haematology 2012 mrcppass

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www.MRCPass.com Haematology

Dr.KhalidYusufElzohrySohagTeachingHospital20122

HaematologyQ001:An8yearoldboywithsicklecelldiseasepresentswithbreathlessness.HisHbis4.5g/dl,WCCis3x10^9/landplateletcountis35x10^9/l.Whichorganismislikelytoberesponsible?

A.CoronavirusB.HIVC.HSVD.ParvovirusE.Epsteinbarrvirus

Answer:d)parvovirus.ParvovirusB19isthecommonestcauseofaplasccrisisinsicklecellanaemia.Recoveryshouldoccurwithin10dayswithconservativetreatment.



HaematologyQ002:A 45 yearoldman isbeing invesgated foreasybruising andmalaise. Investigationsreveal:Haemoglobin9.5g/dLWhitecellcount90x10^9/LNeutrophils45x10^9/L(1.57)Lymphocytes3.5x10^9/L(1.54)Myelocytes30x10^9/LMyeloblasts3x10^9/LPlateletcount750x10^9/LWhichofthefollowingdiagnosisislikely?

A.AcutemyeloidleukaemiaB.AcutelymphocyticleukaemiaC.ChronicmyeloidleukaemiaD.ChroniclymphocyticleukaemiaE.Polycythaemiarubravera

Answer:c)chronicmyeloidleukaemia.Ahighneutrophilcount,plateletcountpointstowardsmyeloidleukaemia.Acute leukaemia is defined as blast cells comprising 30% (in this case only 10% ofmyelocytes)ofthecelltype.HenceitmakesCMLmorelikelythanAML.



HaematologyQ003:A30yearoldladyaendsA&Ewithseverenosebleeds.Herinvestigationsshow:Hb10.5g/dlMCV80flWCC7x10^9/lplatelets3x10^9/lBloodfilmreport:Noplateletclumpsseen.NormalrbcandwbcClottingscreennormalWhatisthemostlikelydiagnosis?

A.ThromboticthrombocytopenicpurpuraB.HaemolyticuraemicsyndromeC.AcutelymphoblasticleukaemiaD.DisseminatedintravascularcoagulationE.Immunethrombocytopenia

Answer:e)Immunethrombocytopenia.Asthereisnoabnormalityintheredandwhitebloodcellsonthebloodfilm,thisismostlikelytobeimmunethrombocytopenia.Featuresconsistentwithadiagnosisofimmunethrombocytopenicpurpura (ITP)are thrombocytopeniawithplateletsbeingnormal insizeormayappear larger thannormal,butuniformlygiantplatelets (approaching thesizeofredcells)shouldbeabsent.Themorphologyofredbloodcellsandwhitebloodcellsshouldbenormal.



HaematologyQ004:A72yearoldladyhasrecentlybeenfoundtobeanaemic.Furtherbloodtestsshow:Hbof9.2g/dlWCCof8.0x10^9/lplateletcountof200x10^9/lMCVis104fl(8096)Ferrinis120g/l(15200)redcellfolateis350g/l(150650)B12is400pmol/l(120700)BloodfilmshowsanisocytosisandpoikilocytosisWhichofthefollowingdiagnosisislikely?

A.ChroniclymphocyticleukaemiaB.AutoimmunehaemolyticanaemiaC.SideroblasticanaemiaD.IrondeficiencyE.Lymphoma

Answer:c)sideroblasticanaemia.AhighMCVwithnormal folateandB12 levels,normal ironandabloodfilm showinganisocytosisandpoikilocytosissuggestssideroblasticanaemia.



HaematologyQ005:A20yearoldmanpresentswithacuteseveredyspnoea.Hehadbeenstungbyawaspseveralhoursago.Onexamination,hewashypotensiveandhadsignsofbronchospasm.Which one of the following investigationswould confirm the type of hypersensitivityreaction?

A.PlasmatryptaselevelB.ESRC.SerumIgElevelD.VenomtoxinlevelE.ComplementC3level

Answer:C)serumIgElevel.TypeIhypersensivityisoccurringinthiscaseofanaphylaxis.Ittakes30minutesfromtimeofexposureantigen.ThereactioninvolvesproductionofIgEwhichisreleasedfrommastcells.



HaematologyQ006:A25yearoldwomanpresentswithdiffuse lymphadenopathy, feverandmalaise.Herbloodfilmshowsatypicallymphocytesandredcellagglutination.Whatisthemostlikelydiagnosis?

A.LegionellaB.InfectiousmononucleosisC.MeningococcalmeningitisD.NonHodgkinslymphomaE.Autoimmunehaemolyticanaemia

Answer:b)Infectiousmononucleosis.InfectiousmononucleosisiscausedbyEpsteinBarrvirus.Itisoneofthecommoncausesofatypicallymphocytes,alongwithcytomegalovirus,HIVandToxoplasma.Thefeaturesoflymphadenopathyandatypicallymphocytessuggestinfectiousmononucleosis.



HaematologyQ007:A62yearoldmanwhowasasymptomatic,wasreferredforinvestigationofahighwhitecell count routinely foundby theGP.Onexamination,hehadpalpable splenomegalyandlookedpale.Resultsreveal:Haemoglobin10.5g/dl(11.516.5)Plateletcount175x10^9/L(150400)Whitecellcount32x10^9/l(411)Neutrophilcount4x10^9/L(1.57)Lymphocytecount27x10^9/L(1.54)HisBloodfilmshowsmanymaturelymphocytesWhatisthebestinitialmanagement?

A.PrednisoloneB.PeriodofobservationC.RadiotherapyD.SplenectomyE.Chlorambucil

Answer:b)periodofobservation.In chronic lymphocytic leukaemia, Indications for therapy include fatigue,lymphadenopathy,anaemiaorthrombocytopenia.Alloftheothersaretreatmentoptionswhichcanbeused.



HaematologyQ008:A20yearoldmanpresentedwithacoughand fevers.Hewasdiagnosedashavingachest infection andwas prescribed two different antibiotics.He feltworse two dayslaterandmentionedthathehaddarkurine.Investigationsshowed:Hb8.5g/dlMCV75flWCC12x10^9/lplatelets155x10^9/lBloodfilmshowed:anisopokilocytosisandbitecellsWhatisthediagnosis?

A.G6PDdeficiencyB.AutoimmunehaemolyticanemiaC.AplasticanemiaD.ImmunethrombocytopenicpurpuraE.Acutemyeloidleukaemia

Answer:A)G6PDdeficiencyThereisevidenceofhaemolysis(bitecellsareschistocytes),inthiscasemostlikelyduetoG6PDdeficiency.Drugsnormallycausinghaemolysis inG6PDdeficiencyaresulphurcontainingdapsone,antimalarials,bactrim/septrim,sulphonamides,primaquinine.



HaematologyQ009:A65yearoldmanhasrecentlybeendiagnosedashavingadeepveinthrombosis.Healso has symptoms of headaches and lethargy. On examination he was flushed.Investigationsreveal:haemoglobin19.5g/dLhaematocrit0.6(0.40.52)whitecellcount10.5x10^9/L(411)plateletcount450x10^9/L(150400)Whichoneoffollowingisthemostappropriateinvestigation?

A.SerumEPOlevelB.BonemarrowaspirateC.NeutrophilalkalinephosphataseD.RedcellmassE.SerumvitaminB12levels

Answer:d)Redcellmass.Themostappropriateinitialinvestigationwillberedcellmassstudieswhichwouldwilldistinguishbetweentruerelativepolycythaemiafromsecondarypolycythaemia.InPolycythaemiaRubraVera, the serum EPO is low (anelevatedEPO level suggestssecondarypolycythaemia).Haematocrit ishigh as is theHb concentration. Thrombocytosis and leukocytosis canoccur.TheNAPscoreandB12levelsarefrequentlyincreased.



HaematologyQ010:A55yearoldwomanwhohadacerebrovascularaccidenttenmonthago,wasreferredfor investigation of recurrent episodesof proximal deep venous thrombosis (DVT)oflowerlimbsinthelastsevenmonths.Investigationsshow:hemoglobin7.9g/dl hematocrit25%meancorpuscularvolume99fl meancorpuscularhemoglobin32pgwhitebloodcells4x10^9.l platelets93x10^9/lreculocytes5.4% lactatedehydrogenase944UI/ltotalbilirubin50umol/l.Abonemarrowbiopsy showed a slighthyperplasiaoferythrocyticbonemarrow cellline.UrineDipstickblood+++Whatisthelikelydiagnosis?

A.HaemolyticuraemicsyndromeB.AntithrombinIIIdeficiencyC.ParoxysmalnocturnalhaemoglobinuriaD.ProteinCdeficiencyE.ProteinSdeficiency

Answer:c)Paroxysmalnocturnalhaemoglobinuria.Paroxysmal nocturnal haemoglobinuria (PNH) is an aplastic anaemia like syndromewhichredcellsarepredisposedtocomplementlysisandresultanthaemolyticanaemia.ThereisapancytopeniaaswellasatendencytowardsBuddChiarithrombosis.Thediagnostic test is theHAM test.Serum (whichcontainscomplements) isacidified(activates the complement pathway) andmixed with red cells which undergo lysis.Haemosiderinisabyproductofhaembreakdowncontainingiron.Excessamountsleadstorenaldamage,andisalsolostintheurine.InPNH,there isa lossofanchorprotein(GPIglycosylphosphatidyl inositol)whichholddierentangense.g.CD59,CD14.Theseareregulatoryproteins for thecomplementpathway.



HaematologyQ011:A17yearoldpaenthassicklecelldisease.Hepresentsunwellwithabdominalpain.Healsohasrightsidedfacialweakness.Investigations:Hb7.5g/dlplatelets140x10^9/lUrea8mmol/lcreanine100mol/lsodium141mmol/lpotassium3.8mmol/lbilirubin45mol/lAST35U/lALP105U/lAlb42g/lLDH1250U/lBloodfilmshowssicklecellsWhichofthefollowingisthemostimportantmanagement?

