Erythrocytapheresis with Recombinant Human Erythropoietin in Hereditary Hemochromatosis Therapy: A New Alternative

Kohan, Abraham I.; Niborski, Ricardo; Daruich, Jorge; Rey, Jorge; Bastos, Fabiana; Amerise, Graciela; Herrera, Rafael; García, M Moreno; Olivera, Walter; Santarelli, María Teresa; Avalos, Julio Sanchez; Findor, Jorge

doi:10.1046/j.1423-0410.2000.7910040.x

Cited by 29 publications

(11 citation statements)

References 31 publications

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“…In addition to the large RBC volume removed by TE, TEs markedly longer lasting effect in comparison to conventional phlebotomy in PV has been attributed to the associated profound loss of iron and the thereby induced growth inhibition of erythropoietin‐independent and dependent erythroid precursor cells . Alternatively, adding recombinant human erythropoietin (rHuEPO) to TE in the induction phase of HH treatment could allow to increase the frequency of RBC depletion thereby accelerating the rate of iron mobilization and restricting the duration of the induction phase . Indeed, in a small noncontrolled case study describing 10 hemochromatosis patients being treated with weekly rHuEPO in addition to TE, the median TE frequency was 3.1 per month and the median length of induction phase was only 3.25 months .…”

Section: Discussionmentioning

confidence: 99%

The efficiency of therapeutic erythrocytapheresis compared to phlebotomy: A mathematical tool for predicting response in hereditary hemochromatosis, polycythemia vera, and secondary erythrocytosis

Evers

Kerkhoffs

Egmond

et al. 2013

J of Clinical Apheresis

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Recently, therapeutic erythrocytapheresis (TE) was suggested to be more efficient in depletion of red blood cells (RBC) compared to manual phlebotomy in the treatment of hereditary hemochromatosis (HH), polycythemia vera (PV), and secondary erythrocytosis (SE). The efficiency rate (ER) of TE, that is, the increase in RBC depletion achieved with one TE cycle compared to one phlebotomy procedure, can be calculated based on estimated blood volume (BV), preprocedural hematocrit (Hct(B)), and delta-hematocrit (ΔHct). In a retrospective evaluation of 843 TE procedures (in 45 HH, 33 PV, and 40 SE patients) the mean ER was 1.86 ± 0.62 with the highest rates achieved in HH patients. An ER of 1.5 was not reached in 37.9% of all procedures mainly concerning patients with a BV below 4,500 ml. In 12 newly diagnosed homozygous HH patients, the induction phase duration was medially 38.4 weeks (medially 10.5 procedures). During the maintenance treatment of HH, PV, and SE, the interval between TE procedures was medially 13.4 weeks. This mathematical model can help select the proper treatment modality for the individual patient. Especially for patients with a large BV and high achievable ΔHct, TE appears to be more efficient than manual phlebotomy in RBC depletion thereby potentially reducing the numbers of procedures and expanding the interprocedural time period for HH, PV, and SE.

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Section: Discussionmentioning

confidence: 99%

The efficiency of therapeutic erythrocytapheresis compared to phlebotomy: A mathematical tool for predicting response in hereditary hemochromatosis, polycythemia vera, and secondary erythrocytosis

Evers

Kerkhoffs

Egmond

et al. 2013

J of Clinical Apheresis

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“…158,159 this technique removes excess iron stores twice as rapidly as phlebotomy of whole blood. 160 in a pilot study conducted in hereditary hemochromatosis patients, erythrocytapheresis showed a reduction of almost 70% in both the total number and the duration of treatments compared with phlebotomy.…”

Section: Dietary Managementmentioning

confidence: 99%

Cardiac Involvement in Hemochromatosis

Gulati

Harikrishnan

Palaniswamy

et al. 2014

Cardiology in Review

114

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Cardiac hemochromatosis or primary iron-overload cardiomyopathy is an important and potentially preventable cause of heart failure. This is initially characterized by diastolic dysfunction and arrhythmias and in later stages by dilated cardiomyopathy. Diagnosis of iron overload is established by elevated transferrin saturation (>55%) and elevated serum ferritin (>300 ng/mL). Genetic testing for mutations in the HFE (high iron) gene and other proteins, such as hemojuvelin, transferrin receptor, and ferroportin, should be performed if secondary causes of iron overload are ruled out. Patients should undergo comprehensive 2D and Doppler echocardiography to evaluate their systolic and diastolic function. Newer modalities like strain imaging and speckle-tracking echocardiography hold promise for earlier detection of cardiac involvement. Cardiac magnetic resonance imaging with measurement of T2* relaxation times can help quantify myocardial iron overload. In addition to its value in diagnosis of cardiac iron overload, response to iron reduction therapy can be assessed by serial imaging. Therapeutic phlebotomy and iron chelation are the cornerstones of therapy. The average survival is less than a year in untreated patients with severe cardiac impairment. However, if treated early and aggressively, the survival rate approaches that of the regular heart failure population.

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“…A limited number of studies have addressed the use of combination therapy using EPO and EA in HH patients. In one study, each patient ( n = 10) was administered 4,000 IU of EPO three times per week and ferritin levels were successfully reduced to normal following 3.25 months of therapy . In the study by Mercuriali et al, patients ( n = 6) received subcutaneous 40,000 IU EPO per week and EA was performed approximately every 2 weeks .…”

Section: Discussionmentioning

confidence: 99%

Efficacy and safety of erythrocytapheresis and low‐dose erythropoietin for treatment of hemochromatosis

Brückl

Kamhieh-Milz

et al. 2016

J of Clinical Apheresis

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Erythrocytapheresis with Recombinant Human Erythropoietin in Hereditary Hemochromatosis Therapy: A New Alternative

Abstract: This method offers better results in less time than traditional phlebotomy. EA with rHuEPO is an effective therapeutic alternative for patients with HH.

Cited by 29 publications

References 31 publications

The efficiency of therapeutic erythrocytapheresis compared to phlebotomy: A mathematical tool for predicting response in hereditary hemochromatosis, polycythemia vera, and secondary erythrocytosis

The efficiency of therapeutic erythrocytapheresis compared to phlebotomy: A mathematical tool for predicting response in hereditary hemochromatosis, polycythemia vera, and secondary erythrocytosis

Cardiac Involvement in Hemochromatosis

Efficacy and safety of erythrocytapheresis and low‐dose erythropoietin for treatment of hemochromatosis

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