Crash Course Haematology and Immunology

193 Pages·2012·9.207 MB·English

by  Yousef Gargani, Daniel Dr Horton-Szar, Caroline Shiach, Matthew Helbert

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