A.IvfluidsB.IvantibioticsC.DiamorphineinjectionsD.BloodtransfusionE.Exchangetransfusion

Answer:e)exchangetransfusion.When there isneurologicaldamageorvisceral sequestration crisis in sickle cell crisis,exchange transfusion is indicated. Exchange transfusion involves drawing out thepatient'sbloodwhileexchangingitfordonorredbloodcells.Itcanbedonemanuallyorautomatically with erythrocytapheresis. It prevents stroke and alsomay be used inpatientswith severeacutechest syndromeand to reduce the riskof ironoverload inpatientswhorequirechronictransfusiontherapy.Studiessuggestthat itmay improveoxygenationandreducehemoglobinSlevels.



HaematologyQ012:A35yearoldmanpresentswithpallorandbreathlessness.BloodtestsshowanaemiawithaHbof7.5g/dl.AbloodfilmshowsHeinzbodies.Whichoneofthefollowingdiagnosesismostlikely?

A.AutoimmunehaemolyticanaemiaB.SideroblasticanaemiaC.G6PDdeficiencyD.PostsplenectomyE.Sicklecelldisease

Answer:c)G6PDdeficiency.Heinzbodiesareprecipitated,denaturedHbwithinredcells.TheyarepresentinG6PDdeficiency.(FavabeanscausehaemolysisinG6PD'BeansmeansHeinz'mnemonic).



HaematologyQ013:Which one of these patient's results is most likely to have a diagnosis of chroniclymphaticleukaemia?

A.Awhitecellcountof35x10^9/landimmaturelymphocyteswithprominentnucleoliintheperipheralblood

B.Awhitecellcountof15x10^9/landmaturelymphocyteswithcleavednucleiintheperipheralbloodfilm

C.Awhitecellcountof65x10^9/lwithneutophils,myelocytesandpromyelocytesonthebloodfilm

D.Awhitecellcountof25x10^9/landsmearcellsontheperipheralbloodfilmE.Awhitecellcountof6x10^9/l,andmaturelymphocyteswithpolarvilliontheblood

filmAnswer:d)Awhitecellcountof25x10^9/land smearcellson theperipheralbloodfilm.Chroniclymphaticleukaemiaischaracterisedbyalymphocytosis.Thebloodfilmshowsmaturelymphocyteswithsmearorsmudgecells(theyaresquashedcells).



HaematologyQ014:A65yearoldwomanhasadiagnosisofchroniclymphocyticleukaemia(CLL).Duringonefollowupappointmentshementionsthatshehasgotprogressivelymorelethargic.Herinvestigationsshow:Hb7.5g/dl,MCV118fl,platelets180x10^9/l,lymphocytes43x10^9/l,reculocytecount10%.Whichtestismostlikelytogivethecorrectdiagnosis?

A.FolatelevelB.MarrowtrephineC.SerumelectrophoresisD.FerritinE.Coomb'stest

Answer:e)Coomb'stest.AraisedreticulocytecountcouldhaveledtothehighMCV.TheclinicalpictureisoneofhaemolysiswhichisoccasionallyseeninCLL.TheCoomb'stestwillhelptoconfirmthis.



HaematologyQ015:A28yearoldmanwithglucose6phosphatedehydrogenasedeficiencypresentswithfatigueandjaundice.Thefeaturesdevelopedfollowingapneumoniaaweekago.Whichofthefollowingislikelytobefound?

A.LowmeancellvolumeB.PositivedirectantiglobulintestC.SpherocytespresentonbloodfilmD.HaemoglobinuriaE.Reducedreticulocytecount

Answer:d)Haemoglobinuria.The clinical scenario describes haemolytic anaemia. Haemoglobinuria is seen inhaemolytic anaemia. Patientmay presentwith fatigue and tiredness. Lowmean cellvolumewouldmean lackof reticulocytosis.This isunlikely, there isusually increasedreticulocyte count in all haemolytic anaemias includingG6PD deficiency. Spherocytesareseen inhereditaryspherocytosisandtheantiglobintest ispositive inautoimmunehaemolycanaemia(notjustG6PDdeficiency).



HaematologyQ016:A 60 year old AfroCarribean man is referred with abdominal discomfort. Onexamination,hehasmassivesplenomegaly.TheFBCshows:Hb8.2g/dlWBC15x10^9/lPlatelets110x10^9/lBloodsmear:erythroblasticpictureWhichofthefollowingdiagnosesisthemostlikely?

A.MyelofibrosisB.PolycythaemiarubraveraC.NonHodgkinslymphoma(NHL)D.AplasticanaemiaE.Chronicmyeloidleukaemia

Answer:a)Myelofibrosis.Myelofibrosisexistis likelytobethecasedescribedabove.Medianageatdiagnosis isabout 60 years, andmedian life expectancy from onset of symptoms is 10 years. Incontrast,acuteMFinadulthoodisarapidlyfataldisorderinwhichsplenomegalyisnotusually observed; bone marrow examination typically reveals numerous bizarremegakaryocytesandblasts.



HaematologyQ017:A32yearoldwomanpresentstothecasualtywithworseningdyspnoeaover3weeks.Shehasnohistoryofjaundiceofanaemia.On examinaon, she had a blood pressure of 125/65mmHg.Her conjunctivaewerepale. Abdominal examination was unremarkable and there was no splenomegaly.Investigationsshow:Hb6.5g/dlWBC13.5x10^9/lPlts255x10^9/lMCV105flLDH680IU/dlDirectCoomb'stestpositiveFilm:Spherocytes++,reticulocytes++Whatshouldbethetreatmentforhercondition?

A.IronreplacementB.BonemarrowexaminationC.VitaminKD.ImmunosupressantsE.B12andfolate

Answer:d)immunosupressants.Thiswoman ismost likely to have autoimmune haemolytic anaemia (anaemia, highLDH, spherocytes on the blood film), and positive Direct Coomb's test. Steroids,intravenousimmunoglobulinmaybeusedasfirstlinetreatment,andbloodtransfusionmaybenecessary.Autoimmunehaemolyticanaemiacanbedue to immunedisorders(SLE),toxicchemicalsanddrugs,(methyldopa,penicillin),antiviralagents(eg,ribavirin),physicaldamage,andinfections(infectiousmononucleosis).



HaematologyQ018:A35yearoldmanpresentswithfeversand lymphadenopathy.AbonemarrowbiopsywasdoneandconfirmsHodgkin'slymphoma.Whichoneofthefollowingformhasthebestprognosis?

A.NodularsclerosingB.LymphocytepredominantC.LymphocytedepletedD.MixedcellularityE.Promyelocytic

Answer:b)lymphocytepredominant.Hodgkin's lymphoma is rare in children. Nodular sclerosing is the commonest andlymphocytedepletedistherarestform.Thelymphocytepredominantformhasthebestprognosis,whilstthelymphocytedepletedformhastheworst.



HaematologyQ019:A30yearoldmanpresentswithmalaiseandisfoundtobeanaemicclinically.Hisbloodtestsreveal:Hbof10.5g/dlWCC8x10^9/lplateletcount180x10^9/dlreculcytecount160x10^9/l(50100)Bilirubinis80mol/lAST30U/lALP110U/lLDHus380U/l(10250)BloodfilmshowsspherocytosisWhichofthefollowingtestsismostappropriate?

A.DirectantiglobulintestB.G6PDacvityC.HbelectrophoresisD.UrinaryhaemosiderinE.Methaemoglobinlevels

Answer:a)Directantiglobulintest.The blood tests with high bilirubin, reticulocyte count and high LDH suggestshaemolysis.Spherocytesonbloodfilmsuggestshereditaryspherocytosis(HS).InHStheredcellsaresmaller,rounder,andmorefragilethannormal.TheconditioniscommoneramongNorthernEuropeans.Thedirectantiglobulintestwillhelptoconfirmthis.



HaematologyQ020:A40yearoldmanhaspresentedwithseizuresandhasaconfirmedcerebralinfarctonheadscan.Heiscommencedonphenytoin.3weekslaterhepresentswithlethargy.His bloods show Hb 8.0 g/dl,MCV 95 fl,WCC 3.2 x 10^9/l, platelets 65 x 10^9/l,Reculocyte count 1%. Ham's testwas negative. Bonemarrowaspirate and trephinebiopsyshowedmarkedhypocellularityofthemarrowwithsomelymphoidaggregates.Whatisthelikelydiagnosis?

A.FolatedeficiencyB.MyelofibrosisC.AplasticanaemiaD.MultiplemyelomaE.Bonymetastasis

Answer:c)aplasticanaemia.The diagnosis is likely to be phenytoin related aplastic anaemia.MCV is normal andthere isa lowreticulocytecountaswellashypocellularbonemarrow .Sideeffectsofphenytoin are cerebellar syndrome, phenytoin encephalopathy, psychosis, locomotordysfunction, hyperkinesia, megaloblastic anemia, decreased serum folate level,decreasedbonemineralcontent,liverdisease,IgAdeficiencyandgingivalhyperplasia.



HaematologyQ021:A 70 year old woman was admitted to hospital with severe breathlessness. Onexaminaonherbloodpressurewas100/55mmHgandshehadaraisedJVPby4cm.Chestxrayshowedmildpulmonaryoedema.Investigationsrevealed:Haemoglobin6.6g/dLMCV108fLMCH32.0pgWhitecellcount3.0x10^9/LPlatelets75x10^9/LSerumvitaminB12normalFolate2(320)g/lWhatshouldbedone?

A.TreatcongestivecardiacfailurethentransfuseB.ImmediatebloodtransfusionC.SerumelectrophoresisD.IronreplacementE.B12andfolatereplacement

Answer:e)B12andfolatereplacement.Blood transfusionmayworsen cardiac failure in this case. Inpatientswho are folatedeficienterythropoiesisrapidlyresolveswhensupplementsaregiven,andtransfusionisrarelyneededintheelderly(unlesstheanaemiaisverysevere).



HaematologyQ022:A35yearoldmanhasrecentlybeendiagnosedwithHodgkin'slymphoma.Inreviewinghissymptoms,whichoneofthefollowingindicatesthepoorestprognosisinHodgkin'slymphoma?

A.Mediastinal,inguinallymphadenopathyandfeverB.MediastinallymphadenopathyandnightsweatsC.Abdominalandinguinallymphadenopathy,andnightsweatsD.CervicalandmediastinallymphadenopathyE.Mediastinalandinguinallymphadenopathy.

