Quality in Laboratory Hemostasis and Thrombosis, Second Edition PDF
Preview Quality in Laboratory Hemostasis and Thrombosis, Second Edition
Quality in Laboratory Hemostasis and Thrombosis Quality in Laboratory Hemostasis and Thrombosis Second Edition Editedby Steve Kitchen, PhD ClinicalScientist SheffieldHemophiliaandThrombosisCentre RoyalHallamshireHospital Sheffield;and ScientificDirector,UKNationalExternalQualityAssessmentScheme(NEQAS) forBloodCoagulation ScientificDirector,WHOandWFHInternationalExternalQualityAssessment ProgramsforBloodCoagulation Sheffield,UK John D. Olson, MD, PhD ProfessorandViceChairforClinicalAffairs DepartmentofPathology UniversityofTexasHealthScienceCenter;and DirectorofClinicalLaboratories UniversityHealthSystem SanAntonio,TX,USA F. Eric Preston, MD, FRCPath, FRCP EmeritusProfessorofHematology UniversityofSheffield Sheffield;and Director,WHOandWFHInternationalExternalQualityAssessmentPrograms forBloodCoagulation Sheffield,UK ForewordbyProfessorDrFritsR.Rosendaal A John Wiley & Sons, Ltd., Publication Thiseditionfirstpublished2013(cid:2)C 2009(cid:2)C BlackwellPublishingLtd;2013byJohnWiley&Sons,Ltd Wiley-BlackwellisanimprintofJohnWiley&Sons,formedbythemergerofWiley’sglobalScientific, TechnicalandMedicalbusinesswithBlackwellPublishing. Registeredoffice: JohnWiley&Sons,Ltd,TheAtrium,SouthernGate,Chichester,WestSussex, PO198SQ,UK Editorialoffices: 9600GarsingtonRoad,Oxford,OX42DQ,UK TheAtrium,SouthernGate,Chichester,WestSussex,PO198SQ,UK 111RiverStreet,Hoboken,NJ07030-5774,USA Fordetailsofourglobaleditorialoffices,forcustomerservicesandforinformationabouthowtoapply forpermissiontoreusethecopyrightmaterialinthisbookpleaseseeourwebsiteat www.wiley.com/wiley-blackwell Therightoftheauthortobeidentifiedastheauthorofthisworkhasbeenassertedinaccordancewith theUKCopyright,DesignsandPatentsAct1988. Allrightsreserved.Nopartofthispublicationmaybereproduced,storedinaretrievalsystem,or transmitted,inanyformorbyanymeans,electronic,mechanical,photocopying,recordingor otherwise,exceptaspermittedbytheUKCopyright,DesignsandPatentsAct1988,withouttheprior permissionofthepublisher. Designationsusedbycompaniestodistinguishtheirproductsareoftenclaimedastrademarks.All brandnamesandproductnamesusedinthisbookaretradenames,servicemarks,trademarksor registeredtrademarksoftheirrespectiveowners.Thepublisherisnotassociatedwithanyproductor vendormentionedinthisbook.Thispublicationisdesignedtoprovideaccurateandauthoritative informationinregardtothesubjectmattercovered.Itissoldontheunderstandingthatthepublisheris notengagedinrenderingprofessionalservices.Ifprofessionaladviceorotherexpertassistanceis required,theservicesofacompetentprofessionalshouldbesought. Thecontentsofthisworkareintendedtofurthergeneralscientificresearch,understanding,and discussiononlyandarenotintendedandshouldnotberelieduponasrecommendingorpromotinga specificmethod,diagnosis,ortreatmentbyphysiciansforanyparticularpatient.Thepublisherandthe authormakenorepresentationsorwarrantieswithrespecttotheaccuracyorcompletenessofthe contentsofthisworkandspecificallydisclaimallwarranties,includingwithoutlimitationanyimplied warrantiesoffitnessforaparticularpurpose.Inviewofongoingresearch,equipmentmodifications, changesingovernmentalregulations,andtheconstantflowofinformationrelatingtotheuseof medicines,equipment,anddevices,thereaderisurgedtoreviewandevaluatetheinformationprovided inthepackageinsertorinstructionsforeachmedicine,equipment,ordevicefor,amongotherthings, anychangesintheinstructionsorindicationofusageandforaddedwarningsandprecautions.Readers