Lenograstim 33.6 mega uGranocyte- 34 powder and solvent for
solution for injection vials| 1 vialP£ 62. 54 | 5 vialP
£ 312. 694 Platelet disorders
Platelet disorders
Idiopathic thrombocytopenic purpura
Acute idiopathic thrombocytopenic purpura is usually self-
limiting in children. A corticosteroid, such as prednisolone
p. 442 , is sometimes used if idiopathic thrombocytopenic
purpura does not resolve spontaneously or if it is associated
with severe cutaneous symptoms or mucous membrane
bleeding; corticosteroid treatment should not be continued
longer than 14 days regardless of the response.
Immunoglobulinpreparations may be used in idiopathic
thrombocytopenic purpura or where a temporary rapid rise
in platelets is needed, as in pregnancy or pre-operatively;
they are often used in preference to a corticosteroid. Anti-D
(Rh 0 ) immunoglobulin p. 771 is licensed for the management
of idiopathic thrombocytopenic purpura.
Other therapy that has been tried under specialist
supervision in refractory idiopathic thrombocytopenic
purpura includes azathioprine p. 518 , cyclophosphamide
p. 535 , vincristine sulfate p. 550 , and ciclosporin p. 519.
Rituximab p. 530 [unlicensed] is also used in specialist
centres but experience of its use in children is limited. For
patients with chronic severe thrombocytopenia refractory to
other therapy, tranexamic acid p. 82 may be given to reduce
the severity of haemorrhage.
Splenectomy is considered in chronic thrombocytopenic
purpura if a satisfactory platelet count is not achieved with
regular immunoglobulin infusions, if there is a relapse on
withdrawing or reducing the dose of corticosteroid, and if
other therapies are considered inappropriate.
Essential thrombocythaemia
Anagrelide below reduces platelets in essential
thrombocythaemia in patients at risk of thrombo-
haemorrhagic events who have not responded adequately to
other drugs or who cannot tolerate other drugs.
4.1 Essential thrombocythaemia
ANTITHROMBOTIC DRUGS›CYCLIC AMP
PHOSPHODIESTERASE III INHIBITORS
Anagrelide
lINDICATIONS AND DOSE
Essential thrombocythaemia in patients at risk of
thrombo-haemorrhagic events who have not responded
adequately to other drugs or who cannot tolerate other
drugs (initiated under specialist supervision)
▶BY MOUTH
▶Child 7–17 years:Initially 500 micrograms daily, dose to
be adjusted at weekly intervals according to response,
increased in steps of 500 micrograms daily; usual dose
1 – 3 mg daily in divided doses (max. per dose 2. 5 mg);
maximum 10 mg per daylUNLICENSED USENot licensed for use in children.
lCAUTIONSCardiovascular disease—assess cardiac function
before and regularly during treatment.concomitant use of
drugs that prolong QT-interval—assess cardiac function
before and regularly during treatment.risk factors for QT-
interval prolongation—assess cardiac function before and
regularly during treatment
lINTERACTIONS→Appendix 1 : anagrelide
lSIDE-EFFECTS
▶Common or very commonAnaemia.arrhythmias.asthenia
.diarrhoea.dizziness.fluid retention.gastrointestinal
discomfort.gastrointestinal disorders.headaches.nausea
.palpitations.skin reactions.vomiting
▶UncommonAlopecia.appetite decreased.arthralgia.
chest pain.chills.confusion.congestive heart failure.
constipation.depression.dry mouth.dyspnoea.erectile
dysfunction.fever.haemorrhage.hypertension.
insomnia.malaise.memory loss.myalgia.nervousness.
oedema.pain.pancreatitis.pancytopenia.pneumonia.
pulmonary hypertension.respiratory disorders.sensation
abnormal.syncope.thrombocytopenia.weight changes
▶Rare or very rareAngina pectoris.cardiomegaly.
cardiomyopathy.coordination abnormal.drowsiness.
dysarthria.influenza like illness.myocardial infarction.
nocturia.pericardial effusion.postural hypotension.
renal failure.tinnitus.vasodilation.vision disorders
▶Frequency not knownHepatitis.nephritis
tubulointerstitial
lCONCEPTION AND CONTRACEPTIONEffective
contraception required during treatment.
lPREGNANCYManufacturer advises avoid (toxicity in
animalstudies).
lBREAST FEEDINGManufacturer advises avoid—present in
milk inanimalstudies.
lHEPATIC IMPAIRMENTManufacturer advises caution in
mild impairment. Avoid in moderate to severe
impairment.
lRENAL IMPAIRMENTManufacturer advises avoid if
estimated glomerularfiltration rate less than
50 mL/minute/ 1. 73 m2lMONITORING REQUIREMENTS
▶Monitor full blood count (monitor platelet count every
2 days for 1 week, then weekly until maintenance dose
established).
▶Monitor liver function.
▶Monitor serum creatinine.
▶Monitor urea.
▶Monitor electrolytes (including potassium, magnesium
and calcium) before and during treatment.
▶Monitor closely for further signs of disease progression
such as malignant transformation.
lPRESCRIBING AND DISPENSING INFORMATIONInitiate only
when signs of disease progression or patient suffers from
thrombosis.
Consider stopping treatment after 3 months if
inadequate response.
lPATIENT AND CARER ADVICE
Driving and skilled tasksDizziness may affect performance
of skilled tasks (e.g. cycling, driving).lMEDICINAL FORMS
There can be variation in the licensing of different medicines
containing the same drug.
Capsule
▶Anagrelide (Non-proprietary)
Anagrelide (as Anagrelide hydrochloride)
500 microgramAnagrelide 500 microgram capsules|
100 capsuleP£ 280. 00 – £ 404. 57 DT = £ 350. 70
▶Xagrid(Shire Pharmaceuticals Ltd)A
Anagrelide (as Anagrelide hydrochloride) 500 microgramXagrid
500 microgram capsules| 100 capsuleP£ 404. 57 DT = £ 350. 70BNFC 2018 – 2019 Essential thrombocythaemia 581
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