Answer:a)mediastinal,inguinallymphadenopathyandfever.StageIIIdiseaseoccurswhenlymphnodesarepresentacrossbothsidesofdiaphragm,henceworseprognosis thanwhen lymphnodesare localised to the same sideof thediaphragm.PresenceofBsymptomsnightsweatsandfeversalsoworsenprognosis.



HaematologyQ023:A25yearoldmanwasadmiedwitha2monthhistoryof rash, fague, intermienthemoptysis, and purpura, culminating in a seizure. On examination, widespreadpetechiaeandpurpurawithscleralicteruswerenoted.Therewasnolymphadenopathyorsplenomegaly.Investigationsshow:plateletcount310^9/Lhemoglobin5.5g/dLmeancorpuscularvolume90fLwhitecellcount19.610^9/Lurea16mmol/Lcreanine270mol/Llactatedehydrogenase2200U/Ltotalbilirubin79mol/Lhaptoglobin6g/LBlood film shows anisocytosis, moderate to marked polychromasia, and slight tomoderatepoikilocytosis,predominantlyschistocytes.Whatisthebesttreatmentoption?

A.HaemodialysisB.AzathioprineC.PlasmaexchangeD.BonemarrowtransplantE.Intravenousimmunoglobulins

Answer:c)plasmaexchange.Thrombotic thrombocytopenic purpura (TTP) is characterised by microangiopathichaemolysisandthrombocytopenia.ThereisaspectrumofpresentationswithTTPHUS.Neurological featuresarepresent in60%ofpatientsofTTPand renal failure isoftenassociatedinHUS(haemolyticuraemicsyndrome).



HaematologyQ024:A8yearoldboypresentstohisGPwithlethargyandpallor.Hisinvestigationsshow:Hb5.5g/dlWBC2.7x10^9/lPlts42x10^9/lNeutrophils0.9x10^9/lWhatisthenextbestinvestigation?

A.PeripheralbloodimmunophenotypingB.BonemarrowcytogeneticsC.HaematinicsD.BonemarrowaspirateandtrephineE.ANAandRheumatoidfactor

Answer:d)Bonemarrowaspirateandtrephine.Pancytopeniamaybeduetobonemarrowfailure(aplasticanaemia)ortobonemarrowinfiltration (leukaemia, lymphomaornonhaemopoieticmalignancy).Aplasticanaemiamay be idiopathic or secondary to drugs, paroxysmal nocturnal haemoglobinuria orFanconis anaemia. In a child of this age, leukaemia (ALL, AML) or aplastic anaemiawouldbethemostlikelycausesofpancytopenia.



HaematologyQ025:A38yearoldwomanpresentstothehaematologistforreviewasshehaslethargy.Sheisonirontablets.Herbloodresultsshow:Hb9.5g/dlMCV105flWCC7x10^9/lplatelets218x10^9/lBloodfilmshowsanisopoikilocytosisandpoikilocytosisWhatshouldbedonenext?

A.IntramuscularirontherapyB.BloodtransfusionC.ErythropoietinD.InvestigationforfolateeficiencyE.Noimmediateaction

Answer:E)noimmediateaction.Thebloodfilmandpoorresponsetoirontherapysuggestssideroblasticanaemia.Sideroblasticanaemia ismanagedbyremovingtheprecipitatingfactorse.g.alcoholormyelodysplasia.



HaematologyQ026:A23yearoldmanpresentswith jaundiceduringaplannedholiday toAfrica.Hehasbeentakingmalarialprophylaxis.Heisapyrexialandapartfromlethargy,feelswell.Hereportspassingdarkurineforthepasttwodays.Whatisthelikelycause?

A.BetathalassemiaB.HaemolysisduetoG6PDdeficiencyC.SicklecellcrisisD.FalciparummalariaE.HepatitisCinfection

Answer:b)HaemolysisduetoG6PDdeficiency.G6PDdeficiencyiscommonintheMediterraneanandAfricanpopulations.InheritanceisXlinked. Intravascularhaemolysis isusuallyprecipitatedbyoxidativestress,suchasinfections anddrugs.Themost commondrugs implicated are antimalarials,dapsoneandsulphonamides.



HaematologyQ027:A 20 year oldman complains of intermient dark urine and abdominal pains.He isfoundtohaveahaemoglobinof9.7g/dl,buttherestofthefullbloodcountisnormal.Whatisthemostlikelydiagnosis?

A.AutoimmunehaemolyticanaemiaB.G6PDdeficiencyC.ParoxysmalnocturnalhaemoglobinuriaD.ParoxysmalcoldhemoglobinuriaE.Hereditaryspherocytosis

Answer:c)Paroxysmalnocturnalhaemoglobinuria.Paroxysmalnocturnalhaemoglobinuriaiscausedbyadefectintheformationofaredcell surfaceproteinanchor, calledGP1.Asa resultof the lackof this surfaceproteinanchor, the red blood cells are more sensitive to complement lysis. Patients haveintravascularhaemolysis,leadingtohaemoglobinuria,andincreasedriskofthrombosis,oftenoccurringinthemesentericvesselsandtheportalvein.Treatmentissupportiveorwithbonemarrowtransplantation.



HaematologyQ028:A35yearoldmanhashada4dayhistoryofdarkurine.Hehas recentlybeenonanantibioticforapresumedurinarytractinfection.Hisbloodtestsshow:Hb5.0g/dl MCV103flMCHC34g/dl(3235) WCC8x10^9/lreculocytes160x10^9/l(50100) platelets130x10^9/lPT13s(11.515.5) APTT38s(3040)urea6mol/l creanine90mol/lsodium140mmol/l potassium4mmol/lbilirubin48(122)mol/l AST18(131)U/lALP150(20120)U/l albumin32g/lLDH1550U/l(10250) Bloodfilmshowsblistercells.Whatisthediagnosis?

A.AutoimmunehaemolyticanaemiaB.HereditaryspherocytosisC.ParoxysmalnocturnalhaemoglobinuriaD.G6PDdeficiencyE.Porphyria

Answer:d)G6PDdeficiency.Thepatient'sclinicaland laboratory findings (eg,markedlydecreasedhemoglobinandhematocrit levelswithamarkedly increased serum LDHactivity),are characteristicofacuteoxidantdamagetotheredbloodcellsandhemolysisduetoglucose6phosphatedehydrogenase(G6PD)deficiency.The blood film in G6PD deficiency shows blister cells (membrane protrusion) (Heinzbodies may also be seen when there is no haemolysis). Treatment is with bloodtransfusion,orinseverecases,exchangetransfusion.Hemolyticcrisisoccursonlyafterexposuretocertainoffendingagents,includingdrugs,infections, exposure to fava beans, and diabetic acidosis. Drugs associated withhemolysis in G6PD deficiency include anmalarials (Primaquine, pamaquine),sulphonamides (Sulphamethoxazole), nitrofurantoin, analgesics (acetaminophen,aspirin,phenacetin),isoniazid(INH),methyleneblue,andnalidixicacid.



HaematologyQ029:A32yearoldCypriotpaentisbeinginvesgatedforanaemia.HehasaHbof7.5g/dlandMCVis70fl.Hisbrotherandsistersarealsoanaemic.Whichoneofthefollowingismostlikely?

A.IncreasedIgMbandonserumelectrophoresisB.RedcellsshowmarkedhypochromiaC.SevereirondeficiencyduetoGIbleedingD.SevereB12deficiencyduetoperniciousanaemiaE.Severefolatedeficiencyduetoceliacdisease

Answer:B)redcellsshowmarkedhypochromiaThis patient is likely to have thalassaemia (probablymajor).Hb electrophoresismayshow increased HbA2 in thalassaemiaminor. The severe imbalance of globin chainsynthesis (alpha >> beta) results in ineffective erythropoiesis and severemicrocytichypochromicanemia,theremayalsobeprecipitateswithindamagedredcells.



HaematologyQ030:A35yearoldmanhasknowntype1VonWillebrandsdisease.Priortosurgery,whichisthebesttesttoassessbleedingtendency?

A.ProthrombintimeB.FactorVIIIantigenC.FactorVIIIlevelsD.BleedingtimeE.Thrombintime

Answer:b)factorVIIIantigen.Bleeding time is usually prolonged, and does not provide quantification of bleedingtendency.FactorVIIIantigenmeasuresthepresenceofvWFandgivesagoodestimateoftendencytobleed.



HaematologyQ031:An19yearoldmanpresentstotheA&Ewithapetechialrashandplateletcountof5x10^9/l. He is otherwisewell. A diagnosis of idiopathic thrombocytopenic purpura ismade.Whichofthefollowingstatementsistrue?

A.ThepatientshouldbegivenaplatelettransfusionB.ThepatientshouldbeobservedC.ThepatientshouldbetreatedwithAntiDD.ThepatientshouldbetreatedwithintravenousimmunoglobulinE.Thepatientshouldbecommencedonsteroids

Answer:e)Thepatientshouldbecommencedonsteroids.In youngerpatientswith ITP, thediseaseusually remits spontaneouslywithin severalweeksandnotreatmentisusuallyrequiredunlessthereissignificantbleeding.However, after adolescence, thedisease tends to run a chronic relapsing course andthereforerequirestherapy.First line therapy isoral steroids.Patientswho are refractory to,or are intolerantofsteroidsmayrespondtointravenousimmunoglobulins(IVIg)orantiD.



HaematologyQ032:A35yearoldmanhasafaintmaculopapularrashonhischestandafewshoylymphnodes.HisbloodsshowHb13.5g/dl,WCC14.0x10^9/l,plts300x10^9/l.Bloodfilmshowsreactivelymphocytes.Whichofthefollowingdiagnosisislikely?

A.TuberculosisB.Nonhodgkin'slymphomaC.HepatitisBD.InfectiousmononucleosisE.Pneumonia

Answer:d)infectiousmononucleosis.Thereareseveral reactive lymphnodesaswellas reactive lymphocytessuggestiveofEbstein Barr virus infection / infectious mononucleosis. Other causes of reactivelymphocytesareCMVinfection,toxoplasmosisandHIV.