shouldconsultwithaspecialistwhereappropriate.ThefactthatanorganizationorWebsiteisreferred tointhisworkasacitationand/orapotentialsourceoffurtherinformationdoesnotmeanthatthe authororthepublisherendorsestheinformationtheorganizationorWebsitemayprovideor recommendationsitmaymake.Further,readersshouldbeawarethatInternetWebsiteslistedinthis workmayhavechangedordisappearedbetweenwhenthisworkwaswrittenandwhenitisread.No warrantymaybecreatedorextendedbyanypromotionalstatementsforthiswork.Neitherthe publishernortheauthorshallbeliableforanydamagesarisingherefrom. LibraryofCongressCataloging-in-PublicationData Qualityinlaboratoryhemostasisandthrombosis/editedbySteveKitchen,JohnD.Olson, F.EricPreston;forewordbyFritsR.Rosendaal.–2nded. p.;cm. Includesbibliographicalreferencesandindex. ISBN978-0-470-67119-1(hardback:alk.paper) I.Kitchen,Steve,Dr.II.Olson,JohnDavid,1944-III.Preston,F.E. [DNLM:1.HemostaticTechniques. 2.Anticoagulants. 3.BloodCoagulationDisorders, Inherited–diagnosis. 4.BloodCoagulationFactors. 5.ClinicalLaboratoryTechniques. 6.Thrombophilia–diagnosis.WH310] 616.1(cid:3)57075–dc23 2012044510 AcataloguerecordforthisbookisavailablefromtheBritishLibrary. Wileyalsopublishesitsbooksinavarietyofelectronicformats.Somecontentthatappearsinprint maynotbeavailableinelectronicbooks. Coverimage:Internal.Top:courtesyofBertVerbuggen;bottom:courtesyofWayneChandler. CoverdesignbyAndrewMageeDesignLtd Setin9/11.5ptSabonbyAptara®Inc.,NewDelhi,India 1 2013 Contents Contributors,vii Foreword,x Preface,xii SteveKitchen,JohnD.Olson,&F.EricPreston Part 1: General Quality Program 1 Generalqualityplanninginthehemostasislaboratory,3 JohnD.Olson 2 Hemostasistestvalidation,performance,andreferenceintervals: internationalrecommendationsandguidelines,12 RichardA.Marlar 3 Causesoferrorsinmedicallaboratories,22 GiuseppeLippi&EmmanuelJ.Favaloro 4 Internationalstandardsinhemostasis,32 TrevorW.Barrowcliffe&AnthonyR.Hubbard 5 Sampleintegrityandpreanalyticalvariables,45 Dorothy(Adcock)Funk 6 Internalqualitycontrolinthehemostasislaboratory,57 SteveKitchen,F.EricPreston,&JohnD.Olson 7 Externalqualityassessmentinhemostasis:itsimportanceandsignificance,65 F.EricPreston,SteveKitchen,&AlokSrivastava 8 Theuniquechallengesofhemostatictestinginchildren,77 M.PatriciaMassicotte,MaryE.Bauman,VanessaChan,& AnthonyK.C.Chan Part 2: Quality in Coagulation Testing 9 Initialevaluationofhemostasis:reagentandmethodselection,95 WayneL.Chandler 10 AssayoffactorVIIIandotherclottingfactors,105 SteveKitchen&F.EricPreston 11 Applicationofmoleculargeneticstotheinvestigationofinherited bleedingdisorders,115 StefanLethagen,MortenDunø,&LarsBoNielsen 12 Detectingandquantifyingacquiredfunctionalinhibitorsinhemostasis,124 BertVerbruggen,MyriamDardikh,&BrittaLaros-vanGorkom v CONTENTS 13 Standardizationofd-dimertesting,136 GuidoReber&PhilippedeMoerloose 14 Point-of-caretestinginhemostasis,147 ChrisGardiner,DianneKitchen,SamuelMachin,&IanMackie Part 3: Quality in Testing for Platelet Function and von Willebrand Disease 15 Diagnosticassessmentofplateletfunction,159 PaquitaNurden,AlanNurden,&MartineJandrot-Perrus 16 Laboratoryevaluationofheparin-inducedthrombocytopenia,174 Theodore(Ted)E.Warkentin&JaneC.Moore 17 LaboratoryevaluationofvonWillebranddisease:phenotypicanalysis,192 EmmanuelJ.Favaloro 18 LaboratoryanalysisofvonWillebranddisease:molecularanalysis,204 AnneC.Goodeve&IanR.Peake Part 4: Quality in Thrombophilia Testing and Monitoring Anticoagulation 19 Qualityissuesinheritablethrombophiliatesting,219 IsobelD.Walker&IanJennings 