Crash Course Haematology and Immunology PDF
Preview Crash Course Haematology and Immunology
Haematology and Immunology First, second and third edition authors: Saimah Arif Arjmand Mufti James Griffin Gareth Kitchen 4 th Edition CRASH COURSE SERIES EDITOR Dan Horton-Szar BSc(Hons), MBBS(Hons), MRCGP NorthgateMedicalPractice, Canterbury, Kent, UK FACULTY ADVISORS Caroline Shiach BSc(Hons), MBChB,MD, FRCPath, FRCP Consultant Haematologist, University Department of Haematology, Wythenshawe Hospital, South ManchesterUniversity Hospitals NHS Trust, Manchester, UK Matthew Helbert MBShB,FRCP, FRCPath, PhD Consultant Immunologist, ManchesterRoyalInfirmary, Manchester, UK Haematology and Immunology Yousef Gargani Medical Student Manchester Medical School Manchester, UK Edinburgh London New York Oxford Philadelphia St Louis Sydney Toronto 2012 ©2012ElsevierLtd.Allrightsreserved. Nopartofthispublicationmaybereproducedortransmittedinanyformorbyanymeans,electronicor mechanical,includingphotocopying,recording,oranyinformationstorageandretrievalsystem, withoutpermissioninwritingfromthepublisher.Detailsonhowtoseekpermission,furtherinformation aboutthePublisher’spermissionspoliciesandourarrangementswithorganizationssuchasthe CopyrightClearanceCenterandtheCopyrightLicensingAgency,canbefoundatourwebsite: www.elsevier.com/permissions. ThisbookandtheindividualcontributionscontainedinitareprotectedundercopyrightbythePublisher (otherthanasmaybenotedherein). Firstedition1998 Secondedition2003 Thirdedition2007 Reprinted,2008,2010(twice) Fourthedition2012 ISBN:9780723436256 BritishLibraryCataloguinginPublicationData AcataloguerecordforthisbookisavailablefromtheBritishLibrary LibraryofCongressCataloginginPublicationData AcatalogrecordforthisbookisavailablefromtheLibraryofCongress Notices Knowledgeandbestpracticeinthisfieldareconstantlychanging.Asnewresearchandexperience broadenourunderstanding,changesinresearchmethods,professionalpractices,ormedicaltreatment maybecomenecessary. Practitionersandresearchersmustalwaysrelyontheirownexperienceandknowledgeinevaluating andusinganyinformation,methods,compounds,orexperimentsdescribedherein.Inusingsuch informationormethodstheyshouldbemindfuloftheirownsafetyandthesafetyofothers,including partiesforwhomtheyhaveaprofessionalresponsibility. Withrespecttoanydrugorpharmaceuticalproductsidentified,readersareadvisedtocheckthemost currentinformationprovided(i)onproceduresfeaturedor(ii)bythemanufacturerofeachproductto beadministered,toverifytherecommendeddoseorformula,themethodanddurationof administration,andcontraindications.Itistheresponsibilityofpractitioners,relyingontheirown experienceandknowledgeoftheirpatients,tomakediagnoses,todeterminedosagesandthebest treatmentforeachindividualpatient,andtotakeallappropriatesafetyprecautions. Tothefullestextentofthelaw,neitherthePublishernortheauthors,contributors,oreditors,assume anyliabilityforanyinjuryand/ordamagetopersonsorpropertyasamatterofproductsliability, negligenceorotherwise,orfromanyuseoroperationofanymethods,products,instructions,orideas containedinthematerialherein. CommissioningEditor:JeremyBowes DevelopmentEditor:CatherineJackson ProjectManager:AndrewRiley Designer/DesignDirection:StewartLarking IllustrationManager:BruceHogarth IconIllustrations:GeoParkin The Publisher's policy is to use paper manufactured from sustainable forests PrintedinChina Series editor foreword TheCrashCourseseriesfirstpublishedin1997andnow,15yearson,wearestill goingstrong.Medicineneverstandsstill,andtheworkofkeepingthisseries