Pattern of care of blood donors with early‐uncomplicated hereditary haemochromatosis in a <scp>S</scp>wiss blood donation centre

Stefashyna, O.; Stangel, Martin; Infanti, Laura; Holbro, Andreas; Thewis, André; Buser, Andreas; O'Meara, Alix

doi:10.1111/vox.12078

Cited by 14 publications

(13 citation statements)

References 38 publications

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“…The worldwide consensus recommends maintaining the iron level between 50 and 100 µg/L with regular monitoring1718). As an alternative therapy, iron-chelating agents have also been used in patients with hemochromatosis, because they eliminate excessive iron effectively and induce the rapid restoration of normal ferritin concentrations1920).…”

Section: Discussionmentioning

confidence: 99%

Diabetes mellitus caused by secondary hemochromatosis after multiple blood transfusions in 2 patients with severe aplastic anemia

Kim

Kang

et al. 2017

Ann Pediatr Endocrinol Metab

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Hemochromatosis is an inherited or secondary disorder caused by excessive iron storage leading to multiple organ damage. We describe 2 patients with diabetes mellitus caused by hemochromatosis secondary to multiple blood transfusions due to severe aplastic anemia. Subject 1, who was diagnosed with severe aplastic anemia at 15 years of age, received multiple red blood cell transfusions before he underwent autologous peripheral blood stem cell transplantation (PBSCT) at 22 years of age. At 21 years of age, hyperglycemia was detected with increased hemoglobin A1c and serum ferritin levels, 9.7% and 12,910 ng/mL (normal range, 20–320 ng/mL), respectively. The 24-hour urine C-peptide level was normal with negative antiglutamic acid decarboxylase antibody. Subsequently, metformin and an iron-chelating agent were administered. However, an intensive insulin regimen was necessary 2 years after the onset of diabetes. Subject 2, who was diagnosed with severe aplastic anemia at 2 years of age, received multiple blood transfusions until she underwent haploidentical PBSCT at 13 years of age. At 11 years of age, she developed diabetes mellitus with a high serum ferritin level (12,559.8 ng/mL). She is currently 18 years old and has been treated with an intensive insulin regimen and estrogen/progesterone replacement therapy because of hypogonadotropic hypogonadism. It is presumed that the loss of insulin secretory capacity and insulin resistance played a role in the pathogenesis of diabetes mellitus due to hemochromatosis in these cases.

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Section: Discussionmentioning

confidence: 99%

Diabetes mellitus caused by secondary hemochromatosis after multiple blood transfusions in 2 patients with severe aplastic anemia

Kim

Kang

et al. 2017

Ann Pediatr Endocrinol Metab

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“…Another therapeutic approach has been applied by Sundic et al () and Stefashyna et al (). They chose to remove a standard volume of RBCs in each patient (400 and 360 ml respectively), which also led to a reduction in total number of treatments, but without a reduction in the treatment duration.…”

Section: Methods For Iron Depletionmentioning

confidence: 99%

“…This approach lacks a potential advantage of erythrocytapheresis because a fixed volume is used instead of personalised removal of RBCs, making it relatively more expensive and therefore less competitive with phlebotomy. Also Stefashyna et al () studied a selected population of patients in order to be able to use the removed RBCs for transfusion purposes. However, if patients with HH could be accepted as blood donors, it would be presumably more cost‐effective when 2, 3 or even 4 RBCs units could be removed through erythrocytapheresis, depending on the patients’ TBV and Hb.…”

Section: Methods For Iron Depletionmentioning

confidence: 99%

How we manage patients with hereditary haemochromatosis

2016

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A number of disorders cause iron overload: some are of genetic origin, such as hereditary haemochromatosis, while others are acquired, for instance due to repeated transfusions. This article reviews the treatment options for hereditary haemochromatosis, with special attention to the use of erythrocytapheresis. In general, therapy is based on the removal of excess body iron, for which ferritin levels are used to monitor the effectiveness of treatment. For many decades phlebotomy has been widely accepted as the standard treatment. Recent publications suggest that erythrocytapheresis, as a more individualized treatment, can provide a good balance between effectiveness, tolerability and costs. Other treatments like oral chelators and proton pomp inhibitors, which are used in selected patients, create the possibility to further individualize treatment of hereditary haemochromatosis. In the future, hepcidin-targeted therapy could provide a more fundamental approach to treatment.

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“…Due to this protective approach, in Switzerland, the maximal number of RBC units/year that can be obtained from male donors is the same with both 2RBC apheresis and WB donation, a fact that reduces the advantage of 2RBC collections for RBC procurement. However, if a higher 2RBC donation frequency allows the obtainment of a larger number of RBC units, it also increases the risk of ID anaemia that can lead to a loss of experienced donors and negatively impact the blood supply in the long term On the other hand, asymptomatic carriers of hereditary haemochromatosis, if allowed to donate blood, benefit particularly from 2RBC apheresis, as a large amount of iron is removed with one procedure and stable iron levels can be maintained by regular 2RBC donation .…”

Section: Rbc Apheresis From the Perspective Of The Blood Donormentioning

confidence: 99%

Red cell apheresis: pros and cons

Infanti

2017

ISBT Science Series

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The collection of red blood cells (RBC) by apheresis, first developed in the 1970's for therapeutic use, for many years was not considered suitable for obtaining blood components for transfusion because of concerns on donor safety and elevated costs compared to whole blood (WB) donation. Single-dose RBC apheresis as a part of multicomponent collections and double-dose RBC (2RBC) apheresis for autologous and allogeneic transfusions were approved by the FDA between 1995 and 1997, and in Europe starting from 2000. The widespread implementation of these procedures followed mainly to address blood supply issues, as large numbers of blood components can be rapidly obtained. However, this potential advantage has been only partially confirmed in real practice, with striking regional differences. Limitations of 2RBC collections regarding both blood supply and donor safety have emerged. In particular, donor motivation, iron deficiency and risk of anaemia are issues that need to be addressed. On the other side, 2RBC apheresis represents a valuable alternative to WB collection for autologous or dedicated donations, can reduce the risk of alloimmunization in chronically transfused patients and can facilitate transfusion support in specific cases (i.e. patients with multiple alloantibodies or rare blood groups). Reducing patient exposure to different donors can also be relevant for preventing the transmission of blood-borne infections.

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Pattern of care of blood donors with early‐uncomplicated hereditary haemochromatosis in a Swiss blood donation centre

Cited by 14 publications

References 38 publications

Diabetes mellitus caused by secondary hemochromatosis after multiple blood transfusions in 2 patients with severe aplastic anemia

Diabetes mellitus caused by secondary hemochromatosis after multiple blood transfusions in 2 patients with severe aplastic anemia

How we manage patients with hereditary haemochromatosis

Red cell apheresis: pros and cons

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