G6PD deficiency is genetically variable so drugs which are safe for one person may cause haemolysis in another. For drugs which cause haemolysis the risk and severity are dose related. Drugs which should be avoided are listed in the BNF.

Erythropoietins

Anaemia is an almost invariable consequence of chronic kidney disease (CKD). It causes many debilitating symptoms, e.g. tiredness, lethargy, muscle fatigue, poor exercise capacity. Anaemia is also a major factor contributing to the high prevalence of cardiovascular disease in renal patients, and the consequent increased morbidity and mortality.

Erythropoietins are accepted for the management of anaemia which develops during treatment with peginterferon and ribavirin for chronic hepatitis C virus infection (see Commissioning Policy for more details)

Please refer to NICE TA323 Erythropoiesis stimulating agents (epoetin and darbepoetin) for treating anaemia in people with cancer having chemotherapy (November 2014)

MHRA Drug Safety Update (January 2018): Recombinant human erythropoietins: very rare risk of severe cutaneous adverse reactions (SCARs)

• Be aware of very rare cases of severe cutaneous adverse reactions, including Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), in patients receiving recombinant human erythropoietins (r-HuEPOs); some cases were fatal
• More severe cases were recorded with long-acting r-HuEPOs (darbepoetin alfa and methoxy polyethylene glycol-epoetin beta)
• Advise patients of the signs and symptoms of severe skin reactions at initiation and instruct them to stop treatment and seek immediate medical attention if they develop widespread rash and blistering; these rashes often occur following fever or flu-like symptoms
• Discontinue all r-HuEPOs permanently in patients who develop severe cutaneous adverse reactions such as SJS or TEN

Darbepoetin alfa

• Solution for injection pre-filled syringes
  • 10 micrograms in 0.4ml, 20 micrograms in 0.5ml, 30 micrograms in 0.3ml, 40 micrograms in 0.4ml, 50 micrograms in 0.5ml, 100 micrograms in 0.5ml, 130 micrograms in 0.65ml, 60 micrograms in 0.3ml, 80 micrograms in 0.4ml, 150 micrograms in 0.3ml, 300 micrograms in 0.6ml, 500 micrograms in 1ml
• Solution for injection pre-filled disposable devices
  • 20 micrograms in 0.5ml, 40 micrograms in 0.4ml, 100 micrograms in 0.5ml, 60 micrograms in 0.3ml, 80 micrograms in 0.4ml, 150 micrograms in 0.3ml, 300 micrograms in 0.6ml, 500 micrograms in 1ml

Epoetin alfa

• Prefilled syringes 1000, 2000, 3000, 4000, 5000, 6000, 8000, 10,000, 20,000, 40,000iu

Notes

1. Prescribe by brand. A biosimilar medicine is a biological medicine that is highly similar and clinically equivalent (in terms of quality, safety, and efficacy) to an existing biological medicine however they cannot be considered generic equivalents of the originator biological medicine. Because they are not identical, biological medicines must be prescribed by brand. The decision which brand to prescribe rests with the responsible clinician in consultation with the patient. Automatic substitution of brands at the point of dispensing is not appropriate for biological medicines.

Epoetin beta

• Prefilled syringes 500, 1000, 2000, 3000, 4000, 5000, 6000, 10,000, 20,000, 30,000iu
• Reco-Pen 10,000, 20,000iu

Epoetin zeta

• Prefilled syringes 1000, 2000, 3000, 4000, 5000, 6000, 8000, 10,000, 20,000, 30,000, 40,000iu

Notes

1. Prescribe by brand. A biosimilar medicine is a biological medicine that is highly similar and clinically equivalent (in terms of quality, safety, and efficacy) to an existing biological medicine however they cannot be considered generic equivalents of the originator biological medicine. Because they are not identical, biological medicines must be prescribed by brand. The decision which brand to prescribe rests with the responsible clinician in consultation with the patient. Automatic substitution of brands at the point of dispensing is not appropriate for biological medicines.

Hypoxia-inducible factor, prolyl hydroxylase inhibitor (HIF-PHI)

Roxadustat

• Tablet 20mg, 50mg, 70mg, 100mg, 150mg

Notes

1. NICE TA807: Roxadustat (Evrenzo) is recommended as an option for treating symptomatic anaemia associated with chronic kidney disease (CKD) in adults (July 2022) only if:
   1. they have stage 3 to 5 CKD with no iron deficiency and
   2. they are not on dialysis at the start of treatment and
   3. the company provides roxadustat according to the commercial agreement

Iron overload

Deferasirox

• Tablets 90mg, 180mg, 360mg

Desferrioxamine

• Powder for solution for injection vial 500mg, 2g

Paroxysmal nocturnal haemoglobinuria and atypical haemolytic uraemic syndrome

Eculizumab

• Concentrate for I/V infusion 10mg/ml, 30ml vial

Notes

1. Please refer to: NICE HST1 Eculizumab recommended for treating atypical haemolytic uraemic syndrome (January 2015). NHS England commissioned

Pegcetacoplan

• Solution for infusion vials 1.08mg/20ml

Notes

1. NICE TA778: Pegcetacoplan (Aspaveli) is recommended, within its marketing authorisation, as an option for treating paroxysmal nocturnal haemoglobinuria (PNH) in adults who have anaemia after at least 3 months of treatment with a C5 inhibitor (March 2022)
   1. It is recommended only if the company provides pegcetacoplan according to the commercial arrangement

Ravulizumab

• Concentrate for solution for infusion vials 300mg/30ml, 1,100mg/11ml

Notes

1. NICE TA698: Ravulizumab (Ultomiris) is recommended, within its marketing authorisation, as an option for treating paroxysmal nocturnal haemoglobinuria in adults (May 2021):
   1. with haemolysis with clinical symptoms suggesting high disease activity, or
   2. whose disease is clinically stable after having eculizumab for at least 6 months, and
   3. the company provides it according to the commercial arrangement
2. NICE TA710: Ravulizumab (Ultomiris) is recommended, within its marketing authorisation, as an option for treating atypical haemolytic uraemic syndrome (aHUS) in people weighing 10kg or more (June 2021):
   1. who have not had a complement inhibitor before, or
   2. whose disease has responded to at least 3 months of eculizumab treatment, and
   3. only if the company provides ravulizumab according to the commercial arrangement