20 Evaluationofantiphospholipidantibodies,233 MichaelGreaves 21 Monitoringheparintherapy,244 MarilynJohnston 22 MonitoringoralanticoagulanttherapywithvitaminKantagonists,253 ArmandoTripodi 23 Monitoringnewanticoagulants,264 ElaineGray&TrevorW.Barrowcliffe Index,273 vi Contributors Trevor W. Barrowcliffe MA,PhD Morten Dunø MD,PhD FormerlyNationalInstituteforBiologicalStandards DepartmentofClinicalGenetics andControl(NIBSC) CopenhagenUniversityHospital(Rigshospitalet) PottersBar,Hertfordshire,UK Copenhagen,Denmark Mary E. Bauman RN,MN,NP Emmanuel J. Favaloro PhD,FFSc(RCPA) StolleryChilden’sHospital DepartmentofHematology UniversityofAlberta ICPMR,WestmeadHospital Edmonton,AB,Canada Westmead,NSW,Australia Anthony K.C. Chan MBBS,FRCPC,FRCPath Dorothy (Adcock) Funk MD ColoradoCoagulation McMasterChildren’sHospital/HamiltonHealth abusinessunitofEsoterix,Inc.,Englewood,CO, SciencesFoundation USA ChairinPediatricThrombosisandHemostasis DepartmentofPediatrics,McMasterUniversity Chris Gardiner PhD Hamilton,ON,Canada HaematologyDepartment UniversityCollegeLondonHospitalsNHSTrust;and Vanessa Chan BSc,MLT NuffieldDepartmentofObstetricsandGynaecology DepartmentofPaediatricLaboratoryMedicine JohnRadcliffeHospital TheHospitalforSickChildren Oxford,UK Toronto,ON,Canada Anne C. Goodeve PhD Wayne L. Chandler MD SheffieldHaemostasisResearchGroup CoagulationLaboratory DepartmentofCardiovascularScience DepartmentofPathologyandGenomicMedicine UniversityofSheffield TheMethodistHospitalPhysicianOrganization Sheffield;and Houston,TX,USA SheffieldDiagnosticGeneticsService SheffieldChildren’sNHSFoundationTrust Myriam Dardikh MSc Sheffield,UK DepartmentofLaboratoryMedicine LaboratoryofHaematology Elaine Gray PhD RadboudUniversityNijmegenMedicalCentre NationalInstituteforBiologicalStandardsand Nijmegen,TheNetherlands Control PottersBar,Hertfordshire,UK Philippe de Moerloose MD HaemostasisUnit Michael Greaves MDFRCPFRCPath DepartmentofInternalMedicine AberdeenRoyalInfirmary UniversityHospitalandFacultyofMedicine HeadofCollegeofLifeSciencesandMedicine, Geneva,Switzerland UniversityofAberdeen,Scotland,UK vii CONTRIBUTORS Anthony R. Hubbard PhD Giuseppe Lippi MD NationalInstituteforBiologicalStandardsand ClinicalChemistryandHaematologyLaboratory Control DepartmentofPathologyandLaboratoryMedicine Hertfordshire,UK AcademicHospitalofParma Parma,Italy Martine Jandrot-Perrus MD,PhD Samuel Machin MBChB,FRCP,FRCPath INSERMU698andParis7University HaematologyDepartment Paris,France UniversityCollegeLondonHospitalsNHSTrust;and HaemostasisResearchUnit,HaematologyDepartment Ian Jennings PhD UniversityCollegeLondon UKNEQASforBloodCoagulation London,UK Sheffield,UK Ian Mackie PhD,FRCPath Marilyn Johnston MLT,ART HaematologyDepartment HemostasisReferenceLaboratory UniversityCollegeLondonHospitalsNHSTrust Hamilton,ON,Canada HaemostasisResearchUnit HaematologyDepartment Dianne Kitchen FIBMS UniversityCollegeLondon London,UK UKNEQASBloodCoagulation Sheffield,UK Richard A. Marlar PhD PathologyandLaboratoryMedicine Steve Kitchen PhD OklahomaCityVAMedicalCenter;and SheffieldHemophiliaandThrombosis DepartmentofPathology Centre UniversityofOklahomaHealthSciencesCenter RoyalHallamshireHospital OklahomaCity,OK,USA Sheffield;and UKNationalExternalQualityAssessmentScheme M. Patricia Massicotte MD,MHSc (NEQAS)forBloodCoagulation StolleryChilden’sHospital WHOandWFHInternationalExternal UniversityofAlberta QualityAssessmentProgramsforBlood Edmonton,AB,Canada Coagulation Sheffield,UK Jane C. Moore ART,BSc