HaematologyQ033:A65yearoldwomanhas symptomsofeasybruising.Shewas referredby theGP forinvestigation.Onexamination,shehadsplenomegaly.Resultsshow:Haemoglobin6.5g/dL(11.516.5)Whitecellcount17x10^9/l(411)Plateletcount32x10^9/l(150400)Bloodfilmshowslymphocytosis,myeloblastsandpromyelocytes.Whichoneoffollowinginvestigationsisofprognosticvalueinthissituation?

A.BloodfilmB.BonemarrowaspirateC.CytogenetickaryotypingD.ImmunophenotypingE.Serumelectrophoresis

Answer:c)cytogenetickaryotyping.Cytogeneticmonitoringoftheclinicalcourseofacutemyeloidleukaemia(suggestedbyblasts) is oen associated with a specific chromosomal change, ie, t(8;21) in M2.Establishmentofthechangeatdiagnosisallowsrecognitionoftheleukemiccellsinthemarrowwhenrelapseorresidualdiseaseistobeevaluated.Italsoprovidesaprognosticdeterminant.



HaematologyQ034:A52yearoldfemalepresentswithacutechestpainandbreathlessness.Thechestpainswerepleuriticand started todevelopaweekago.Examinaon revealsprominentP2and clear breath sounds. She had bilateral ankle oedema. A urine dipstick showedprotein+++.Whichisthemostlikelyexplanationforthesefindings?

A.FactorVLeidenB.ReducedantithrombinIIIactivityC.ReducedlevelsofVonWillebrandsfactorD.ReducedddimerconcentrationE.ReducedfactorVIII

Answer:B)reducedantithrombinIIIactivity.AT III deficiency is associated with venous thrombosis. In this case, the history isconsistentwithaclinicaldiagnosisofpulmonaryemboliandrenalveinthrombosis.



HaematologyQ035:A 35 year old man has had allogeneic bone marrow transplantaon which is HLAmatched.2weekslaterhedevelopsadiffuserashalloverhisbody,feelssickandvomitsseveralmes.Histemperatureis38C.Bloodtestsshow:Hb11.0g/dlWCC3x10^9/lNeutrophils1.5x10^9/lplatelets18x10^9/lPT18s(11.515.5)urea7mol/lcreanine70mol/lsodium135mmol/lpotassium4mmol/lbilirubin28mol/lAST48U/lALP155U/lalbumin32g/lLDH550U/lWhichofthefollowingismostlikely?

A.BonemarrowfailureB.ParvovirusinfectionC.LeukaemicspreadD.AplasticanaemiaE.Graftversushostdisease

Answer:e)graftversushostdisease.Therash,systemicsymptoms,derangedliverenzymespointtowardsGVHD.Tcellsfromthe donor are attacking the recipient. Treatment is with immunosuppression:ciclosporin,methylprednisolone,methotrexateorantithymocyteglobulin(ATG).



HaematologyQ036:A25 yearoldwomanpresentedunwellwithdiarrhoeaoccurring5 mesaday for4days.Shehadnotpassedurineforaday.Investigations:Haemoglobin8.2g/dLWhitecellcount14.2x10^9/LNeutrophils10.5x10^9/LPlatelets32x10/LFibrinogen5g/dLSerumsodium138mmol/LSerumpotassium6.3mmol/LSerumurea38mmol/LSerumcreanine450umol/LSerumalbumin29g/LDipstickurineBlood+Protein+Whatisthediagnosis?

A.IdiopathicthrombocytopenicpurpuraB.MyelodysplasticsyndromeC.DisseminatedintravascularcoagulationD.HaemolyticuraemicsyndromeE.Aplasticanaemia

Answer:d)Haemolyticuraemicsyndrome.ThemostlikelydiagnosisisHaemolyticuraemicsyndromeduetodiarrhoeaassociatedwith E coli infection. A stool sample for culture and blood film are importantinvestigationstobeperformed.



HaematologyQ037:A60yearoldmanhasa severalmonthhistoryof lowerbackpainandweakness.Hisbloodtestsshow:Hb11.0g/dlMCV95flWCC2.5x10^9/lplatelets130x10^9/lPT13s(11.515.5)APTT28s(3040)urea26mol/lcreanine280mol/lsodium138mmol/lpotassium4mmol/lbilirubin38mol/lAST26U/lALP150U/lalbumin33g/ltotalprotein95g/lWhatisthemostlikelydiagnosis?

A.MultiplemyelomaB.MetastaticbladdercarcinomaC.LymphomaD.ParaglangliomaE.Chronicmyeloidleukaemia

Answer:a)multiplemyeloma.Multiplemyelomaismostlikely.Thereisraisedprotein(6080normalrange)indicatedprobably paraproteinaemia. There is also lowwhite cell count due to bonemarrowinfiltration,andrenalfailure.Thesymptomsofbonepainalsosuggestsinfiltration.



HaematologyQ038:A 20 yearold girl is being invesgated for anaemia.Her father has previously had asplenectomy.Herbloodfilmshowsspherocytesandanaemia.Inviewofthelikelydiagnosis,whichisthemostusefulinvestigation?

A.ReticulocytecountB.MottcellC.HaemosidnerinuriaD.HaptoglobinE.IgGandC3complement

Answer:E)IgGandC3complement.Thispatient is likelytohavehereditaryspherocytosis inviewofthefamilyhistoryandthatthefatherhashadsplenectomyastreatment.However,raisedreticulocytecountanddecreasedhaptoglobinsandincreasedhaemosidurinuriawillbeallbepresentasasingletest,unhelpful.TheDirectAntiglobulinTestisusedtodetectIgGorC3boundtothe surface of the red cell. In this scenario, it is helpful to exclude autoimmunehaemolyticanaemia,sincespherocyteswouldalsobepresentonthebloodfilminAIHA.



HaematologyQ039:A34 yearold ladyhas apasthistoryof anepisodeofdeep vein thombosis and twomiscarriages.ShepresentsnowwithfurtherepisoderofDVT.Shehadathrombophiliascreenandwasfoundtohaveapositiveanticardiolipinantibody.Whatisthebesttreatment?

A.ClopidogrelB.Warfarin3monthsC.LongtermlowmolecularweightheparinD.AspirinandWarfarinE.Lifelongwarfarin

Answer:E)LifelongwarfarinThispatientwithrecurrentDVTshasthepresenceof lupusanticoagulant.Sherequireslifelongwarfarintreatment.



HaematologyQ040:A 40 year oldmalewho has a rheumatoid arthritis is admittedwith a urinary tractinfection.Resultsshow:haemoglobin7.5g/dL(1116)whitecellcount1.5x10^9/L(411)platelets70x10^9/L(150400)Whichoneofthefollowingdrugsisthemostlikelycauseofpancytopenia?

A.AzathioprineB.CyclophosphamideC.PrednisoloneD.CyclosporinE.Chloroquine

Answer:a)Azathioprine.Azathioprine is a thiopurine analogue drug which is metabolised in the liver tomercaptopurine. Themain side effects arebonemarrow suppression (may lead to apancytopenia)andalsodruginducedhepatitis.



HaematologyQ041:A 50 year oldman presentswithmulple bruises in the arms aerworking in thegarden.Invesgaonsshowed:Hb13.2g/dl,WCC5x10^9/l,plateletcount5x10^9/l.A bone marrow examination showed normal numbers of megakaryocytes and adiagnosisofidiopathicthrombocytopenicpurpurawasmade.Whatisthemostappropriatetreatment?

A.TranexemicacidB.OralprednisoloneC.BloodtransfusionD.PlatelettransfusionsE.Intravenousimmunoglobulin

Answer:b)oralprednisolone.Themostappropriate treatment for thispatientwho is symptomatic from ITP isoralprednisolone. If the bleeding becomes severe, then IV immunoglobulin should beconsideredinadditiontothesteroids.



HaematologyQ042:An12yearoldgirlhasrecurrentepistaxis.HerinvesgaonsshowHb11g/dl,Plts300x10^9/l,PT16sec(1316sec),APTT95sec(2838sec).Whichofthefollowingdeficienciesismostlikely?

A.FactorVdeficiencyB.FactorVIIdeficiencyC.VonWillebrand'sfactorD.AnticardiolipinantibodyE.FactorXdeficiency

Answer:c)vonWillebrand'sfactor.vonWillebrand'sdiseasewouldbemost likelydue to theprolongedAPTT, the restofthefactors(sameonesasthosewhichwarfarinacton)prolongPT.



HaematologyQ043:A70yearoldmanisonlifelongoralancoagulaonforrecurrentDVT.Hepresentswithminor bleeding from his gums for 1 day. His INR is 9.0. All other investigations arenormalandheisotherwisewell.Whatisthemostappropriatecourseofaction?

A.Stopwarfarin,monitorINRandrestartwhenINR



HaematologyQ044:A 65 year old lady has a diagnosis of Non Hodgkin's lymphoma and has recentlycommenced chemotherapy. She now complains of feeling breathless and unwell.Onexamination,sheispaleandslightlyjaundiced.Shehassplenomegaly.Investigationsshow:Hb3.5g/dlMCV106flWCC8x10^9/lplatelets250x10^9/lReculocytes125x10^9/l(N2090)HerFBCprechemotherapywasnormal.Whatisthemostlikelyexplanationforthis?

A.ParoxysmalcoldhaemoglobinuriaB.BonemarrowsuppressionC.MegaloblasticanaemiaD.AutoimmunehaemolysisE.Paroxysmalnocturnalhaemoglobinuria

Answer:d)Autoimmunehaemolysis.Anaemia,raisedMCVandhighreticulocytecountsuggestshaemolysis.ThismayoccursecondarytoNHL.Inaddition,thereisastrongassociationwithfludarabine.



HaematologyQ045:Apatienthashadasplenectomybecauseofhereditaryspherocytosis.Howlongshouldpenicillinprophylaxisbeused?

A.DuringacuteinfectionsB.1yearC.10yearsD.15yearsE.Lifelong

Answer:e)lifelong.Followingsplenectomy,patientsshouldreceivelifelongpenicillinprophylaxis.Themajorcomplicationofsplenectomy isoverwhelmingsepsiswithencapsulatedbacteria(eg,Spneumoniae,Hinfluenzae,Nmeningitidis).Theoverallriskofsepsisinasplenicpatientsisapproximately2%butvariesdependingontheageandunderlyingdiseases.



