Philip Kelly MRCP 2 book 3

HAEMATOLOGY-QUESTIONS Case 3A25-year-oldAsianlady presentedtoherCPagain with increasing tiredness,lethargyandeasybruising... HAEMATOLOGY QUESTIONS Case 4A27-year-oldAfro-Caribbeanmanpres

Acknowledgements

Introduction

viiviii

Claire)HemmawayMRCPMRCPath

SpecialistRegistrarinPaediatricHaematology,HammersmithHospital,London

Neurology

David L HBennett MBPhDMRCP

SpecialistRegistrar inNeurology, Department ofNeurology,

King's CollegeHospital,London

Ophthalmology

JasminKSinghMBBSMRCOphth

Senior HouseOfficerinOphthalmology,TheWesternEyeHospital,

London

AmirHamid BMedSci BMBSMRCOphth

SeniorHouseOfficer inOphthalmology,ManchesterRoyal EyeHospital,

Manchester

Rheumatology

William CDixon MRCP

ClinicalResearch Fellow,arc EpidemiologyUnitandSpecialistRegistrar

inRheumatology,University ofManchester,Manchester

Iwouldliketoexpress mygratitudetotheteam at PasTestparticularly

CathyDickens forherunswervingsupportand toleranceduringthepreparationofthis series.Manypatients havebeengraciousenoughtocontributeto ourongoingeducationby allowingtheir imagestobe usedin

these volumes.The serieswould have beenimpossiblewithout thehelpofthefollowing:MrsSue Hemmawayfor preparationofthehaematology

section;DrPhilipBeerand David Roper,Hammersmith Hospital, forhelp

withbloodfilms; Dr Mark Layton,Consultanthaematologist,

Hammersmith Hospital;Mr Riordan-Eva,Consultantophthalmologist,

KingsCollegeIHospital;MedicalPhotography, Radiology and Medicineat

KingGeorge Hospital,llford;Medical Illustrationat Bartsand The LondonSchoolofMedicine andDentistry;Radiologyand The Department ofDiabetes and Metabolismat Bartsand The LondonNHS Trust.

Philip Kelly

TheMRCP(UK)Part2writtenexamination consistsoftwo 3hourpapers,eachwithupto 100multiplechoicequestions;theyareeither Ifrom5

(bestof5) or'n' frommany,where2 answers arechosen from 10.Eachquestionwillhaveaclinical scenarioandmightcontaininvestigationsto

interpret; manymightalsocontain animage.Thereis apass markagreed

bytheexaminersbutacandidate'sperformanceisalso assessedinrelation

toothercandidates

This3book seriesprovidespractice questionswithextensiveexplanations

toaid candidates preparingfortheexamination.The authors arc allclinicianswritingsections in theirchosen fields andassuch have beenchosenfortheir clearunderstandingoftherequired knowledgebaseforthisimportantexam.The breadthofknowledgeforthisexam is vastand

theyhaveattemptedtocoverthe'syllabus'ascompletelyaspossible

Great carehas beentakentoexplainareasthatcause difficultyas

thoroughlyaspossible.Noapologyismadewhere the format of thequestionsdiffers slightlyfromtheexam.These booksare notmerelypractice papers buteducationalaids and whereatopiccanbe best

explained bydiversionfromthestrictformat ofthe exam, for thesakeof

understanding,this has been done

Thisbookcovershaematology,neurology,ophthalmologyand

rheumatologyandisbesttaken-inconcertwithitscolleagues withinthe

series-as asupplementto athoroughclinicalgrounding,thegeneralmedicaltextsand thecoreclinicaljournals

HAEMATOLOGY QUESTIONSCase1

A38-year-old Nigerian ladywasreferredtotheGastroenterologyClinicfor investigationofhyperbilirubinaemia.Her pastmedicalhistory

includedacholecystectomyforgallstonediseasemorethan10years ago

Onexaminationshewasjaundicedandpale.Therewas no

lymphadenopathy.Asplenictipwaspalpable

Herbloodresultswereasfollows:

HAEMATOLOGY- QUESTIONS

2 Which threediagnosesareinthe differentialdiagnosis?

ÿ A Amembrane disorder, eghereditaryspherocytosis(HS)