DepartmentsofPathologyandMolecularMedicine, Britta Laros-van Gorkom MD,PhD andMedicine MichaelG.DeGrooteSchoolofMedicine,McMaster DepartmentofHaematology University RadboudUniversityNijmegenMedical HamiltonRegionalLaboratoryMedicineProgram Centre Hamilton,ON,Canada Nijmegen,TheNetherlands Lars Bo Nielsen MD,PhD Stefan Lethagen MD,PhD DepartmentofClinicalBiochemistry CopenhagenUniversity RigshospitaletandDepartmentofBiomedical Copenhagen;and Sciences;and MedicalandScience UniversityofCopenhagen HaemophiliaR&D CopenhagenUniversityHospital(Rigshospitalet) NovoNordiskA/S,Denmark Copenhagen,Denmark viii CONTRIBUTORS Alan Nurden PhD Alok Srivastava MD,FRACP,FRCPA,FRCP CentredeRe´fe´rencedesPathologiesPlaquettaires DepartmentofHematology PlateformeTechnologiqued’InnovationBiome´dicale ChristianMedicalSchool HoˆpitalXavierArnozan Vellore,India Pessac,France Armando Tripodi PhD Paquita Nurden MD,PhD AngeloBianchiBonomiHemophiliaandThrombosis CentredeRe´fe´rencedesPathologiesPlaquettaires Center PlateformeTechnologiqued’InnovationBiome´dicale DepartmentofInternalMedicine HoˆpitalXavierArnozan UniversitySchoolofMedicineandIRCCSCa` Pessac,France GrandaMaggioreHospitalFoundation Milan,Italy John D. Olson MD,PhD DepartmentofPathology Bert Verbruggen PhD UniversityofTexasHealthScienceCenter LaboratoryofClinicalChemistryandHaematology SanAntonio,TX,USA JeroenBoschHospital’s-Hertogenbosch, TheNetherlands Ian R. Peake PhD SheffieldHaemostasisResearchGroup Isobel D. Walker MDMPhil(MedicalLaw), DepartmentofCardiovascularScience FRCPath UniversityofSheffield UniversityofGlasgow,Glasgow;and Sheffield,UK NEQASforBloodCoagulation Sheffield,UK F. Eric Preston MD,FRCPath,FRCP UniversityofSheffield Theodore (Ted) E. Warkentin MD Sheffield;and DepartmentsofPathologyandMolecularMedicine, WHOandWFHInternationalExternalQuality andMedicine AssessmentProgramsforBloodCoagulation MichaelG.DeGrooteSchoolofMedicine Sheffield,UK McMasterUniversity TransfusionMedicine Guido Reber PhD HamiltonRegionalLaboratoryMedicineProgram HaemostasisUnit Hamilton,ON,Canada DepartmentofInternalMedicine UniversityHospitalandFacultyofMedicine Geneva,Switzerland ix Foreword Thouartalwaysfiguringdiseasesinme,butthou developmentinimprovingaccuracyandcomparabil- artfulloferror:Iamsound ity of hemostasis laboratory tests. Here, the Scien- (WilliamShakespeare.Measurefor tific and Standardization Committee of the Interna- measure(1604);ActI,SceneII) tionalSocietyonThrombosisandHaemostasis,work- ingtogetherwiththeWorldHealthOrganization,has A correct diagnosis is the cornerstone of medicine. playedamajorrole.Overtheyearswehavewitnessed Withoutit,noremedycanbeprescribed,orprognosis theemergenceoflargeexternalqualityassurancepro- given.Althoughlaboratorytestsareonlyapartofthe grams,inwhichsamplesaresometimessenttomore diagnosticarsenal,togetherwithhistorytaking,clin- thanathousandparticipatinglaboratories.Suchpro- ical examination, and imaging techniques, few diag- grams not only allow laboratories to evaluate their nosesarearrivedatwithoutsomeformoflaboratory performance, but also to group results by reagent or test.Inadequatetestsmayleadtoeitherfalsereassur- instrument,whichleadstovaluableinsights,andfur- ance or false alarm. They may lead to the erroneous ther quality improvement. Newly added chapters to choicenottogivetreatmentwhentreatmentwouldbe the second edition deal with the causes of labora- beneficial, or even to prescribe the wrong treatment, tory error, the understanding of which is indispens- which is likely to be harmful. It is therefore of the