HaematologyQ046:A13yearoldchildhashadrecurrentepisodesofbonepain.Hehasbeenadmittedtohospital several times due to severe pains in the last 5 years. He has X rayswhichshownecrosisofthehip.Whichofthefollowingdiagnosisislikely?

A.MultiplemyelomaB.Paget'sdiseaseC.OsteopetrosisD.SicklecelldiseaseE.Thalassemia

Answer:d)sicklecelldisease.Aseptic necrosis of the hip, cholecystitis, renal papillary necrosis and proliferativeretinopathyareclinicalfeaturesofsicklecelldisease.



HaematologyQ047:A20yearoldwomanwithsicklecellanemiapresentswithacuteshortnessofbreath.Achestx rayobtainedwithaportableunit initiallyshowednoabnormalitiesexcept forbibasilarhazyopacities.Fiveandahalfhoursafteradmission,heroxygen saturaondecreased to76percentwitha respiratory rateof24breathsperminute.A repeat chest radiograph revealedincreasedinterstitialmarkings.Howshouldshebetreated?

A.AntibioticsandfluidsB.IntubationandventilationC.NoninvasiveventilationandplasmaexchangeD.SplenectomyE.Highflowoxygen

Answer:c)noninvasiveventilationandplasmaexchange.This is a case of acute chest syndrome related to sickle cell anaemia.Non invasivveventilation(CPAP)andplasmaexchangeisthebestoption,oftenalongwithantibioticsbecausethechestsyndromecanbeprecipitatedbyinfection.



HaematologyQ048:A 25 year old female has had her first DVT when she started taking the oralcontraceptivepill.SherevealsthathermotherhasalsohadDVTbefore.Whichofthefollowingisshelikelytohave?

A.FactorVleidendeficiencyB.ProteinCdeficiencyC.ProteinSdeficiencyD.AntithrombinIIIdeficiencyE.Lupusanticoagulant

Answer:a)factorVleidendeficiency.Although they are all possibilities, the family history suggests factor V leiden orantithrombin III deficiency.A femalewho hasDVT precipitated by theOCP suggestsfactorVleidenmoresothanantithrombinIII(malewouldsuggestthis).



HaematologyQ049:A32yearoldladyhadanewbornbabywithmarkedjaundice.Serumbilirubinwas359mmol/Landhaemoglobinlow.Themotherhashadonepreviousnormaldelivery.Haemolyticdiseaseofthenewbornwassuspected.Whichofthesestatementsislikely?

A.FatherisORhveB.FatherisABRh+veC.MotherisABRh+veD.FatherisABRhveE.MotherisORh+ve

Answer:b)FatherisABRh+veToAnswerthisquestion,ABOgroupislessrelevantandRhstatusisrelevant.Rhgroupingof foetus isdecidedby theRh statusof the father.The first childwouldhavebeenRh+veand led to sensitisation (antibodiesdevelopedby themother).Themother is Rh ve and the father is Rh +ve (who could also be homozygous orheterozygousforRh).



HaematologyQ050:A35yearoldmancomplainsoflegcrampsandisgivenquinineforthefirsttimebyhisGP. He then presents unwell and complains of dark urine. hisHb is 7.4 g/dl. Directantiglobulintest(DAT)testisnegative.Whichofthefollowingislikely?

A.G6PDdeficiencyB.AutoimmunehaemolyticanaemiaC.ParoxysmalcoldhaemoglobinuriaD.PhosphokinasedeficiencyE.Sicklecelldisease

Answer:a)G6PDdeficiency.The direct antiglobulin test (Coomb's) is negative and suggests that this is notautoimmune since there is no antibody opsonisation on red cells. Quinine canprecipitatehaemolysisinG6PDdeficiencyascanaspirin,sulphonamides,favabeansandantimalarialagents.



HaematologyQ051:A40yearold ladywithpresentswithchronicdiscomfort in leftupperquadrantoftheabdomen.Investigationsshow:Hb16.9g/dlMCh55(2832)pgMCV69flWhitecellcount11x10^9/lPlatelets490x10^9/lWhatistheunderlyingcause?

A.EssentialthrombocythaemiaB.PrimarypolycythaemiaC.RenalcellcarcinomaD.MyelodysplasiaE.Chronicmyeloidleukaemia

Answer:B)primarypolycythaemia.The raisedHb,white cell andplatelet count are consistentwithpolycythaemia rubravera.Splenomegaly is common,andoccasionally splenic infarctionaswellwhichmaylead to left upper quadrant pains. Essential thrombocythaemia is associated withanaemia.

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HaematologyQ053:An12yearoldboybleedsexcessivelyafteralaceration.Investigationsshow:Hb13.5g/dlWBC5.8x10^9/lPlts270x10^9/lPT15sec(1316sec)APTT85sec(2838sec)FactorVIIIandFactorIXlevels:normalAPTT50:50mixwithnormalplasma:37secWhichofthefollowingisthemostlikelydiagnosis?

A.HaemophiliaAB.FactorXIdeficiencyC.FactorXdeficiencyD.FactorXIIdeficiencyE.FactorVIIdeficiency

Answer:b)FactorXIdeficiency.An isolatedprolongedAPTTwillbecausedbydeficiencies in factorsVIII, IX,XIandXIIandbyvonWillebrandsdisease.FactorXIIdeficiency isnotassociatedwith increasedbleeding.With normal Factor VIII and IX levels, this patient is most likely to have Factor XIdeficiency.FactorXIdeficiencyisamildbleedingdisorderwithautosomalcodominantinheritance.DiagnosisisbyspecificFactorXIlevelestimation.



HaematologyQ054:A 48 year old man has a diagnosis of acute myeloid leukaemia. He was givenchemotherapy.A fewweeks into induction chemotherapy, he develops jaundice andfevers.Bloodculturesdidnotgrowanyorganisms.Despite intravenousantibiotics,thepatientremainedpyrexial.Whatisthelikelycause?

A.CMVB.CandidiasisC.MetastaticdiseaseD.TuberculosisE.Rubella

Answer:A)CMVThelikelycauseofpersistingpyrexiaplushepatitisinthisimmunocompromisedpatientaftertreatedwithappropriateantibioticswouldbeaCMVinfection.CMVinfectioncanalso cause a pneumonitis and colitis. Treatment with an antiviral agent such asganciclovircouldbeconsidered.

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HaematologyQ058:A35yearoldwomanpresentswithjaundiceandlethargy.Investigationsreveal:Haemoglobin8.5g/dL(1116)Whitecells7x10^9/lPlatelets190x10^9/lreticulocytecount130x10^9/L(2585)serumbilirubin55umol/L(120)BloodfilmshowsspherocytesWhichofthefollowingshouldbedone?

A.DirectantiglobulintestB.UltrasoundoftheabdomenC.BonemarrowbiopsyD.BonemarrowaspirateE.G6PDenzymelevel

Answer:a)directantiglobulintest.One of the first tests to consider in a patientwith haemolytic anaemia is the directantiglobulintest(Coomb's).Thistoexcludeautoimmunehaemolyticanaemia.



HaematologyQ059:A42yearoldwomanhasahistoryofpositivelupusanticoagulant.Shehadapulmonaryembolus diagnosed 8 years ago, and two presentationswhichwere consistentwithdeepveinthrombosis6and12monthsgo.Whatisthebestmanagement?

A.HighdoseaspirinB.LifelongwarfarinC.AntenataladviceD.AvoidanceoforalcontraceptivepillE.6monthsofwarfarinthenreassess

Answer:b)lifelongwarfarin.Morethanonethromboticeventwiththepresenceoflupusanticoagulantsuggeststhatthepatientrequireslifelongwarfarin.



HaematologyQ060:A 65 year oldwoman has a haemoglobin of 5.5 g/dl. She has lethargy but no othersymptoms.Herbloodfilmshowsovalmacrocytesandhypersegmentedneutrophils.Shehasahistoryofhypothyroidismandisonthyroidreplacement.Shealsohasvitiligo.Whatisthemostlikelydiagnosis?

A.MultiplemyelomaB.MyelodysplasiaC.PerniciousanaemiaD.IrondeficiencyanaemiaE.Haemolyticanaemia

Answer:c)Perniciousanaemia.Pernicious anaemia (PA) is a disease of the stomach that is characterised bymegaloblascanaemiaduetovitaminB12deficiency. It issecondaryto intrinsicfactordeficiencyandgastricatrophy.Itusuallyhasanautoimmunebasis.PerniciousAnaemiaprimarily affects the elderly most patients are over 60 years of age.Women areaectedmoreoenthanmen,inaraoof3:2.Itmaybeassociatedwithautoimmunediseases,suchasAddison'sdisease,hypothyroidismandalsoanincreasedriskofgastriccarcinoma.



HaematologyQ061:A40yearold ladyhasbeenonwarfarinforpreviousDVT.She isnowbreathlessandaCTPAconfirmspulmonaryembolusdespiteher INRsbeing intherapeucrangeof23.Sheisalsohyponatraemicwithasodiumof129mmol/l.Withtheshortsynacthentest,shehasalowcorsolof80nmolat0mingoingupto200nmolat30min.Whichofthefollowingdiagnosisislikely?

A.AutoimmunepolyendocrinesyndromeB.AdrenaltumourC.ProteinCdeficiencyD.FactorVleidendeficiencyE.Presenceoflupusanticoagulant

Answer:e)presenceoflupusanticoagulant.Antiphospholipid syndrome ismost likelydue to the recurrent thrombotic tendency,lupus anticoagulant or anticardiolipin antibodiesmay be present. It has a propensitytowardsadrenalveinthrombosisandcancausehypoadrenalismasinthiscase.



HaematologyQ062:A 20 year old girl receives a blood transfusion. 5 minutes aer the transfusion iscommenced,shedevelopsatachycardiaandabdominalpains.Whichof the following is thecorrectmanagementofanacutehaemolytic transfusionreactionduetoABObloodgroupincompatibility?

