Reduction of body iron in HFE -related haemochromatosis and moderate iron overload (Mi-Iron): a multicentre, participant-blinded, randomised controlled trial

Ong, Sim Yee; Gurrin, Lyle C.; Dolling, Lara; Dixon, Jeannette L.; Nicoll, Amanda; Wolthuizen, Michelle; Wood, Erica M.; Anderson, Gregory J.; Ramm, Grant A.; Allen, Katrina J.; Olynyk, John K.; Crawford, Darrell H. G.; Ramm, Louise E.; Gow, Paul J; Durrant, Simon; Powell, Lawrie W.; Delatycki, Martin B.

doi:10.1016/s2352-3026(17)30214-4

Cited by 40 publications

(48 citation statements)

References 33 publications

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“…Clinical evidence has shown that iron removal via phlebotomy can reverse liver fibrosis, cardiac dysfunction and skin hyperpigmentation, although arthropathy and pancreatic endocrine dysfunction are variably affected by this therapy . Moreover, phlebotomy has been shown to improve chronic fatigue, one of the most prevalent symptoms of HH .…”

Section: Treatment For Iron Overload Caused By Haemochromatosismentioning

confidence: 99%

Diagnosis and management of hereditary haemochromatosis

et al. 2020

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Hereditary haemochromatosis, one of the most common genetic disorders in the United States, can produce systemic iron deposition leading to end-organ failure and death if untreated. The diagnosis of this condition can be challenging as elevated serum ferritin may be seen in a variety of conditions, including acute and chronic liver disease, a range of systemic inflammatory states, and both primary and secondary iron overload syndromes. Appropriate and timely diagnosis of haemochromatosis is paramount as simple interventions, such as phlebotomy, can prevent or reverse organ damage from iron overload. The recognition of other aetiologies of elevated ferritin is also vital to ensure that appropriate intervention is provided and phlebotomy only utilized in patients who require it. In this review, we summarize the existing data on the work up and management of hereditary haemochromatosis and present a practical algorithm for the diagnosis and management of this disease.

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Section: Treatment For Iron Overload Caused By Haemochromatosismentioning

confidence: 99%

Diagnosis and management of hereditary haemochromatosis

et al. 2020

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“…(26)(27)(28) Numerous studies reporting on therapeutic erythrocytapheresis found that up to 25% of collections had complications-mostly light citrate toxicity but also hypotension-related reactions and vein ruptures. (20,(27)(28)(29)(30)(31) An RCT of therapeutic erythrocytapheresis versus phlebotomy found no significant difference in the number of adverse events per number of procedures (27) Fibrosis at last Biopsy F2 F3F4…”

Section: Adverse Events With Treatmentmentioning

confidence: 99%

Systematic Review of the Clinical Outcomes of Iron Reduction in Hereditary Hemochromatosis

et al. 2020

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1470 design, region where study was performed), participant demographics (age, percent male, hemochromatosis diagnosis), iron reduction therapy regimens, and clinical outcomes on mortality, cirrhosis, liver fibrosis, portal hypertension, HCC, liver transplant, arthritis, joint replacement, diabetes, cardiovascular disease, heart failure, erectile dysfunction, hypogonadism, quality of life, fatigue, biochemical iron indices, and liver function tests (Table 2). QUalIty aSSeSSMeNt oF INClUDeD StUDIeS Two authors independently assessed the risk of bias in the included studies (A.P. and T.C.). The ROBINS-I tool (4) or the Cochrane risk of bias tool (5) were used to evaluate the nonrandomized and randomized studies, respectively. Disagreements were resolved by discussion, and a consensus decision was made.

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“…More specifically, it is believed that patients with serum ferritin levels of more than 1000 μg/L should undergo phlebotomy to reduce the incidence of liver cirrhosis and hepatocellular carcinoma . A recent randomized controlled trial investigated the effects of iron removal in HFE‐related hemochromatosis with moderate iron overload (ferritin 300‐1000 μg/L) via erythrocytapheresis (versus sham erythrocytapheresis) . The results of this trial demonstrated benefits in terms of cognitive outcomes, markers for fibrosis, and oxidative stress with erythrocytapheresis.…”

Section: Discussionmentioning

confidence: 99%

Complications of a severe autoimmune hemolytic anemia crisis: transfusional iron overload and gangrenous cholecystitis

et al. 2018

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BACKGROUND Evans syndrome is a rare autoimmune disorder that is defined by the simultaneous or sequential presence of two or more cytopenias without an obvious underlying precipitating cause. Evans syndrome usually follows a chronic relapsing and remitting course and is quite rare, making it difficult to evaluate in clinical studies. CASE REPORT A 66‐year‐old male patient with a 17‐year history of Evans syndrome presented with fulminant autoimmune hemolytic anemia (AIHA). He presented with a markedly elevated C‐reactive protein (CRP; 46 mg/L [normal, 0‐5 mg/L]) before onset of a decrease in hemoglobin. He required the transfusion of 20 units of red blood cells while awaiting response to aggressive immunosuppressive therapy including high‐dose corticosteroids, intravenous immunoglobin therapy, and rituximab. He achieved a complete hematologic response. RESULTS His postdischarge course was complicated by acute cholecystitis requiring laparoscopic cholecystectomy. In addition, his transfusional iron overload requiring 16 phlebotomies to reduce his ferritin level from 4933 μg/L to 326 μg/L, with phlebotomies ongoing every 2 weeks to achieve a ferritin level of less than 100 μg/L. CONCLUSION Neither transfusional iron overload nor acute cholecystitis are well‐recognized complications of a severe episode of AIHA. An elevated CRP has been recently recognized as an important prognostic marker in patients with immune thrombocytopenic purpura and this case suggests a need to evaluate its utility in AIHA.

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Reduction of body iron in HFE -related haemochromatosis and moderate iron overload (Mi-Iron): a multicentre, participant-blinded, randomised controlled trial

Cited by 40 publications

References 33 publications

Diagnosis and management of hereditary haemochromatosis

Diagnosis and management of hereditary haemochromatosis

Systematic Review of the Clinical Outcomes of Iron Reduction in Hereditary Hemochromatosis

Complications of a severe autoimmune hemolytic anemia crisis: transfusional iron overload and gangrenous cholecystitis

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