able in optimizing laboratory performance, and the utmostimportancethatwheneverlaboratorytestsare performance and interpretation of hemostatic tests performed,theresultsarereliable. inchildren. Laboratory tests in the field of thrombosis and Inthesecondpartofthebook,Chapters9through hemostasis are notoriously difficult, which is related 23,adetaileddescriptionisgivenofallmajorassays to the large variety in techniques that are used, and in hemostasis, grouped in a series of chapters on the sensitivity of many assays to small preanalytical coagulation factor assays, on primary hemostasis andanalyticalvariation.Therefore,qualityassurance (plateletsandvonWillebrandfactor),andonthrom- iscrucial,andnohemostasislaboratorycanaffordnot bophiliatestingandanticoagulanttreatmentmonitor- toinvestininternalandexternalqualitycontrol.The ing. These chapters give the reader invaluable infor- book,QualityinLaboratoryHemostasisandThrom- mationontheperformanceandinterpretationofthese bosis, edited and written by authorities in the field, tests.Anewlyaddedchapterthatwasmuchmissedin sinceitsfirsteditionin2008,hasbecomeaindispens- thefirsteditiondealswithheparin-inducedthrombo- able help for those who wish to set up a hemostasis cytopenia. laboratory,aswellasthosewhoalreadyworkinsuch Theultimatetestforalaboratorytestiswhetherit aplace.For,toquotefromthefirstchapter:“Process improvesmedicalcare,thatis,reducesmorbidityand isneveroptimized;itcanalwaysbeimproved.” mortality, which depends on the effect a negative or The book has two parts: the first eight chapters positive test result has on the treatment of a patient. giveascholarlyoverviewoftheconceptsthatunder- A test that does not affect clinical management is a liequalityassurance,explainingthevariousaspectsof wasteofresources.Bothatthebeginningandtheend test validation, with its components, of which accu- of laboratory tests there is usually a clinician, who racyandprecisionarethemostimportant:doesatest first makes the decision to order a test, and subse- measure what it is supposed to measure, and does quentlyhastointerpretthetestresult.Althoughthese it do so with acceptable reproducibility. Subsequent clinical decisions and interpretation are not part of chaptersinthisfirstpartexplainindetailhowinter- the content of this book, which would have made it nal quality control deals with precision and external unwieldytosaytheleast,theseareofobviousimpor- quality control with accuracy. The development of tance, and one of the tasks of the individuals work- international standards is an important and ongoing inginhemostasislaboratoriesistoeducateclinicians x FOREWORD about the clinical value of the various assays. I am evant, since the purpose is to discriminate between quite confident that in the field of hemostasis and levels of over 150 or 200 IU/dL versus plasma con- thrombosis more useless than useful testing is done, centrations around 100 IU/dL. The same error in a andthatinmedicineasawholethegreatestwasteof factor VIII assay to diagnose hemophilia A could be money is on redundant diagnostics. The practice of disastrous. medicineknowsawidevarietyoftests,whichgener- Aclinician,whenorderingatest,willhavetodeal allyservethreepurposes,eithertodiagnoseadisease, with so-called prior probabilities, which is of partic- or to test for a risk factor for disease, or to screen ularrelevanceinscreeningtests.Aslightlyprolonged for either of these. This distinction is rarely sharply aPTT has a vastly different meaning when found in made, while it seems that