A.StoptransfusionandassessB.RepeatcrossmatchandretransfuseC.IntravenousdextroseD.Hydrocorsone100mgintravenouslyE.Continuetransfusionslowly

Answer:a)StoptransfusionandassessThe immediate treatmentofanacutehaemolytic transfusion reactiondue toamajorblood group incompatibility is todiscontinue theblood transfusion immediately. Thisshouldbe followedbyassessment forpossibleshockandresuscitationwith fluidse.g.colloids.



HaematologyQ063:A35yearoldmanhasrecentlybeencommencedonlowmolecularweightheparinandthenwarfarinfollowingadiagnosisofDVT.Whichofthefollowingiswellknownlongtermsideeffectofheparin?

A.PolycythaemiaB.VisuallossC.RenalimpairmentD.OsteoporosisE.Hirsutism

Answer:d)osteoporosis.Heparin induced thrombocytopenia,osteoporosisand thrombosiscanoccur.Warfarincancauseskinnecrosis.



HaematologyQ064:A patientwho is known to have hereditary spherocytosis and hasmild jaundice andgallstonesisawaitingsplenectomy.Howlongpriortosplenectomyshouldpneumococcalvaccinationbeadministered?

A.1dayB.5daysC.3weeksD.3monthsE.6months

Answer:c)3weeks.Pneumococcal immunisation shouldbeadministered to thepaent24weeksbeforesplenectomy.



HaematologyQ065:A28yearoldwomanhashadadiagnosisofpulmonaryembolus.Shehasthefollowinginvestigations:Haemoglobin11.3g/dl,whitecellcount4.0x10^9/L,plateletcount45x10^9/L.Whichofthefollowingdiagnosesismorelikely?

A.HomocystinuriaB.ProteinCdeficiencyC.FactorVleidendeficiencyD.AntiphospholipidsyndromeE.ProteinSdeficiency

Answer:d)antiphospholipidsyndrome.Apartfromathrombotictendency,antiphospholipidsyndromeisassociatedwithalowwhitecellcountandthrombocytopenia.



HaematologyQ066:A28yearold lady is30weekspregnantwhenshepresentswitha leftsidedDVT.Shehashadapreviousmiscarriagebefore.Herinvestigationsshow:Hb10.2g/dlMCV68flWBC8.0x10^9/lPlts250x10^9/lDirectCoomb'sTest:positiveReculocytecount90x10^9/l(25125)APTT51sec(normal2838sec)PT16sec(normal1316sec)ProteinCacvity0.75iu/ml(0.671.38)TotalproteinS100%(64154)Whichofthefollowingdiagnosisislikely?

A.HeparininducedthrombocytopeniaB.ProteinCdeficiencyC.ProteinSdeficiencyD.FactorVleidenE.Antiphospholipidsyndrome

Answer:e)antiphospholipidsyndrome.Of the following choices, antiphospholipid syndrome is most likely because of therecurrentthrombotictendency.RaisedAPTTandpositiveDirectCoomb'stest(measurespresenceofantibodiesonredcells)canbecausedbylupusanticoagulant.



HaematologyQ067:A32yearoldwomanpresentswithbleedinggumsandeasybruising.Hermedicationsarelansoprazoleandcisprideforrefluxoesophagitis.Investigationsshow:Haemoglobin12.5g/dL(13.016.5)MCV90fl(8395)Platelets35x10^9/L(150400)Bloodfilm:occasionalgiantplateletsProthrombinme12s(11.515.5)Whatisthelikelydiagnosis?

A.DisseminatedintravascularcoagulationB.ImmunethrombocytopeniaC.ThromboticthrombocytopenicpurpuraD.MegakaryocyticthrombocytopeniaE.Druginducedthrombocytopenia

Answer:b)Immunethrombocytopenia.This isa caseof immune thrombocytopenia inwhich lowplateletswithother countsbeingnormalapartfromslightanaemia(duetobleeding).



HaematologyQ068:Achildhassevereanaemiaandbeendiagnosedashavingthalassemiamajor.Whichof the following is themajor formofhaemoglobinpresentwhen the conditionexists?

A.HaemoglobinA2B.HaemoglobinCC.HaemoglobinFD.HaemoglobinHE.HaemoglobinA

Answer:c)HaemoglobinF.In Betathalassaemiamajor there is a complete defect in production of beta globinchains,which leadsto impaired formaonofHbA (which ismadeupof2alphaand2betachains).HaemoglobinFisthemajorhaemoglobinasthishaemoglobinismadeupofalphaandgammachains.Patientswiththalassemiamajorrequire lifelongbloodtransfusions(hypertransfusions)withironchelationtherapy(desferrioxamine).

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HaematologyQ070:A60yearoldmanhasbeenonwarfarinforaDVT.HehadanINRof9duringafollowupappointmentwhenhewasnoticedtohavehaematuriaandepistaxis.Whatistheappropriatemanagement?

A.2unitsbloodtransfusionB.2unitsofplateletsC.Freshfrozenplasma1unitD.StopwarfarinandobserveE.0.5mgofvitaminKiv

Answer:e)0.5mgofvitaminKiv.The patient has minor bleeding. According to The British Society of Haematologyguidelines,when INR is>8withminorbleeding,warfarinshouldbediscontinueduntiltheINRis



HaematologyQ071:A55 yearoldmanhashad varicose vein repair.Hedevelops a swollen leg fivedaysfollowingsurgeryandanultrasoundofthelegconfirmsaDVT.Thisisdespitehimhavingprophylacticlowmolecularweightheparin.HisHbis11g/dl,WCC13x10^9/l,plateletcountis45x10^9/l.WhichofthefollowingcouldhavecausedtheDVT?

A.Behcet'sdiseaseB.FactorVleidendeficiencyC.ProteinCdeficiencyD.ImmunethrombocytopenicpurpuraE.Heparininducedthrombocytopenia

Answer:e)heparininducedthrombocytopenia.TypeIheparininducedthrombocytopenia(HIT)occurswithinafewdaysofheparinandisusuallymild.In this case, type IIHIT ismore likely, and this occurs slightly later (515 days). It isassociatedwiththrombosisandalowplateletcount.Alternativeanticoagulationshouldbeused(hirudin,danaparoidsodium).



HaematologyQ072:A55yearoldmanhasG6PDdeficiency.Hepresentswithahaemolyccrisisaeradrugwasstarted.Whichoneofthefollowingisprobable?

A.CarbamazepineB.GentamicinC.ParacetamolD.ChloramphenicolE.Phenytoin

Answer:d)Chloramphenicol.Haemolysis inG6PD deficiency is due to oxidave damage (decreased generaon ofNADPHduetoenzymedeficiency).Thecommoncategoriesofdrugsare:SulphonamidesAntimalarialsAntipyretics(aspirin+paracetamol)Others:Chloramphenicol,nitrofurantoin,Dapsone,Probenecid,VitK



HaematologyQ073:A32yearoldwomanwhois20weekspregnantpresentedwithlethargy,confusionanddrow siness.Onexamination shehasbilateral legweakness and apurpuric rashwasnoticedonbothlegs.Investigationsshowed:Hb8.2g/dlWCC7.2x10^9/lplatelets25x10^9/lreculocytes3%Urea28mmol/lCreanine360umol/lBloodfilmshowed:fragmentedcellsandpolychromasiaWhattreatmentshouldbeadministered?

A.DexamethasoneB.PlasmaexchangeC.PlatelettransfusionD.CyclophosphamideE.Bloodtransfusion

Answer:B)plasmaexchange.Thispatienthasthromboticthrombocytopenicpurpuraassuggestedbyhaemolysisonthebloodfilm,anaemia,thrombocytopenia,renalfailureandalsoneurologicalfeatures.Treamentof choice isplasmaexchangewith fresh frozenplasma infusion.Highdosesteroidsmayalsobebeneficial.Plasmaexchangeremovesantibodieswhichisthemainpathogenicprobleminthedisease.



HaematologyQ074:A23yearoldwomanpresentedveryunwellwithamiscarriage.Onexamination, shewasverypaleandbreathless.Shehadabloodpressureof90/60mmHg.Investigationsrevealed:Haemoglobin9g/dLPlatelets52x10^9/LProthrombinme20sec(1115)APTT55sec(3040)Fibrinogen0.3g/L(4)Bloodfilm:FragmentedcellsWhatshouldbeadministered?

A.IntravenoushydrocortisoneB.VitaminCC.TranexemicacidD.DDAVPE.Cryoprecipitate

Answer:e)cryoprecipitate.This is a case of disseminated intravascular coagulation (DIC) in which fibrinolyticsystembecomesactivated,leadingtothrombinformation.Unregulatedfibrinolysisandsystemicfibrinogenolysisoccurswithreleaseofplasminintothecirculation.Typically, theblood filmshows fragmentedredbloodcells.Treatmentaims tocorrectthecoagulopathywithbloodproductse.g.cryoprecipitatetoreplacefibrinogen,orfreshfrozenplasma.VitaminKcanalsobegivenineventofsignificantbleeding.



HaematologyQ075:A55yearoldpaentisknowntohavealcoholicliverdisease.Hedrinks15pintsofbeeradayandhasoesophagealvariceswhenhehadendoscopy3monthsago.Henowhasmelaenawiththefollowingbloodresults:Hb7.5g/dlMCV103flWCC11x10^9/lplatelets100x10^9/lPT20s(11.515.5)APTT40s(3040)Fibrinogen0.8.0g/L(1.85.4)urea17mol/lcreanine105mol/lsodium130mmol/lpotassium4mmol/lbilirubin62mol/lAST328U/lALP200U/lalbumin32g/lApartfrombloodtransfusion,whichofthefollowingwouldbeuseful?

A.FactorVIIIB.CryoprecipitateC.ExchangetransfusionD.HaemodialysisE.Albumin

Answer:b)cryoprecipitate.Withalcoholicliverdisease,thereisaprolongedPTandlowplateletcount.However,insevere alcoholic liver disease fibrinogen can also be lowas in this case, thuscryoprecipitatewouldbeuseful.



HaematologyQ076:A50yearoldmanpresentswithhypertension.Furtherbloodtestsrevealthefollowing:Hb18.6g/dl,WCC16x10^9/l,plts600x10^9/l.Theerythropoienlevelisnormal.Whatisthemostlikelydiagnosis?