ÿ B G6PDdeficiency

CD C Autoimmunehaemolyticanaemia(AIHA)

CD D Ahaemoglobinopathy

CD E Haemolyticuraemicsyndrome(HUS)

ÿ F Disseminatedintravascular coagulation(DIC)

CD G Thromboticthrombocytopenicpurpura(TTP)

HAEMATOLOCY QUESTIONS

Case 2

A /6-year-oldNigeriangirlpresentedto A&Ewithweakness down the leftsideofherbody.Shehadbeen inalessonatschoolwhenthe weakness

developedsuddenly.Shewas on nomedicationsandhadno pastmedical

historyapartfrommildintermittentjoint and bonepains.Hermotherhadbeendiagnosed withSLE5 years earlier.Herfatherwaswell andworked

in the(lilyas an accountant.Shehadone sisterwhowaswell She hadbeen bornprematurelyat 25weeks

Onexamination shewasjaundicedandhad4/5weaknessaffectingherleftupper and leftlowerlimbs.Herfacewasspared.Asystolicmurmur washeardatthe leftsternaledge

Onfurther questioning,she maintained that hersclerawerealwaysyellow

Herbloodtestresultswere asfollows:

HAEMATOLOGY-QUESTIONS Case 3

A25-year-oldAsianlady presentedtoherCPagain with increasing

tiredness,lethargyandeasybruising

Herblood resultswere asfollows:

ÿ C Acuteblood loss

ÿ D Irondeficiencyanaemia

HAEMATOLOGY QUESTIONS Case 4

A27-year-oldAfro-Caribbeanmanpresentedto A&Ewithpriapism.Otherwisehe felt well in himself Theonlyother findingonexamination

was aleftupperquadrantmass, which extended7cmbelow the

The filmisshown below:

1 What is thediagnosis?

ÿ A Sicklecell disease(SCD)

LJ B Acutemyeloidleukaemia(AML)

ÿ C Chronic myeloidleukaemia(CML)

D D Acutelymphoblasticleukaemia(ALL)

D E Chroniclymphocyticleukaemia(CLL)

ÿ F High-grade non-Hodgkin's lymphoma(NHL)

CD C Follicularlymphoma

ÿ H Sildenafil overdose

Hedevelopsfeversof40°C,pleuriticchest painandhaemoptysis.He

denies shortnessofbreathat rest or onexertion.Hisoxygensaturations are

97% on air.

TheCXRandCT scan areshown below andonthenextpagerespectively:

HAFMATOLOGY QUESTIONS Case 6

A 65-year-oldMoroccandiplomatpresentedtohospitalwitha3-monthhistoryoffatigue.He was aheavydrinkerbutdidnotsmoke.Hehadtravelledwidelyas partofhisjob - throughoutAfrica, Asia, Europe and

America Hehada pasthistory ofrheumatoidarthritis, treated in thepast

withsteroids,butthediseasewascurrentlyquiescent.Hehadhadadeep

venousthrombosis(DVT) 6years earlierforwhich he hadbeen treated

withwarfarin for6months

On examinationhe hadalow-gradefever,hewaspale, jaundicedand had

hepatosplenomegalywiththe liverbeing5cmbelow the costal marginandthespleen17 cmbelow the costalmargin.Therewas noperipheral

lymphadenopathyandnoevidenceofan activearthropathy

Hisblood resultsareshown below:

B1 2and folate Normal

Proteinelectrophoresis IgMkappaparaprotein2g/L

HAEMATOLOGY QUESTIONS

1 Whatisthemain causeofthe anaemia?

ÿ A Irondeficiency

CD C Anaemiaofchronicdisease

CD D Myelodysplasia

CD E Non-Hodgkin's lymphoma

CD F Haemolysis

' i G Hypersplenism

CD H None of theabove

2 Choosethreepossibledifferentialdiagnosesfromthefollowinglist:

I J A Lymphoproliterative disorder

C B Chronicliverdisease

. 1 D Acutemyeloidleukaemia

CD E Multiple myeloma

i ) F Chronic myeloidleukaemia

CD G Myelofibrosis

CD H Acutehepatitis

HAEMATOLOGY QUESTIONSCase7

A55-year-oldmanpresentedtohisCPfeeling tired,weak andgenerallyunwell.Hegaveahistoryof increasing shortness of breathoverthe lastfewweeks andanon-productivecough.Hispastmedicalhistoryincludedhypertension,stableangina, diabetes mellitus and rheumatoidarthritis

