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CoagulationforDentistsThrombogenesisHematology:BasicPrinciplesandPractice,Hoffmanet.al.ChurchillLivingston,2000RegulationofCoagulationStandardLaboratoryTestsTheCaseofMr.HagemanFitzgeraldFletcherTheCaseofMr.HagemanFitzgeraldFletcherContactfactorsContactFactorDeficiencySeveralfactorsareinvolvedincontactactivationoftheintrinsicpathwayFactorXII(Hageman)autoactivateswhenexposedtoanegativelychargedsurfacePrekallikrein(Fletcher)andHighMolecularWeightKininogen(Fitzgerald)areallinolvedAcuriousity,inthatallthreecanresultinmarkedprolongationoftheaPTTwithoutclinicaleffectFletcher,thefirstdescribedpatient,actuallyhadapulmonaryembolusTheCaseofMr.HagemanFitzgeraldFletcherTheCaseofMissPenelopevonWillebrandTheCaseofMissPenelopevonWillebrandTheCaseofMissPenelopevonWillebrandvWFcirculateswithFVIIIandbindswithplateletVonWillebrandsDiseaseOneofthefewnamedafterthedoctor,insteadofthepatientGeneticdisorder,qualitativeorquanitativedeficiencyoflargeglycoproteinvWFVonWillebrandFactorDisulfidebondedpolymersof220kDproteinMadeinendothelialcells,storedthereandinalphagranulesoftheplateletChromosome12Bindingsiteonthemoleculeforcollagen,heparin,plateletglycoprotiein(GP)Ib,areasforristocetaninducedplateletaggregationThreedifferentsubtypes(quantitativeI,qualitativeII,andsevereIII)withsub-subtypes,A,B,C,..A1,A2…etc.Genetics1%ofpopulationhavesometypeofdefectOfthese,80%typeIA:noconsistentgeneticabnormalitywhile15-20%typeIIlocalizedtoexon28,GPIbbindingdomainVeryraretypeIIIisgenedeletion(homozygous)ManagementDDAVP(exceptintypeIIB,contraindicated,ortypeIII,ineffective)DDAVPraisesfactorVIIIandvWF3-5xTestaheadoftimetoensurelevelsoffactorVIII,vWF,bleedingtimeHumate-PManagementbyDentists:CallthehaematologistTheCaseofMissPenelopevonWillebrandTheCaseofMr.HemaFoeliaGoldman:CecilTextbookofMedicine,21sted.,Copyright©2000W.B.SaundersCompanyTheCaseofMr.HemaFoeliaHemophiliaA:deficiencyinFVIIIHaemophiliaA(andalittlebitaboutB)320kDprotein,madeinliverStablecomplexwithvWF(muchlarger,itisapolymer