A.SecondarypolycythaemiaB.PolycythaemiarubraveraC.MyelofibrosisD.Gaucher'sdiseaseE.RecombinantEPOuse

Answer:b)polycythaemiarubravera.Inpolycythaemia rubra vera, theHb,WCC andplatelet counts arehigh alongwith anormalEPOlevel.EPOisraisedinsecondarypolycythaemia(e.g.hypoxia).



HaematologyQ077:An80yearold ladycomplainsofmildbreathlessnessand lethargy.Shemenons thatshe is a vegetarian.There isnohistoryofhaemetemesisormelaena. Shehas apastmedicalhistoryofcongestivecardiacfailure.Onexaminaon,she ispale,andhasviligoonherhands.ShehasaJVPof+4cmandfineinspiratorycrepitations.Herinvestigationsshow:Hb4.5g/dl MCV105WBC3.3x10^9/l Plts120x10^9/lWhatisthemostimportantinitialmanagement?

A.BloodtransfusionB.StartvitB12andfolicacidC.RedcellmassstudiesD.BonemarrowaspirateE.Schillingtest

Answer:b)StartvitB12andfolicacid.PancytopeniaandraisedMCVsuggestssevereB12orfolatedeficiency.Vitiligoisalsoaclueastoautoimmunephenomenon,andperniciousanaemiamaybeassociated.Inthislady,bloodtransfusionmayexacerbatecardiacfailure,andsheisnotactivelybleeding,hencereplacementofB12andfolate isabetteroption(symptomswill improvewithin12weeks).B12deficiencycanoccurasaresultofperniciousanaemia (intrinsic factordeficiency),dietarye.g.vegetarian,Crohnsdisease,Serum folate level less than5ng/mlorserumvitaminB12levellessthan100pg/mlisdiagnostic.Folatedeficiencyistreatedbygivingfolicacidorallyat1to5mgdaily.B12deficiencyisusuallytreatedbyparenteraladministraonofB12.Thetreatmentscheduleconsistsofgiving1000gcobalaminintramuscularlydailyfor1014daysfollwedby1000gonceaweek till hematocrit becomes normal followed by 1000 g once amonth for life inpatients with pernicious anemia or those withmalabsorption. Therapeutic doses offolatewillcorrect thehematologicabnormalitiesduetocobalamindeficiencyalsobuttheneurologicabnormallitiescanworsen, it isbest togiveB12firstorbothB12andfolatebutneverfolatealone.



HaematologyQ078:A50yearoldwomanpresentedwitha fiveyearhistoryofpain inthemiddleofbothfeet.Shealsohadahistoryofbackpain,paininbothsidesofherhip,andpaininbothmetacarpals.Shehadaserumferritinconcentrationof1087g/l,withnormalresultsinliver function tests and a normal glucose concentration, full blood count, anderythrocytesedimentationrate.Whatisthediagnosis?

A.Wilson'sdiseaseB.OchronosisC.MarblebonediseaseD.HaemochromatosisE.Thalassemia

Answer:d)Haemochromatosis.Haemochromatosishasanautosomalrecessivepatternof inheritanceandaects1 in250ofthenorthernEuropeanpopulaon,withupto10%ofpeoplecarryingthegene.Inheritance of the disease has long been associatedwith the ssue type HLA A3. Aspecificmutaonofthegene,C282Yiscommon.Theclinicalpresentationofhaemochromatosisisvariableandnotconfinedtotheclassictriadofcirrhosis,diabetes,andskinpigmentation. Inthiscasethepresentation iswitharthropathy.



HaematologyQ079:A56yearoldmanwasdiagnosedwithmyelofibrosis.Whichofthefollowingisthemostcommonpresentationofthedisease?

A.BleedingB.RespiratorypainC.HyperuricaemiaD.FatigueE.Bonepain

Answer:D)fatigueClinicalfeaturesofmyelofibrosisinclude:usually develops in adults over age 50 patients commonly presentwith fatigue andweaknessspleenisoftenmassivelyenlargedhepatomegalyoccursinoverhalfofcases



HaematologyQ080:A 60 year old woman was admied with a 10month history of rash, fatigue,intermittent hemoptysis, and purpura. On admission, widespread petechiae andpurpurawith scleral icteruswere noted.No lymphadenopathy or splenomegalywaspresent.Investigationsshowed:plateletcountof310^9/L hemoglobinlevel5.5g/dLmeancorpuscularvolume103fL whitecellcount3.610^9/Lneutrophilsat0.67withoutmyeloidblasts reticulocytecountwas0.20serumurea15.4mmol/L(43mg/dL) creanine,239mol/Llactatedehydrogenase(LDH)2505U/L totalbilirubin19mol/L(4.6mg/dL) haptoglobin,6g/LReviewoftheperipheralsmearrevealednotableredcellmorphologyand24nucleatedredbloodcellsper100whitebloodcells,manyofwhichweredysplastic.Whatisthemostlikelydiagnosis?

A.IdiopathicthrombocytopenicpurpuraB.ThalassemiaC.SicklecelldiseaseD.MyelodysplasiaE.Chronicmyeloidleukaemia

Answer:d)myelodysplasia.~Thereisagradualhistoryofprogressionandthepatienthasapancytopenia,sheisalsoin the right age group formyelodysplasia.Myelodysplasia can be classified into fivesubtypesRefractoryanaemiaRefractoryanaemiawithringsideroblastsRefractoryanaemiawithexcessblastsRefractoryanaemiawithexcessblastsintransformation(nearAML)CML.Few patients require aggressive therapy such as chemotherapy, it is reserved foryounger patients to prevent progression to AML. Supportive therapy includes bloodtransfusions,platelettransfusionsorGCSFtoimprovebloodcounts.Howevermediansurvivalisonly2years.



HaematologyQ081:An18yearoldgirlpresentswithepistaxis.She isfoundtohaveaprolongedAPTT.HerMotherhashadpreviousbleedingepisodeswithsimilarcoagulationtestresults.Whatisthemostlikelydiagnosis?

A.FactorVIIdeficiencyB.FactorVdeficiencyC.ProteinCdeficiencyD.VonWillebrand'sdiseaseE.HaemophiliaB

Answer:d)vonWillebrand'sdisease.The APTT is a general clotting screen which detects defects in the intrinsic clottingpathway (factors XII, XI, IX, and VIII, towhich VonWillebrand factor is linked). VonWillebrand's disease is a predominantly autosomal dominant condition which isassociatedwithableedingtendency,whichisusuallymildandwithaprolongedAPTT.TheteststodiagnosevWDinclude:bleedingtime(prolonged)factorVIIIleveltest(measurestheleveloffactorVIIIanditsabilitytofunction)vonWillebrandfactorantigentest(thedisorderisconsideredmildifapersonhas20%to40%ofthenormalamount,severeiftheamountislessthan10%ofnormal)ristocetin cofactor activity test (measures how well the von Willebrand factor isworking)



HaematologyQ082:Apatientpresentswithacutepromyelocyticleukaemia.Whatisthelikelymechanismunderlyingleukaemogenesis?

A.Aberrantfusionof2genesB.PosttranslationalmodificationC.OverexpressionofcellularoncogeneD.ImpaireddegradationofproteinE.Shorttelomere

Answer:A)aberrantfusionof2genesAcutepromyelocyc leukaemia is frequentlydue to chromosomal translocaon t (15;17).



HaematologyQ083:A 15 year old male comes to the hematology clinic for his specialty care for vonWillebrandDisease.ThepastmedicalhistoryrevealsthathewasdiagnosedwithType1vonWillebrandDisease as a toddler after abnormal bruising and prolonged bleedingwasnotedbyhisfamily.Whattreatmentisrecommendedifheweretohaveasignificantepisodeofbleeding?

A.FreshfrozenplasmaB.FactorXC.FactorIXD.AspirinE.DDAVP

Answer:e)DDAVP.Treatmentmay includeDesmopressin (DDAVP),FactorVIIIand tranexemicacid invonWillebrand'sdisease.

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HaematologyQ085:A25yearoldwomanhasthefollowinginvestigationsattheantenatalclinic:Hb10.3g/dlWBC5.6x10^9/lPlts290x10^9/lMCV69flMCH17.2Iron20(1429)mol/lFerrin150(15200)mol/lWhatisthemostusefulinvestigation?

A.MyelomascreenB.HaemoglobinelectrophoresisC.FolatelevelsD.HbFlevelE.HbA2level

Answer:e)HbA2level.Iron deficiency anaemia and thalassaemia trait are the most likely diagnoses ofmicrocytic anaemia. Irondeficiency isunlikely in this case in viewof the iron studiesbeingnormal.BetathalassaemiatraitisdiagnosedbythepresenceofaraisedHbA2.Ifbothconditionsareexcluded,thenalphathalassaemiaisthemostlikelydiagnosis.



HaematologyQ086:A30yearold ladywithvonWillebrand'sdisease isdue tohaveplascsurgery toherfaceandseeksadvice from thehaematologist.Shementions thatshehasahistoryofepistaxisandbleedinggums.Whichofthefollowingisthemostusefulassessmentofhercoagulationstatus?

A.ProthrombintimeB.ActivatedpartialthromboplastintimeC.ThrombintimeD.BleedingtimeE.FactorVIIIactivityassay

Answer:e)factorVIIIactivityassay.AlthoughbleedingtimeisprolongedinvonWillebrand'sdisease,thefactorVIIIactivityassaywill give ameasurement of the severity of her disease. The other useful testswould be the ristocetin cofactor assay and vWF antigen assays for vonWillebrand'sdisease.

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HaematologyQ088:A 25 year old ladywhowas pregnantwas treated for a deep vein thrombosiswithintravenousheparin.Arecenttestshows:Haemoglobin10.2g/dLWhiteCellCount8x10^9/lPlatelets32x10^9/lWhatisthebestcourseofactionthiswoman?

