron-induced organ damage. Taken together, caring for patients with suspected or established hemochromatosis still remains a challenge, where understanding the genetics, biochemistry and cell biology of hemochromatosis will aid better diagnosis and treatment of affected individuals.

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Effectiveness of Erythrocytapheresis in Idiopathic Hemochromatosis. Report of 14 Cases

Cited by 19 publications

References 8 publications

In hereditary hemochromatosis, red cell apheresis removes excess iron twice as fast as manual whole blood phlebotomy

In hereditary hemochromatosis, red cell apheresis removes excess iron twice as fast as manual whole blood phlebotomy

How I treat hemochromatosis

Pathogenesis, Diagnosis and Treatment of Hemochromatosis

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