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张志升台湾台大医院输血医学科
2016/6/26
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Content
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
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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。
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Evaluationofachildwiththrombocytopenia
Plateletcount<150,000cells/uL,age>3mouths
CBC,bloodsmearevaluation
Anemia+thrombocytopenia
pancytopenia
Plateletclumpspresent
pseudothrombocytopenia
Illappearing?
No
Congenitalanomalies?
Yes
No
PMNhypersegmentationRBCmacroovalocytosis?↓B12or↓RBCfolate
B12orfolatedeficiency
MedicationsImmunizationsIrradiationToxins?
No
Yes
Macrothrombocytes
Drug-inducesLiveimmunizationIrradiationToxins
Yes
No
Othermorphologicplateletchanges
Nootherplateletchange
Bonemarrow
CyanoticheartdiseaseFanconianemiaDyskeratosiscongenitaTrisomy13or18Syndromes:Kasabach-MerrittTARAlportvariants
Syndromes:
May-hegglinHermansky-PudiakGrayplatelet
ITPHereditarythrombocytopeniaBernard-Soulier
↑NImegakaryocytes
↓megakaryocytes
LeukemiaAplasticanemiaDrug-inducedAmegakaryocyticthrombocytopeniaMyelodysplasia
ITPisadiagnosisofexclusionResponsetotherapy,ifneeded(corticosteroid,IVIG,anti-Dantibody),confirmsthediagnosis
Yes
PTT,PT,TT
prolonged
DICR/Osepsis
SeeConsumptionalcoagulopathy
Normal
↑↑SpleenSignsofportalhypertensionplatelet>50,000+/-pancytopenia
MaleEczemaRecurrentinfectionSmallplatelets
LymphadenopathyHepatosplenomegalySuperiorvenacavasyndromeAbdominalmass
Chronicallyillappearing
Acute,fibrileillness
WBCenzymeassaysUltrasonographyThicksmear
Biopsyoflymphnode,massorbonemarrowconsidertumorlysisandsuperiorvenacavasyndromes
HIVassayANAU/ARenalfunction
Bloodculture?antibiotics
MalariaGaucherdiseasePortalhypertensionHepaticschistosomiasisCavernoustransformationoftheportalvein
Wiskott-Aldrichsyndrome
Lymphoma:HodgkinNon-HodkinNeuroblastomaleukemiaMyelodysplasia
HIVAutoimmuneorconnectivetissuediseaseHUS/TTP+othermicroangiopathiesProstheticcardiacvalve
R/OADMAT-13DATAuto/alloanti-plateletantibody
SepsisVaricellaEBVCMVDenquehemorrhagicfeverHIVHUSHantavirusParvovirusOthervirusesTTP
Auto/allanti-plateletantibodiesstudy
Heparin-inducedthrombocytopenia
CheckPF4
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免疫性血小板低下
Neonatalalloimmunethrombocytopenia(NAIT)
Platelettransfusionrefractoriness(PTR)
Post-transfusionthrombocytopenicpurpura(PTP)
Passivealloimmunethrombocytopenia(PAT)
Transplantation-associatedalloimmunethrombocytopenia(TAATP)
Alloantigensimplicatedinalloimmunethrombocytopenia
in24thregionalcongressofISBT
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即输血小板两次以上无法达到预期血小板的增加数称为“血小板输注无疗效”
成因:异体免疫,自体抗体,ABO血型不符,病人因素(如急性出血或组织移植排斥..等),药物治疗(如抗生素vanycin..)所引起
血小板输血后的品质评估:1小时后其“校正血小板增加数”>7000,此方法亦为侦测有无“异体免疫”的间接方式
CCI:(输血后血小板数-输血前血小板数)XBSA/输注血小板量。
血小板输注无效症
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Content
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
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SpecificityofHPAAbinNAIT(Japan)
in24thregionalcongressofISBT
TheriskofICHwasthehighestwithanti-HPA-3
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2013ISBTIPIWP-AR
TheriskofICHwasthehighestwithanti-HPA-3
Anti-HPA-4a/4bisthehighestrateinNAITinJapan(anti-HPA-4a7%,anti-HPA-4b52%)
HPA-4inpatiblemaycauserejectionrectionreactionsinsolidorgantransplantation.(VoxSanguinis,2010july,Supplement1,Vol99)
%ofCD36typeIdeficientindividualsareatrisktobeimmunizedthroughbloodtransfusionorpregnancyinChina.
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新生儿免疫血小板低下症(NAIT)
NAITduetoanti-HLAantibodies
CaseofNAITsuspectedlyduetoanti-HLAarereported,buttheassociationneedstobefirmed
ClassIHLAAbsarefoundinaboutonethirdofmultiparouswomen(15~31%),andanti-HPAAbslessfrequently;however,plateletdestructionisusuallycausedbytheanti-HPAAbs.
Protectiveimmunemechanismoftheplacenta:anti-HLAantibodiesadsorbedbythestromalcellsofplacentaexpressingpaternalantigens;routinely,theinfantsarebornwithnormalplateletcounts.
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