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Chronic iron overload and how it can be treated1

What is chronic iron overload?

Chronic iron overload occurs when the body’s limited iron storage capacities are exceeded over a sustained period of time. There is no natural mechanism to remove excess iron from the body, leading to iron build up within cells. This can damage the cell membrane resulting in fibrosis and cell death.

A cause of chronic iron overload is the use of regular blood transfusions. Blood transfusions can be a requirement for managing many chronic health conditions, such as MDS, SCD and thalassaemia. Iron overload can also occur in non-transfusion-dependent thalassaemia as a result of increased absorption of dietary iron through the GI tract.

How can iron overload be treated?

You may be able to reduce the level of iron in patients suffering from iron overload by treating them with ICT, such as EXJADE. This can prevent iron levels from reaching harmful concentrations.

Guidelines support the use of ICT in the following iron-overloaded patients

Current guidelines support the use of ICT for the treatment of iron overload in MDS, SCD and thalassaemia patients.

Lower-risk MDS patients2–5

Consider ICT in iron-overloaded patients with lower-risk MDS, who are intolerant of desferrioxamine.2

      British Committee for Standards in Haematology (BCSH)2  
     
    Patient Group

    ICT is recommended in MDS patients with a very good prognosis that are:

    • Intolerant to desferrioxamine
    Triggers

    ICT is recommended in MDS patients that have:

    • >20 units of transfused RBCs
    • Serum ferritin >1000 µg/L in patients that are predicted to continue red cell transfusions

    ICT cannot be routinely recommended for patients with MDS with transfusional iron overload. Consideration may be given to ICT for patients with a very good prognosis, specifically patients with WHO:

    • RA
    • RARS
    • Isolated del(5q)
     
      National Comprehensive Cancer Network (NCCN)3  
     
    Patient Group

    ICT is recommended in MDS patients that have:

    • An IPSS score of low- or intermediate-1-risk MDS
    Triggers

    ICT is recommended in MDS patients that have:

    • Received more than 20–30 RBC transfusions
    • Ongoing RBC transfusions anticipated
    • Serum ferritin >2,500 µg/L, with the aim to achieve <1,000 µg/L

    Patients with low creatinine clearance (<40 mL/min) should not be treated with EXJADE or deferoxamine

     
      European Society for Medical Oncology (ESMO)4  
     
    Patient Group

    ICT is recommended in MDS patients that have:

    • An IPSS score of low- or intermediate-1-risk MDS
    Triggers

    ICT is recommended in MDS patients that have:

    • Received 20–60 RBC concentrates
    • Serum ferritin above 1,000–2,500 µg/L
    • Significantly reduced cardiac T2*
     
      Scottish Medicines Consortium (SMC)5  
     
    Patient Group

    Indication under review: ICT is recommended in MDS patients that are:

    • ≥2 years of age
    • Diagnosed with rare acquired or inherited anaemias

    SMC restriction:

    • Use in MDS patients with an IPSS score of low- or intermediate-1 risk MDS
    Triggers

    ICT is recommended in MDS patients when:

    • Deferoxamine therapy is contraindicated or inadequate
     

    EXJADE is the only licensed agent for ICT in MDS when desferrioxamine therapy is contraindicated or inadequate.2

    SCD patients6

    Consider ICT in iron-overloaded patients with SCD who are receiving intermittent/occasional simple transfusions over many years, on regular simple transfusions or on regular exchange transfusions.

      Sickle Cell Society  
     
    Patient Group

    ICT is indicated in SCD patients that require:

    • Intermittent/occasional simple transfusions over many years
    Triggers

    ICT is recommended in SCD patients that have:

    • Appropriate MRI monitoring if serum ferritin is persistently >1,000 μg/L
    • LIC >7 mg/g dw
    • LIC of 5–7 mg/g dw and co-morbidities associated with the heart or liver
    • Regular simple transfusions
    • Received 10–20 units of blood
    • Serum ferritin >1,000 µg/L

    (Continue while transfused with the aim to keep LIC <5 mg/g dw and cardiac T2* >20 ms)

    • Regular exchange transfusions
    • Appropriate MRI monitoring if serum ferritin is persistently >1,000 μg/L
    • LIC >7 mg/g when starting long-term automated transfusion therapy

    (Iron chelation can be stopped once the ferritin is <500 μg/L or LIC <5 mg/g/dw)

     

    Thalassaemia patients7

    Consideration of ICT is recommended in iron-overloaded patients with transfusion-dependent thalassaemia.

      United Kingdom Thalassaemia Society  
     
    Patient group

    ICT is recommended in thalassaemia patients that are:

    • ≥2 years of age
    Triggers

    ICT is recommended in thalassaemia patients that have:

    • 10–12 transfusions
    • Serum ferritin >1,000 µg/L on two readings
    • Children <6 years of age should initially be offered subcutaneous desferrioxamine infusions. If the patient is intolerant or fails to comply, then EXJADE should be started as soon as possible to prevent worsening iron loading
     
     

    Abbreviations: BCSH, British Committee for Standards in Haematology; dw, dry weight; ICT, iron chelation therapy; IPSS, International Prognostic Scoring System; GI, gastrointestinal; LIC, liver iron concentration; MDS, myelodysplastic syndromes; MRI, magnetic resonance imaging; NCCN, National Comprehensive Cancer Network; NHS, National Health Service; PACE, Patient and Clinician Engagement; RA, refectory anaemia; RARS, refractory anaemia with ringed sideroblasts; RBC, red blood cell; SCD, sickle cell disease; SMC, Scottish Medicines Consortium; T2*, T2-star weighted imaging; WHO, World Health Organization.

    References

    1. Cappellini MD, et al. Guidelines for the management of transfusion dependent thalassaemia (TDT). 3rd ed. Nicosia, Cyprus: Thalassaemia International Federation; 2014.
    2. Killick S, et al. Guidelines for the diagnosis and management of adult myelodysplastic syndromes. Br J Haematol 2014;164:503–525.
    3. NCCN. Myelodysplastic syndromes. Version 2. 2018.
    4. Fenaux, et al. Myelodysplastic syndromes: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 2014;25 (Supplement 3): iii57–iii69.
    5. SMC. Re-submission: Deferasirox 125mg, 250mg, 500mg dispersible tablets (EXJADE®). SMC No. (347/07). 2016.
    6. Sickle Cell Society. Standards for clinical care of adults with sickle cell disease in the UK, 2nd Edition, 2018.
    7. United Kingdom Thalassaemia Society. Standards for the clinical care of children and adults with thalassaemia in the UK, 3rd Edition, 2016.
    HCP20-C005a June 2020.
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