Retacrit 4 , 000 units/ 0. 4 ml solution for injection pre-filled syringes|
6 pre-filled disposable injectionP£ 115. 40 (Hospital only)
Retacrit 5 , 000 units/ 0. 5 ml solution for injection pre-filled syringes|
6 pre-filled disposable injectionP£ 144. 25 (Hospital only)
Epoetin zeta 40000 unit per 1 mlRetacrit 20 , 000 units/ 0. 5 ml
solution for injection pre-filled syringes| 1 pre-filled disposable
injectionP£ 96. 16 (Hospital only)
Retacrit 40 , 000 units/ 1 ml solution for injection pre-filled syringes|
1 pre-filled disposable injectionP£ 193. 32 (Hospital only)
Retacrit 30 , 000 units/ 0. 75 ml solution for injection pre-filled syringes|
1 pre-filled disposable injectionP£ 144. 25 (Hospital only)1.1a Atypical haemolytic uraemic
syndrome and paroxysmal
nocturnal haemoglobinuria
IMMUNOSUPPRESSANTS›MONOCLONAL
ANTIBODIESEculizumab 04-Dec-2017
lDRUG ACTIONEculizumab, a recombinant monoclonal
antibody, inhibits terminal complement activation at the
C 5 protein and thereby reduces haemolysis and
thrombotic microangiopathy.lINDICATIONS AND DOSE
Reduce haemolysis in paroxysmal nocturnal
haemoglobinuria (PNH), in those with a history of blood
transfusions (under expert supervision)
▶BY INTRAVENOUS INFUSION
▶Child:Refer for specialist advice, experience very
limited
Reduce thrombotic microangiopathy in atypical
haemolytic uraemic syndrome (aHUS) (specialist use
only)
▶BY INTRAVENOUS INFUSION
▶Child 2 months–17 years (body-weight 5–9 kg):Initially
300 mg once weekly for 2 weeks, followed by 300 mg
every 3 weeks
▶Child 2 months–17 years (body-weight 10–19 kg):Initially
600 mg once weekly for 1 week, then reduced to 300 mg
once weekly for 1 week, followed by 300 mg every
2 weeks
▶Child 2 months–17 years (body-weight 20–29 kg):Initially
600 mg once weekly for 3 weeks, followed by 600 mg
every 2 weeks
▶Child 2 months–17 years (body-weight 30–39 kg):Initially
600 mg once weekly for 2 weeks, then increased to
900 mg once weekly for 1 week, followed by 900 mg
every 2 weeks
▶Child 2 months–17 years (body-weight 40 kg and above):
Initially 900 mg once weekly for 4 weeks, then
increased to 1. 2 g once weekly for 1 week, followed by
1. 2 g every 2 weekslUNLICENSED USENot licensed for use in children for
paroxysmal nocturnal haemoglobinuria.
lCONTRA-INDICATIONSPatients unvaccinated against
Neisseria meningitidis.unresolvedNeisseria meningitidis
infection
lCAUTIONSActive systemic infection
CAUTIONS, FURTHER INFORMATION
▶Meningococcal infectionVaccinate againstNeisseria
meningitidisat least 2 weeks before treatment (tetravalent
vaccine against serotypes A, C, W 135 and Y
recommended); revaccinate according to current medical
guidelines. Patients receiving eculizumab less than
2 weeks after receiving meningococcal vaccine must be
given prophylactic antibiotics until 2 weeks aftervaccination. Advise patient to report promptly any signs of
meningococcal infection. Other immunisations should
also be up to date.
lINTERACTIONS→Appendix 1 : monoclonal antibodies
lSIDE-EFFECTS
▶Common or very commonAlopecia.asthenia.chills.cough
.decreased leucocytes.diarrhoea.dizziness.fever.
gastrointestinal discomfort.headache.hypertension.
increased risk of infection.influenza like illness.joint
disorders.muscle complaints.nausea.oropharyngeal
pain.pain.skin reactions.sleep disorders.taste altered.
tremor.vomiting
▶UncommonAbscess.anxiety.appetite decreased.chest
discomfort.constipation.cystitis.depression.dysuria.
haemorrhage.hotflush.hyperhidrosis.hypersensitivity.
hypotension.meningitis meningococcal.mood swings.
nasal complaints.oedema.palpitations.paraesthesia.
sepsis.spontaneous penile erection.throat irritation.
tinnitus.vascular disorders.vertigo.vision blurred
▶Rare or very rareAbnormal clotting factor.conjunctival
irritation.feeling hot.gastrooesophageal reflux disease.
gingival discomfort.Grave’s disease.infusion related
reaction.jaundice.malignant melanoma.menstrual
disorder.syncope.urogenital tract gonococcal infection
lCONCEPTION AND CONTRACEPTIONManufacturer advises
effective contraception during and for 5 months after
treatment.
lPREGNANCYNo information available—use only if
potential benefit outweighs risk. Human IgG antibodies
known to cross placenta.
lBREAST FEEDINGNo information available—manufacturer
advises avoid breast-feeding during and for 5 months after
treatment.
lMONITORING REQUIREMENTS
▶Monitor for 1 hour after infusion.
▶Forparoxysmal nocturnal haemoglobinuria, monitor for
intravascular haemolysis (including serum-lactate
dehydrogenase concentration) during treatment and for at
least 8 weeks after discontinuation.
▶Foratypical haemolytic uraemic syndrome, monitor for
thrombotic microangiopathy (measure platelet count,
serum-lactate dehydrogenase concentration, and serum
creatinine) during treatment and for at least 12 weeks after
discontinuation.
lDIRECTIONS FOR ADMINISTRATIONDilute requisite dose to
a concentration of 5 mg/mL with Glucose 5 % or Sodium
Chloride 0. 9 % and mix gently; give over 25 – 45 minutes. If
infusion-related reactions occur, infusion time may be
increased to 4 hours in child under 12 years or 2 hours in
child over 12 years.
lPRESCRIBING AND DISPENSING INFORMATIONConsult
product literature for details of supplemental doses with
concomitant plasmapheresis, plasma exchange, or plasma
infusion.
lPATIENT AND CARER ADVICEPatient or carers should be
advised to report promptly any signs of meningococcal
infection.
A patient information card should be provided.
lNATIONAL FUNDING/ACCESS DECISIONS
NICE decisions
▶Eculizumab for treating atypical haemolytic uraemic
syndrome (January 2015 )NICE HST1
Eculizumab, within its marketing authorisation, is
recommended for funding for treating atypical haemolytic
uraemic syndrome, only if all the following arrangements
are in place:
.coordination of eculizumab use through an expert
centre,and
.monitoring systems to record the number of people with
a diagnosis of atypical haemolytic uraemic syndrome568 Anaemias BNFC 2018 – 2019
Blood and nutrition9