血小板低下症的区分及血小板输注合理评估.ppt

血小板低下症的区分及血小板输注合理评估张志升台湾台大医院输血医学科2016/6/26Content血小板低下症的原因新生儿血小板低下症药物诱导血小板低下症血栓性血小板低下症特发性血小板减少紫癜肝素诱导血小板低下症血小板低下症的原因骨髓制造血小板的功能减少血小板制造可因放射线、化学治疗或病毒感染等原因受抑制,而骨髓本身疾病,如再生不良性贫血、白血病,骨髓分化不良等皆可引起制造减少。血小板在周边血液被破坏增加常见的有经由产生自体免疫抗体来破坏,如全身性红斑性狼疮、特发性血小板减少性紫斑症、某些药物等,而病毒感染也常引起血小板低下,如登革热病毒、人类免疫缺乏病毒等。此外血小板低下也常见于脾脏肿大病人,如肝硬化病人因门脉高压引起脾脏肿大,造成血小板破坏增加。EvaluationofachildwiththrombocytopeniaPlateletcount<150,000cells/uL,age>3mouthsCBC,bloodsmearevaluationAnemia+thrombocytopeniapancytopeniaPlateletclumpspresentpseudothrombocytopeniaIllappearing?NoCongenitalanomalies?YesNoPMNhypersegmentationRBCmacroovalocytosis?↓B12or↓RBCfolateB12orfolatedeficiencyMedicationsImmunizationsIrradiationToxins?NoYesMacrothrombocytesDrug-inducesLiveimmunizationIrradiationToxinsYesNoOthermorphologicplateletchangesNootherplateletchangeBonemarrowCyanoticheartdiseaseFanconianemiaDyskeratosiscongenitaTrisomy13or18Syndromes:Kasabach-MerrittTARAlportvariantsSyndromes:May-hegglinHermansky-PudiakGrayplateletITPHereditarythrombocytopeniaBernard-Soulier↑NImegakaryocytes↓megakaryocytesLeukemiaAplasticanemiaDrug-inducedAmegakaryocyticthrombocytopeniaMyelodysplasiaITPisadiagnosisofexclusionResponsetotherapy,ifneeded(corticosteroid,IVIG,anti-Dantibody),confirmsthediagnosisYesPTT,PT,TTprolongedDICR/OsepsisSeeConsumptionalcoagulopathyNormal↑↑SpleenSignsofportalhypertensionplatelet>50,000+/-pancytopeniaMaleEczemaRecurrentinfectionSmallplateletsLymphadenopathyHepatosplenomegalySuperiorvenacavasyndromeAbdominalmassChronicallyillappearingAcute,fibrileillnessWBCenzymeassaysUltrasonographyThicksmearBiopsyoflymphnode,massorbonemarrowconsidertumorlysisandsuperiorvenacavasyndromesHIVassayANAU/ARenalfunctionBloodculture?antibioticsMalariaGaucherdiseasePortalhypertensionHepaticschistosomiasisCavernoustransformationoftheportalveinWiskott-AldrichsyndromeLymphoma:HodgkinNon-HodkinNeuroblastomaleukemiaMyelodysplasiaHIVAutoimmuneorconnectivetissuediseaseHUS/TTP+othermicroangiopathiesardiacvalveR