Onexamination,hewaspaleand had pitting ankle oedema.HisBPwas

1 70/1 00mrnHg.Otherwise,examination wasunremarkable

Hisbloodscounts were asfollows:

Reticulocytes 0% (absolute count 0 x 1 0'VL)

HisCXRisshown below:

0 C Carcinomaofthelung withbonemarrowinvolvement

0 D Red cellaplasia

0 E Anaemiaofchronicdisease

0 F Mixedhaematinicdeficiency

Onfurtherquestioningthisladyalsocomplainsofdiplopiawhilereading

3 What additionalinvestigationcouldyouperform?

HAEMATOLOGY - QUESTION'S Case 8

A60-year-oldNigeriangentlemanpresents intheUKforasecondopinion.Threeyearsbeforehe hadbeen toldhe wasanaemic.Hehad

splenomegalytobelowtheumbilicusandaliveredge.HehadabonemarrowinNigeriawhichwasinconclusive.His pastmedicalhistoryincludeshypertension.Hehas travelledextensively throughout Africaandhashadrecurrentattacks ofmalaria,treatedwithchloroquineand,mostrecently, Paludrind*

Ile ispaleandhassplenomegalyasdescribed above.Hehasnostigmata

ofliver disease.Onauscultationofhis heart soundshe hasasystolic

murmur,loudestattheapex.Thereis nopalpablelymphadenopathy

I lisinvestigation results are shown below:

Hep A/B/Cserology Negative (IgG and IgM)

USabdomen Spleen2Icm

0 F Malarial antibodylitres

0 G Leucocyteglucocerebrosidaseactivity

i) H CTchest/abdomen/pelvis

HAEMATOLOGY QUESTIONS

Case9

A 5 1-year-oldCaucasiangentlemanpresentedwithaflu-likeillness anda

symmetricalpolyarthropathy affectinghisknees,ankles, shoulders,

elbows and wrists There was obviousswellingof hiskneesandankles and

apapularrashoverhisarms.Afullexaminationwasotherwise

unremarkable.He wasinitiallytreatedwithantibiotics byhis GPhut failed

toimprove.Hethenpresentedto A&Ewiththesame symptomsandwasadmittedfor investigationandcommencedonanti-inflammatories.X-rays

werecarriedoutofalltheinvolvedjoints, which showednobonyerosions There was someimprovementandhe wasdischargedafter a few

days

6weeks later hewasreadmittedwithshortnessof breath,acoughanda

persistarthropathy.The rash had resolved.Heremainedapyrexial

TheCXR is shownbelow:

HEMATOLOGY QUESTIONS Hisblood resultsareshown below:

bronchoalveolarlavage(BAL).

1 Whatisthemostlikely diagnosis?

i 1 I Catscratch disease

I J Non-Flodgkin's lymphoma

t_ K Polyarteritisnodosa

HAEMATOI.OGY- QUESTIONS

He istreatedforhisunderlyingconditionsuccessfully.Hecontinuedtobefollowed upregularly.Atoneoftheseroutinefollow-upshe hasthefollowing fullbloodcount:

II AEMATOLOGY-QUESTIONS

Case 10

An80-year-oldIndiangentleman,who has been in thiscountryfor30

years but whofrequentlygoesbacktoIndia(last visit morethan Iyearpreviously),presentedtohisCPnon-specificallyunwelland hadalarge

bruiseoverhiship followingafall.Hispastmedicalhistoryincluded

hypertension,angina andhypercholesterolaemia

Onexaminationhewasapyrexial.HisBPwas180/70mrnHg.Therewere numerousbruises( <2 cm) overhis lower limbs anda 1 0 cm x 10cm

bruiseoverhislefthip

Ilisbloodresultsareshownbelow:

