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P44 Pediatrics
Oncology Toronto Notes 2019
The American Society of Hematology 2011 Evidence-Based Practice Guideline for Immune Thrombocytopenia
Blood 2011;117(16):4190-207 Recommendations
• Bonemarrowexaminationisunnecessary in children and adolescents with the typical features of ITP.
• Bone marrow examination is not necessary in children who fail IVIg therapy (grade 1B).
• Childrenwithnobleedingormildbleeding (defined as skin manifestations only, such as bruising and petechiae) may be managed with observation alone regardless of platelet count.
• For pediatric patients requiring treatment, a single dose of IVIg (0.8-1 g/kg) or a short course of corticosteroids may be used as first-line treatment.
• IVIgcanbeusedifamorerapidincreaseinthe platelet count is desired.
• Anti-Dtherapyisnotadvisedinchildrenwitha hemoglobin concentration that is decreased due to bleeding, or with evidence of autoimmune hemolysis.
Suggestions
• Bonemarrowexaminationisalsonotnecessary in similar patients prior to initiation of treatment with corticosteroids or before splenectomy.
• Testingforantinuclearantibodiesisnot necessary in the evaluation of children and adolescents with suspected ITP.
• Asingledoseofanti-Dcanbeusedasfirst-line treatment in Rh-positive, nonsplenectomized children requiring treatment.
Clinical Presentation
• 50%present1-3wkafterviralillness(e.g.URTI,chickenpox)
• suddenonsetofpetechiae,purpura,epistaxisinanotherwisewellchild
• clinicallysignificantbleedinonly3%(severebleedmorelikelywithplateletcount<10)with<0.5%risk
of intracranial bleed
• no lymphadenopathy, no hepatosplenomegaly
• labs:thrombocytopeniawithnormalRBC,WBC
• bonemarrowaspirateonlyifatypicalpresentation(≥1celllineabnormal,hepatosplenomegaly)
• differentialdiagnosis:leukemia,drug-inducedthrombocytopenia,HIV,infection(viral),autoimmune
(SLE, ALPS)
Management
• observationvs.pharmacologicinterventionhighlydebated;spontaneousrecoveryin>70%ofcases within 3 mo
• treatment with IVIg or prednisone if mucosal or internal bleeding, platelets <10, or at risk of significant bleeding (surgery, dental procedure, concomitant vasculitis or coagulopathy)
• life-threateningbleed:additionalplatelettransfusion±emergencysplenectomy • persistent(>3-12mo)orchronic(>12mo):re-evaluate;treatifsymptomspersist • supportive:avoidcontactsportsandASA/NSAIDs
Hemophilia
• seeHematology,H31
von Willebrand’s Disease
• see Hematology, H30 Oncology
• canceristhesecondmostcommoncauseofdeathafterinjuriesinchildren>1yrofage
• causeisrarelyknown,butincreasedriskforchildrenwith:chromosomalsyndromes(e.g.Trisomy21),
cancer predisposition syndromes (e.g. Li-Fraumeni syndrome), prior malignancies, neurocutaneous
syndromes, immunodeficiency syndromes, family history, exposure to radiation, chemicals, biologic agents • leukemiasarethemostcommontypeofpediatricmalignancy(30%)followedbybraintumours(25%),
and lymphomas (15%)
• somemalignanciesaremoreprevalentincertainagegroups
■ newborns: neuroblastoma, Wilms’ tumour, retinoblastoma
■ infancy and childhood: leukemia, neuroblastoma, CNS tumours, Wilms’ tumour, retinoblastoma ■ adolescence: lymphoma, gonadal tumours, germ cell tumours, bone tumours
• uniquetreatmentconsiderationsinpediatricsbecauseradiation,chemotherapy,andsurgerycanimpact growth and development, endocrine function, and fertility
• goodprognosis:treatmentshaveledtoremarkableimprovementsinoverallsurvivalandcureratesfor many pediatric cancers (>80%)
Lymphadenopathy
Clinical Presentations
• featuresofmalignantlymphadenopathy:firm,discrete,non-tender,enlarging,immobile±suspicious mass/imaging findings ± constitutional symptoms
• fluctuance,warmth,ortendernessaremoresuggestiveofbenignnodes(infection)
Differential Diagnosis
• infection
■ viral: URTI, EBV, CMV, adenovirus, HIV
■ bacterial: S. aureus, GAS, anaerobes, Mycobacterium (e.g. TB), cat scratch disease (Bartonella) ■ other: fungal, protozoan, Rickettsia
• autoimmune:rheumatoidarthritis,SLE,serumsickness
• malignancy:lymphoma,leukemia,metastaticsolidtumours • storagediseases:Niemann-Pick,Gaucher’s
• other:sarcoidosis,Kawasakidisease,histiocytoses