A.ChangetoclexaneB.CommencewarfarinC.ChangetoaspirinD.ChangetodanaparinoidE.Continueivheparin

Answer:D)changetoDanaparinoidThispatienthasHeparinInducedThrombocytopaemia.WhenHITissuspected,heparintreatment should be converted to danaparoid , which is a low molecular weightheparinoid.Itisusuallygivenasanintravenousinfusion



HaematologyQ089:A 65 year old man complains of breathlessness and redness. He has confirmedpulmonaryemboli.HisHbis18g/dl,WCCis15x10^9/landplateletcountis70010^9/l.Whichofthefollowingcanbehelpfulinconfirmingthediagnosis?

A.BloodfilmB.BonemarrowbiopsyC.RedcellmassD.NAPscoreE.Kleihauertest

Answer:c)redcellmass.Thediagnosisispolycythaemiarubraveraandthiscanbeconfirmedbyaraisedredcellmass.NAPscoreisdecreasedinCML.AKleihauertestisusedtoconfirmtransplacentalbloodlossfromfetustomother.



HaematologyQ090:A50yearoldmanwithnonHodgkin'slymphomaisonRituximab.WhichofthefollowingantigensdoesRituximabhaveanactionon?

A.CD8B.CD8C.CD19D.CD20E.CD154

Answer:d)CD20.RituximabisananbodytoCD20expressedonBcellsandisusedinBcelllymphomas(to try to cause cell lysis). The receptor is present inmore than 90% of Bcell nonHodgkin's lymphomas. Molecules that aach to CD20 can aect the growth anddevelopment of the tumor cells. Rituximab is an antibody thatwas developed usingcloningandrecombinantDNAtechnologyfromhumanandmurinegenes.



HaematologyQ091:A32yearoldwhoisknowntohaveITPpresentswithbleedingofhergums.Herplateletcountnormallyrunsat87x10^9/lbutnowhasdroppedto42x10^9/l.Whatisthebestmanagementplan?

A.ObservationB.SteroidsC.PlatelettransfusionD.FFPE.Wholebloodtransfusion

Answer:B)steroidsChronic ITP rarely resolves spontaneously. First line treatment iswith prednisolone.Patients with chronic ITP who require surgery may be given intravenousimmunoglobulins which produce a transient rise in platelet count by blocking Fcreceptorsonsplenicmacrophages.Platelet transfusion shouldbe givenonly in lifethreateninghaemorrhage toenhancehaemostasis.



HaematologyQ092:A45yearoldwomanpresentswithanupperGIbleedandrequiresabloodtransfusion.Halfwaythroughthefirstunitofblood,sheexperiencesgeneraliseddiscomfort.Whatisthemostappropriatecourseofaction?

A.ChestXrayandabdominalxrayB.BloodculturesC.GiveanalgesiaandcontinueD.IVsteroidsE.StopthebloodtransfusionandgiveIVfluids

Answer:e)StopthebloodtransfusionandgiveIVfluids.Acutetransfusionreactionscancausegeneralizeddiscomfort,loinpainandpainatthecannulasitemayallprecedehaemoglobinuriaandrenalfailure.Ifatransfusionreactionis suspected, the transfusion should be stopped immediately and IV fluids shouldbeadministered,topreventshock.



HaematologyQ093:A55yearoldmanenquiresaboutrisksofbloodtransfusion.Whichofthefollowinginfectionsisbloodscreenedfor?

A.VaricellazosterB.HepatitisBC.CytomegalovirusD.MalariaE.Salmonella

Answer:b)HepatitisB.CMV,malariaandsalmonellacanallbetransmittedbybloodproducts.IntheUK,routinetestingfordonorbloodisfor:

HIVHepB&CSyphilisABO+RhD



HaematologyQ094:A 60 year oldman presentswith extensive bruising.He has a history of fague anddizzinessforthepastfewmonths.Onexamination,hehasapurpuricrashonhistrunkandlimbs.Investigationsshow:Hb7.5g/dlMCV105flWCC7x10^9/lplatelets100x10^9/lProthrombinme20(1217)sFibrinogen90(150460)mg/dLBloodfilm:50%blastcells.Whatistheclinicalpictureconsistentwith?

A.ErythroleukaemicreactionB.AplasticanaemiaC.DisseminatedintravascularcoagulationD.HaemolyticanaemiaE.Immunethrombocytopenicpurpura

Answer:C)disseminatedintravascularcoagulation.Theclinicaldiagnosis is likelytobeacutemyeloblastic leukaemia(AML).AMLsubtypesaredistinguishedfromotherrelatedblooddisordersbythepresenceofmorethan30%blasts in theblood,bonemarrow ,orboth.Oneof thecommoncomplications isDIC,whichresultsinanelevatedprothrombintime,decreasedfibrinogenlevelandincreasedfibrindegradationproducts.Acutepromyelocytic leukemia(APL),alsoknownasM3, isthemostcommonsubtypeofAMLassociatedwithDIC. In leucoerythroblasticpicture,nucleatedredcellsandwhitecellprecursorsarefoundintheperipheralblood.



HaematologyQ095:A60yearoldwomanhashadaprolongedITUstayduetoseverepneumoniaandsepsisrequiringmechanicalventilation.Shewasnoted tohaveworseninganaemia followingdischargefromITUat4weeks.HerHbis6g/dl,MCV109fl,WCC2.2x10^9/l,platelets110x10^9/l.Whatisthelikelycauseofanaemia?

A.UpperGIbleedB.AplasticanaemiaC.AcutemyeloidLeukaemiaD.ImmunethrombocytopenicpurpuraE.Acutefolatedeficiency

Answer:e)Acutefolatedeficiency.Apatientwhohasbeen in intensive care for a significantperiodmaynotbe gettingenough folate,especiallywith increasedneeds for recovery.Anacutedeficiencystatemaythusdevelop.Thiswouldprecipitateapancytopeniaandmacrocyticanaemia.



HaematologyQ096:A 20 year old female presents with severe colicky abdominal pain, voming andconspaonof3daysduraon.Shehadaprevioushistoryofadmissiontohospitalwithsimilarfeatures.Herabdominalxrayandultrasoundscanwerenormal.Shewastreatedwithantibiotics,analgesics and antiemetics. Her urine was discoloured and she had a tonic clonicseizurewhilstontheward.Whatisthelikelydiagnosis?

A.VariegateporphyriaB.AcuteintermittentporphyriaC.Fabry'sdiseaseD.Gaucher'sdiseaseE.Matureonsetdiabetesoftheyoung

Answer:b)acuteintermittentporphyria.Acute intermittent porphyria is autosomal dominant disorder caused by a defect inporphobilinogendeaminaseactivity. Ifperipheralneuropathy,suchaspain inthebackandlegsorparathesiasoccursitisalmostalwaysprecededbyabdominalpain.Otherautonomicneuropathies thatmaybe seenare sweating,vascular spasm, labilehypertension,and sinus tachycardia.Centralnervousdysfunction canbe seenaswellwithseizures,coma,bulbarparalysis,orcerebellarinvolvement.ThedefectinporphobilinogendeaminasecausesabuildupofALAandporphobilinogen(PBG)whichcausestheirincreasedsecretionintheurine.

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HaematologyQ099:A45yearoldwomanpresentswithhaemetemesis.Shehasahaemoglobin(Hb)of4.5g/dLandplateletcountof350x10^9/L.Whichofthefollowingisthemostappropriateproductordrugtouse?

A.Freshfrozenplasma(FFP)B.IvmethylprednisoloneC.PlasmaproteinfractionD.PackedredcellsE.Ironinfusion

Answer:D)packedredcells.Thepatientwho is anaemic andbleedingneeds ablood transfusionwithpacked redcells(blood).Thisalsocontainssomewhitecells,platelets&asmallamountofplasmaplus60100mlofaddive.



HaematologyQ100:A75yearoldwomanwithchronicmyeloid leukemia (CML),treatedwithhydroxyureaandinterferonfor12yearssufferedfromgradualdiseaseprogressionforoneyear.Investigationsshow:Haemoglobin11.6g/dLwhitecellcount47x10^9/L(neutrophils,80%;lymphocytes,13%;metamyelocytes,6%;blasts,1%)plateletcount1220x10^9/LWhatshouldthepatientbetreatedwith?

A.CyclophosphamideB.PrednisoloneC.RadiotherapyD.DesferrioxamineE.Imatinib

Answer:e)Imatinib.Gleevec(imatinibmesylate,Novartis),isanoraldrugwhichinterfereswiththeactionoftheabnormalBcrAbltyrosinekinaseinCMLwhitebloodcells.BeforeGleevec, themostcommondrugsused to treatCMLwere theoral treatmentshydroxyureaandbusulphan.An intravenoustreatment,cytarabine, issometimesused incombinationwith immunetherapy (interferon).Bonemarrowor stemcell transplantation tends tobe limited toyoungerpatients.

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HaematologyQ102:A30 yearoldpaenthas significantGIbleeding,but is concerned about the risksofbloodtransfusion.Whichofthefollowingisscreenedforindonatedblood?

A.JCvirusB.HumanTcellleukaemiavirusC.HIV1D.NewvariantCJDE.Toxoplasmosis

Answer:c)HIV1.IntheUKeveryblooddonationistestedforevidenceofhepatitisB,hepatitisC,HIV1,HIV2andsyphilis.However,althoughtherearerecentconcernsregardingtranmissionofnewvariantCJD,therearenoreliablescreeningmethodsyet.



HaematologyQ103:A35yearoldwomanwithahaematologicalconditionhasbeentransfusedwithgroupspecificplateletsonseveraloccasions.Herplateletcountdropsquickly5daysfollowingplatelettransfusion.Whatshouldshebetreatedwith?

A.FreshfrozenplasmaB.CryoprecipitateC.PackedredcellsD.IntravenousimmunoglobulinE.FactorVIII

Answer:D)intravenousimmunoglobulin.Post transfusion purpura is a transfusion reacon occurring occurs 5 14 days aertransfusionofplateletsorfreshfrozenplasma.ThisoccurswhenindividualslackingthePLA1 angen are transfused with blood containing PLA1 posive platelets. It isuncommonasonly23%ofthepopulaonarePLA1negave.Treatment of choice is intravenous immunoglobulin or plasma exchange. FurtherplatelettransfusionsshouldbewashedorbeHPA1Anegative.

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