clinically one type (diag- a healthy woman who had four uneventful deliver- nosingadisease)isalmostalwaysindicatedanduse- ieswhohascometothehospitalforatuballigation, ful, and another type (testing for risk factors) only thaninan18-month-oldboywhoneedstoundergoa rarelyis.Whileitislogicaltofindoutwhichdisease duodenoscopy with possible biopsies. She is unlikely a patient with complaints has, it is not so logical to to have a bleeding tendency, even when the aPTT tryandidentifythecausesofthatdisease,orevento is prolonged, while the young boy may suffer from tryandidentifythoseriskfactorsinnondiseasedindi- hemophilia. Screening tests affect the likelihood of viduals, such as relatives of individuals with throm- disease, which, according to Bayes’ theorem, is also bosis.Thereasonthedistinctionbetweendiagnosing a function of the prior probability of disease. Virtu- a disease and identifying a risk factor is not always ally,allteststhatusereferencerangesbasedonstatis- sharplymade,ispossiblybecauseinsomediseasesin ticalcutoffvalues,suchasthepopulationmeanplus thefield,notablybleedingdisorders,thereisanalmost orminustwostandarddeviations,arescreeningtests, one-to-onerelationshipbetweenthecauseofthedis- that do neither establish a risk factor or a disease, ease and the disease itself. While excessive bleeding butonly,whenabnormal,affectthelikelihoodofthat is the disease and the clotting factor level a cause, state. Nature does not use standard deviations, and individuals with no factor VIII or IX will invariably usingacutoffoftwostandarddeviationsbydefinition havetheclinicaldiseaseofhemophilia,andtherefore, finds 2.5% of the population below, or over, such a measuringtheclottingfactorlevelhasbecomesynony- cutoff. In reality, diseases and risk factors may have moustodiagnosinghemophilia.Thisisquitedifferent prevalencesthatexceed,or,moreusual,liefarbelow forthrombosis.Thrombosis(deepveinthrombosisor thisfigure.Testsusing“normalranges”thereforecan pulmonary embolism) is a disease, whereas throm- never establish an abnormality, and should be fol- bophilia is not. Given the multicausal nature of the lowed by more specific tests, such as clotting factor etiologyofthrombosis,inwhichmultipleriskfactors assaysorgenetictests. need to be present to lead to disease, it is far from Over the last decades, major progress has been self-evidentthattestingforthrombophilicabnormali- made in quality assurance of hemostatic laboratory tieshasanyclinicalvalue.Sofar,therearenoclinical assays. In this new edition of Quality in Labora- studies that show a benefit of such testing, although tory Hemostasis and Thrombosis, all chapters have itisperformedonabroadscale.Wheneveryouorder been updated and several new chapters have been a test or are requested to perform a test, question added. This book will remain an indispensable part whethertheresultcouldpossiblychangeanything.If ofeveryhemostasislaboratory,where,givenitshand- not, or if the only benefit is to satisfy the doctor’s onnature,itwillrarelysittogetdustyontheshelves. curiosity,thetestshouldnotbedone. The reliability of a particular assay should be FritsR.Rosendaal viewed in the context in which the test is ordered. FormerChairmanISTHCouncil,PresidentXXIV Suppose one would order a test for high factor VIII ISTHCongress,FormerChairmanNetherlands as a prothrombotic risk factor, the above mentioned SocietyofThrombosis&Haemostasis,Leiden notwithstanding,anerroroffiveIU/dLwouldbeirrel- UniversityMedicalCentre,TheNetherlands xi
The list of books you might like