relevantfortoday’sstudentsisanongoingprocess.Thesefourtheditionsbuildon thesuccessoftheprevioustitlesandincorporatenewandrevisedmaterial,tokeep theseriesup-to-datewithcurrentguidelinesforbestpractice,andrecent developmentsinmedicalresearchandpharmacology. Wealwayslistentofeedbackfromourreaders,throughfocusgroupsandstudent reviewsoftheCrashCoursetitles.Forthefourtheditionswehavecompletely re-writtenourself-assessmentmaterialtokeepupwithtoday’s‘single-bestanswer’ and‘extendedmatchingquestion’formats.Theartworkandlayoutofthetitleshas alsobeenlargelyre-workedtomakeiteasierontheeyeduringlongsessionsof revision. Despitefullyrevisingthebookswitheachedition,weholdfasttotheprinciples onwhichwefirstdevelopedtheseries.CrashCoursewillalwaysbringyouallthe informationyouneedtoreviseincompact,manageablevolumesthatintegrate basicmedicalscienceandclinicalpractice.Thebooksstillmaintainthebalance betweenclarityandconciseness,andprovidesufficientdepthforthoseaimingat distinction.Theauthorsaremedicalstudentsandjuniordoctorswhohaverecent experienceoftheexamsyouarenowfacing,andtheaccuracyofthematerialis checkedbyateamoffacultyadvisorsfromacrosstheUK. Iwishyouallthebestforyourfuturecareers! DrDanHorton-Szar v Prefaces Author Haematologyandimmunologyaretwowordsthataresuretostrikefearintomost medicalstudents.Theybothhavethereputationofbeingcomplex,lab-based specialitiesthatlosetheirimportanceoncewereachthe‘realworld’.Inreality, knowledgeofbothhaematologyandimmunologyareimmenselyimportantforthe practiceofallspecialities,bothclinicalandlab-based.Thisincludestopicsranging fromanaemia,whichisencounteredbyallclinicians;tonon-steroidal anti-inflammatorydrugs,whichareprescribedbyjuniordoctorsthroughout theworld. InCrashCourse:HaematologyandImmunologywehavemadeeveryeffortto demonstratethesoundphysiologicalpriniciplesthatunderpinthenew developmentsinbothspecialities.Thus,providingthereaderwiththefoundations neededtograspthetreatmentsthatwillemergeafterthepublicationofthisbook. Wehavealsoincorporatedclinicalscenariostoreinforcetheknowledgeand understandingofthebasicscientificpriniciples.Wehopeyouenjoyhaematology andimmunology. Yousef Gargani Faculty advisors Immunologyandhaematologyaretwoofthemostrapidlymovingdisciplinesin modernmedicine.Inthesetwodisciplinesmoleculardiscoveriesarerapidly translatedintodiagnostictestsandtreatments.InthisCrashCourseyou’llread aboutexcitingstuffsuchasdesignerdrugsforleukaemiaandgenetherapyfor immunodeficiency. Theflipsideofallthisprogressisthatthesetwosubjectsarenotalwaystaughtinan up-to-datefashionandsomeofthenewerfactsgetmissedout.It’sbeenapleasure toworkwithYousefonthiseditionofthebook.Hischarm,intelligenceandenergy willtakehimalongway. Matthew Helbert and Caroline Shiach vi Acknowledgements Figure acknowledgements Figure12.7adaptedwithpermissionfromCJaneway.Immunobiology,4thedition. ChurchillLivingstone,1999 Figs.1.4,10.5,10.10,10.15,10.18and12.11,andFigs10.20and12.20adapted withpermissionfromIRoitt,DMaleandIBrostoff.Immunology,4thedition. Mosby,1996 Figs.2.1,2.12and11.3takenwithpermissionfromAStevensandJLowe.Human Histology,2ndedition.Mosby,1997 Fig.1.6adaptedwithpermissionfromCHaslett(editor).Davidson’sPrinciplesand PracticeofMedicine,18thedition.ChurchillLivingstone,1999 Fig.5.8reproducedwithpermissionfromMMakrisandMGreaves.Bloodin SystemicDisease.Mosby,1997 Fig.6.17adaptedwithpermissionfromTGordon-SmithandJMarsh.Medicine (HaematologyPart1).TheMedicinePublishingCompany,2000 Figs.2.16,3.1,3.7,3.12,4.2,4.6,4.8,4.11,5.9reproducedwithpermissionfrom Dacie&LewisPracticalHaematology,10e,ChurchillLivingstone,2006 Figs.9.8,12.2,12.10,12.14reproducedwithpermissionfromRNairnand MHelbert.Immunologyformedicalstudents,2ndedition.Mosby,2007 Figs.2.15,4.4reproducedwithpermissionfromDrShiachandthelibraryofCentral ManchesterUniversityHospitals vii Intentionally left as blank Contents 5. Haematologicalmalignancies. . . . . . . 