AbstractThisarticlesummarizesourapproachtothemanagementofchildrenandadultswithprimaryimmunethrombocytopenia(ITP)whodonotrespondto,cannottolerate,orareunwillingtoundergosplenectomy.WebeginwithacriticalreassessmentofthediagnosisandadeliberateattempttoexcludenonautoimmunecausesofthrombocytopeniaandsecondaryITP.Forpatientsinwhomthediagnosisisaffirmed,weconsiderobservationwithouttreatment.Observationisappropriateformostasymptomaticpatientswithaplateletcountof20to30×/Lorhigher.Weuseatieredapproachtotreatpatientswhorequiretherapytoincreasetheplateletcount.Tier1options(rituximab,thrombopoietinreceptoragonists,low-dosecorticosteroids)havearelativelyfavorabletherapeuticindex.WeexhaustallTier1optionsbeforeproceedingtoTier2,which