BNF for Children 2011-2012

448 9.1.3 Drugs used in hypoplastic, haemolytic, renal anaemias BNFC 2011–20129 Nutrition and bloodteroids are used in hypoplastic and haemolytic anaemias.Antilymphocyte immunoglobulin given intravenouslythrough a central line over 12–18 hours each day for 5days produces a response in about 50% of cases ofacquired aplastic anaemia; the response rate may beincreased when ciclosporin is given as well. Severereactions are common in the first 2 days and profoundimmunosuppression can occur; antilymphocyteimmunoglobulin should be given under specialist supervisionwith appropriate resuscitation facilities. Alternatively,oxymetholone tablets (available from ‘specialorder’manufacturers or specialist importing companies,see p. 809) may be used in aplastic anaemia at a dose of1–5 mg/kg daily for 3 to 6 months.It is unlikely that dietary deficit of pyridoxine (section9.6.2) produces clinically relevant haematologicaleffects. However, certain forms of sideroblastic anaemiarespond to pharmacological doses, possibly reflectingits role as a co-enzyme during haemoglobin synthesis.Pyridoxine is indicated in both idiopathic acquiredand hereditary sideroblastic anaemias. Although completecures have not been reported, some increase inhaemoglobin can occur with high doses. Reversiblesideroblastic anaemias respond to treatment of theunderlying cause but pyridoxine is indicated inpregnancy, haemolytic anaemias, or during isoniazidtreatment.Corticosteroids (section 6.3) have an important placein the management of haematological disorders includingautoimmune haemolytic anaemia, idiopathicthrombocytopenias (section 9.1.4) and neutropenias,and major transfusion reactions. They are also used inchemotherapy schedules for many types of lymphoma,lymphoid leukaemias, and paraproteinaemias, includingmultiple myeloma.ErythropoietinsEpoetins (recombinant human erythropoietins) areused to treat the anaemia associated with erythropoietindeficiency in chronic renal failure, see below.Epoetin beta is also used for the prevention of anaemiain preterm neonates of low birth-weight; a therapeuticresponse may take several weeks. Only unpreservedformulations should be used as other preparationsmay contain benzyl alcohol (see Excipients, p. 2).There is insufficient information to support the use oferythropoietins in children with leukaemia or in thosereceiving cancer chemotherapy.Darbepoetin is a glycosylated derivative of epoetin; itpersists longer in the body and can be administered lessfrequently than epoetin.Other factors, such as iron or folate deficiency, thatcontribute to the anaemia of chronic renal failure shouldbe corrected before treatment and monitored duringtherapy. Supplemental iron may improve the response inresistant patients and in preterm neonates (see section9.1.1.1). Aluminium toxicity, concurrent infection, orother inflammatory disease can impair the response toerythropoietin.Erythropoietins—haemoglobin concentrationIn chronic kidney disease, the use of erythropoietinscan be considered in a child with anaemia. The aimof treatment is to relieve symptoms of anaemia andto avoid the need for blood transfusion. The optimumhaemoglobin concentration is dependent onthe child’s age and factors such as symptoms, comorbidities,and patient preferences. The haemoglobinconcentration should not be increased beyondthat which provides adequate control of symptomsof anaemia. In adults, overcorrection of haemoglobinconcentration with erythropoietins in those withchronic kidney disease may increase the risk ofserious cardiovascular events and death; haemoglobinconcentrations higher than 12 g/100 mL shouldbe avoided in children.For MHRA/CHM advice relating to adults, see BNFsection 9.1.3.Pure red cell aplasiaThere have been very rare reports of pure red cellaplasia in patients treated with erythropoietins. Inpatients who develop a lack of efficacy with erythropoietintherapy and with a diagnosis of pure red cellaplasia, treatment with erythropoietins must be discontinuedand testing for erythropoietin antibodiesconsidered. Patients who develop pure red cell aplasiashould not be switched to another form oferythropoietin.DARBEPOETIN ALFACautions see EpoetinContra-indications see EpoetinHepatic impairment manufacturer advises cautionPregnancy no evidence of harm in animal studies—manufacturer advises cautionBreast-feeding manufacturer advises avoid—noinformation availableSide-effects see Epoetin; also, oedema, injection-sitepain; isolated reports of pure red cell aplasia particularlyfollowing subcutaneous administration inpatients with chronic renal failure (discontinue therapy)—seealso notes aboveIndication and doseSymptomatic anaemia associated with chronicrenal failure in children on dialysis (see alsonotes above). By intravenous or subcutaneous injectionChild 11–18 years initially 450 nanograms/kgonce weekly adjusted according to response byapprox. 25% at intervals of at least 4 weeks;maintenance dose, given once weekly or onceevery 2 weeksSymptomatic anaemia associated with chronicrenal failure in children not on dialysis (see alsonotes above). By intravenous or subcutaneous injectionChild 11–18 years by subcutaneous or intravenousinjection, initially 450 nanograms/kg onceweekly or by subcutaneous injection, initially750 nanograms/kg once every 2 weeks; adjustedaccording to response by approx. 25% at intervalsof at least 4 weeks; maintenance dose, given sub-