ITP typically presents with the sudden appearance of a petechial rash, spontaneous bruising and/or bleeding in an otherwise well child.

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1 Acute Immune Thrmbcytpenia Purpura (ITP) Backgrund Primary immune thrmbcytpenia (ITP) is an acquired immune mediated disrder characterised by islated thrmbcytpenia, defined as a peripheral bld platelet cunt f less than 100 x 10 9 /L in the absence f any bvius initiating r underlying cause. Pathphysilgy Immune mediated thrmbcytpenia is primarily a disease f increased peripheral platelet destructin with mst patients having autantibdies directed against specific platelet membrane antigens. Antibdy cated platelets are rapidly cleared by tissue macrphages predminately in the spleen leading t a substantial fall in the platelet cunt. Impaired platelet prductin and T cellmediated effects als play a rle. Epidemilgy: 4/ children in the UK Typically less than 10 years f age Clinical Manifestatins ITP typically presents with the sudden appearance f a petechial rash, spntaneus bruising and/r bleeding in an therwise well child. Histry Histry shuld be fcussed n assessing the risk r extent f bleeding and excluding ther causes f thrmbcytpenia Type, severity and duratin f bleeding Presence f systemic symptms such as fever, anrexia, bne r jint pain and weight lss which may be indicative f an underlying disease such as malignant r autimmune disease A histry f viral infectin within the preceding mnth is present in abut 60% f cases Prir histry f significant disease r expsure t relevant drugs (e.g. Phenytin, Valprate, Carbamazepine, Vancmycin, Septrin) Family histry f thrmbcytpenia r bleeding disrders The pssibility f nn accidental injury shuld be cnsidered in yung children presenting with bruising Examinatin

2 Physical examinatin shuld be nrmal aside frm bleeding manifestatins: Cutaneus bleeding such as petechiae,purpura and bruising Mucsal bleeding invlving nasal passages, ral mucsa, gastr intestinal, r geniturinary tracts Mucsal bleeding is significantly mre cmmn if the platelet cunt is <10 x10 9 /L Significant enlargement f lymph ndes, liver r spleen shuld prmpt cnsideratin f an alternative diagnsis Neurlgical signs which may be indicative f intracranial haemrrhage particularly in thse patients with a histry f head trauma Labratry results: FBC/bld film thrmbcytpenia shuld be the ONLY abnrmality in the 3 main cell lines (atypical lymphcytes may be present in viral infectins) Anaemia may be present IF there has been significant bld lss Cagulatin screen nrmal Bne marrw examinatin Bne marrw examinatin is unnecessary in children with typical features f ITP and n ther wrrying signs r symptms Cntrary t previus guidance it is n lnger necessary prir t initiatin f treatment with sterids It is recmmended in patients wh d nt respnd t treatment r prir t splenectmy Diagnsis: Diagnsis is ne f exclusin. The presence f atypical features determines whether further investigatin is needed t rule ut specific causes f thrmbcytpenia including: Bne marrw disease including leukaemia and ther malignancy r aplastic anaemia Inherited thrmbcytpenia including TAR syndrme, Wisktt Aldrich syndrme, Bernard Sulier syndrme, type IIB vn Willebrand disease Autimmune disease such as SLE r ther rheumatid disrders Immundeficiency such as cmmn variable immundeficiency and Di Gerge syndrme

3 Viral infectins including HIV, Hepatitis r CMV Prgnsis: ITP is typically benign and self limiting. 75% reslve spntaneusly within three mnths f nset 5 15% becmes persistent (lasting 3 12 mnths frm diagnsis) r chrnic (lasting > 12 mnths) mre cmmn if presenting > 10 years f age Intracranial haemrrhage is a rare but serius cnsequence ( %) Management: General advice: Reassure patients and parents mst patients can live cmfrtably and safely with petechiae and a lw platelet cunt N IM injectins Stp/d nt prescribe NSAIDS and review any ther medicatin Cnsider Nrethisterne r the OCP in girls wh have menstruating Advise t avid cntact sprts r activities with a high risk f trauma r head injury All patients shuld have pen access t CAU until their platelet cunt has recvered All families shuld be given an infrmatin leaflet and the cntact details fr the ITP Supprt Assciatin ( Specific treatment: The decisin t treat shuld be based upn the severity f bleeding symptms and nt n the platelet cunt r cutaneus signs alne. In general specific treatment t raise the platelet cunt shuld nly be cnsidered in thse patients with severe bleeding including GI bleeding, uncntrlled epistaxis r intracranial haemrrhage (see flw chart) Fllw up: Refer ALL patients via t Dr Ktecha/Dr Bhuller wh will arrange fllw up All patients with a platelet cunt <10 shuld have a repeat FBC in 1 week (arrange thrugh Paediatric Day Unit)

4 All fllw up will be with the Paediatric haematlgy team References: 1. Prvan D, Stasi R, Newland AC et al. Internatinal cnsensus reprt n the investigatin and management f primary immune thrmbcytpenia. Bld 2010; 115: Neunert C, Lim W, Crwther M et al. The American Sciety f Hematlgy 2011 evidence based practice guideline fr immune thrmbcytpenia. Bld 2011; 117: Flw Chart: Management f acute ITP Establish diagnsis Assess severity based n clinical symptms Severe GI bleeding, uncntrlled epistaxis, intracranial haemrrhage Mild N active bleeding, cntrlled epistaxis, bruising, petechiae 1. Admit and bserve 2. Grup and save 3. Is the bleeding life threatening? YES XM 2 units RBC Infrm haematlgist Immediate treatment: 1. IVIG (1g/kg) Cnsider: 1. IV methylprednislne 2 Platelet transfusin 1 NO Discuss with haematlgist Cnsider treatment with: 1. Prednislne (2mg/kg OD starting dse) 3 2. IVIG (1g/kg) 2 1. N treatment required 2. Reassurance and advice 3. Discharge with pen access 4. Refer t Haematlgy Apprved by: Remember Childrens Clinical additinal Practice Grup supprtive measures: NB: Paper cpies Bld f transfusin this dcument may nt be mst recent versin. The definitive versin is held n INsite Dcuments Lcal cntrl f bleeding

5 PRESCRIBING AND ORDERING IVIG: IVIG is indicated in life threatening bleeds r intracranial haemrrhage. It is a RED classificatin and therefre des nt require apprval frm the Trust Immunglbulin Assessment Panel prir t issue. 1. Cmplete new patient request frm (available n insite) and t immunglbulins.mailbx@uhl tr.nhs.uk 2. Prescribe immunglbulin n drug chart as per IV plicy 3. Send prescriptin and a printed cpy f the request frm t pharmacy wh will issue Krishna Ktecha Review date: April 2018