45 Serieseditorforeword . . . . . . . . . . . . . . . v Introductiontohaematological Prefaces . . . . . . . . . . . . . . . . . . . . . . vi malignancies . . . . . . . . . . . . . . 45 Acknowledgements. . . . . . . . . . . . . . . . . vii Investigationsofhaematological malignancies . . . . . . . . . . . . . . 54 1. Principlesofhaematology . . . . . . . . . 1 6. Haemostasis. . . . . . . . . . . . . . . 57 Overviewofhaematology—thecell Plateletsandbloodcoagulation . . . . . . 57 lines . . . . . . . . . . . . . . . . . . . 1 Antiplateletdrugs . . . . . . . . . . . . 59 Haemopoiesisanditsregulation. . . . . . . 2 Plateletdisorders. . . . . . . . . . . . . 60 Bonemarrow . . . . . . . . . . . . . . . 3 Thecoagulationcascade . . . . . . . . . 63 Thespleen. . . . . . . . . . . . . . . . . 4 Clottingtests . . . . . . . . . . . . . . 66 2. Redbloodcellsandhaemoglobin. . . . . . 9 Clottingfactordisorders . . . . . . . . . 66 Structureandfunctionoferythrocytes. . . . 9 Thrombosis . . . . . . . . . . . . . . . 69 Erythropoiesis . . . . . . . . . . . . . . . 9 7. Bloodtransfusion . . . . . . . . . . . . 73 Ironandhaemmetabolism . . . . . . . . 12 Introductioninbloodproducts . . . . . . 73 Haemoglobin . . . . . . . . . . . . . . 15 Indicationsforredcelltransfusion. . . . . 73 Thecytoskeletonoftheredcell. . . . . . 17 Red-cellantigens. . . . . . . . . . . . . 74 Metabolismofredcells. . . . . . . . . . 18 Cross-matchingandbloodtransfusion. . . 75 Fullbloodcountandreticulocyte count . . . . . . . . . . . . . . . . . 19 8. Principlesofimmunology . . . . . . . . 79 Peripheralbloodfilm . . . . . . . . . . . 20 Anoverviewofimmunology . . . . . . . 79 3. Redbloodcelldisorders . . . . . . . . . 23 9. Theinnateimmunesystem. . . . . . . . 83 Anaemiacausedbyhaematinic Barrierstoinfection . . . . . . . . . . . 83 deficiency . . . . . . . . . . . . . . . 23 Cellsofinnateimmunity . . . . . . . . . 83 Anaemiaofchronicdisease. . . . . . . . 24 Solubleproteins . . . . . . . . . . . . . 86 Anaemiaduetoincreasedred-cell Innateimmunesystempattern destruction(haemolyticanaemias) . . . . 27 recognitionmolecules. . . . . . . . . . 90 Haemoglobinopathies . . . . . . . . . . 31 10. Theadaptiveimmunesystem. . . . . . . 91 Investigationofhaemoglobinopathies . . . 33 Theimmunoglobulindomain . . . . . . . 91 Anaemiaduetobloodloss . . . . . . . . 34 Generationofantigenreceptordiversity. . 94 Marrowdefects . . . . . . . . . . . . . 34 Humoralimmunity. . . . . . . . . . . . 97 Polycythaemia(erythrocytosis) . . . . . . 35 Cell-mediatedimmunity . . . . . . . . .102 4. Whitebloodcells . . . . . . . . . . . . 37 11. Thefunctioningimmunesystem . . . . .109 Structureandfunctionofthewhite bloodcells . . . . . . . . . . . . . . . 37 Responsetotissuedamage. . . . . . . .109 Differentiationofwhitecells . . . . . . . 39 Immuneresponsetopathogens. . . . . .112 Differentialwhitecount . . . . . . . . . 40 12. Immunedysfunction. . . . . . . . . . .117 Reactiveproliferationofwhitecells . . . . 42 Hypersensitivity . . . . . . . . . . . . .117 Leucopenia . . . . . . . . . . . . . . . 42 Allergy . . . . . . . . . . . . . . . . .120 ix
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