高白细胞血症.ppt

Hyperleukocytosisreferstoalaboratoryabnormalitythathasbeenvariablydefinedasatotalleukemiabloodcellcountgreaterthan50-100x109/L(100,000/microL)..INTRODUCTIONINTRODUCTIONleukostasisalsocalledsymptomatichyperleukocytosisamedicalemergency,apathologicdiagnosistheone-weekmortalityrateisapproximately20to40percent.EPIDEMIOLOGYleukemiatypeandpatientpopulation.large,poorlydeformableblastsininfants,agesof10and20years,males,Tcellphenotype病因端粒酶ATRT，三氧化二砷机理1.白细胞可塑性小，变性能力差，过高的白细胞在微循环中大量淤滞，导致血流减慢，血液粘滞度增高，特别易在脑、肺、肾、腹腔血管梗塞，预后很差。2.白血病细胞耗氧量高，导致组织缺氧，加之白血病细胞浸润破坏血管壁致脏器出血、水肿，更由于血小板计数减少和大量白血病细胞崩解释放出促凝血物质，极易形成DIC。SIGNSANDSYMPTOMSthemainclinicalsymptomsofleukostasisandcausesofearlydeatharerelatedtoinvolvementofthecentralnervoussystem(40%)lungs(30%)Pulmonarysignsandsymptomsdyspneaandhypoxiawithorwithoutdiffuseinterstitialoralveolarinfiltratesonimagingstudies.MeasurementofthearterialpO2canbefalselydecreasedsincetheWBCsinthetesttubeutilizeoxygen.PulseoximetryprovidesamoreaccurateassessmentofO2saturationinthissetting.Neurologicalsignsandsymptomsvisualchanges,headache,dizziness(头晕),tinnitus（耳鸣）,gaitinstability,confusion,somnolence（嗜睡）,and,occasionally,coma（昏迷）.anincreasedriskofintracranialhemorrhagethatpersistsforatleastaweekafterthereductionofwhitecellcount（reperfusioninjury）noncontrastedCTorMRIisindicatedinpatientswithneurologicabnormalities.cautiousaboutusingintravenouscontrastdyeatatimewhenrenalfunctionmaybecompromisedbyleukostasisortumorlysissyndrome,anddehydration.fever（80%）inflammationassociatedwithleukostasisconcurrentinfection.treatempiricallyforinfectioninallsuchpatientsLesscommonsignsorsymptoms：

electrocardiographicsignsofmyocardialischemiaorrightventricularoverloadworsening