1 Which threediagnosesare atthetopofthe differentialdiagnosis?

[ i A Autoimmunehaemolyticanaemia

ÿ C Irondeficiencyanaemia

LJ D Non-Hodgkin'slymphoma

O E Myelodysplasia

Q E Chroniclymphocyticleukaemia

D C Acutemyeloidleukaemia

D II Hairy cell leukaemia

HAEMATOLOGY- QUESTIONS

2 The bonemarrowis shown below:twodiagnosescanbe made.What arethey?

HAEMATOLOGY QUESTIONS Case 11

A26-year-oldgentlemanwithsickle cell disease who hadrecentlyreturned from The Gambia, where hewasborn,presentedtoA&E.He

complainedofabdominalandback pain.Ilispastmedicalhistory

includedrecurrentpainfulcrisesandtwoepisodesofchestcrisiswhich

requiredITUadmission.Hehadacholecystectomy5yearsago becauseofrecurrentcholecystitis

He was onhydroxycarbamide (hydroxyurea),1gdaily, whichhadreducedthe frequencyofhis admissions forpainfulcrisesdramatically

Onexaminationhewaspale, pyrexial(38.5 !'C),significantly jaundiced

and hadgeneralisedabdominal tenderness Chest auscultation was clear

Hisoxygensaturations were99%on air.

I lisblood resultsareshown below:

Boneprofile Normal

1 Whatarethe first threeinvestigationsyou woulddo?

C A Group andsave

CD H Noneofthe above

CD I All ofthe above

3 What investigationshouldbe donepriortocommencing therapy?

ÿ A Chest X-ray

I IAFMATOLOGY -QUESTIONS

Case 12

/I45-year-old CaribbeangentlemanwasreferredtoHaematologyOutpatientswithwidespreadsmall-volumelymphadenopathy,nightsweats,weightinss and diarrhoea.Headmittedtoheavy drinking(in

excessof50 units a week)andwasasmoker(50 aday) He hadhadmultiplesexualpartnersin the last20years.Hehadbeenborn inJamaica,buthadlivedin theUKfor 2 years.HereturnedtoJamaicafrequently

On examinationhe hadalow-gradepyrexia,widespreadsmall-volume

lymphadenopathy, hepatosplenomegaly(liver 2 cmbelowthecostalmargin andasplenictip)

Hisinvestigation resultswere asfollows:

1 What is themostlikely diagnosis?

ÿ A Sarcoidosis

ÿ B Tuberculosis

ÿ C Ilumanimmunodeficiencyvirus(HIV)

ÿ D Adult T-cellleukaemia/lymphoma(ATLL)secondaryto

HTI.V-(humanT-cell leukaemiavirusinfection)

D E Iiodgkin'sdisease

ÿ F Sezarysyndrome

ÿ c; Ilyperparathyroidism

ÿ H Metastatic carcinomaofthelung

HAEMATOLOGY QUESTIONS

Case 13

A 54-year-old lady presentedtoherCPwithincreasedbruisingandtiredness She hadbeendiagnosedwithchronicmyeloidleukaemia 19yearsbefore.Shewasinitiallytreatedwithinterferonand thenwent on tohaveanautologousbonemarrowtransplant.Shehadbeencompletely

well for1 9years Cytogeneticanalysishadalwaysshown0%Philadelphia

chromosomessincehertransplant

On examinationshe hadnumerousbruises,buttherewasnothingelsetofind

Herbloodresults showedthefollowing:

WCC 0.6 x 10"/L Bilirubin 22pmol/L

Neutrophils 0.1 x 10''/L AST 35 U/L

The film showedthrombocytopenia,leucopenia,anormocyticanaemia

andanoccasionalcirculating myeloidprecursor.

The bonemarrow isshown below:

HAEMATOLOCY QUESTIONS

Cytogenetic analysisshowed the presence ofPhiladelphiachromosomes

1 What is thediagnosis?

0 A Immunethrombocytopenia(UP)

ÿ ) B Myelodysplasia

O C Chronicmyeloidleukaemia(CMC)inchronicphase

J D Chronicmyeloidleukaemia(CML)in blastcrisis

O E Acutemyeloidleukaemia(AML)

_l F Acutelymphoblasticleukaemia(ALL)

The cells showninthe bonemarrowhave thefollowingmarkersontheirsurface:

CD19 positive CD7 negative

TDT positive CD13 negative

CD34 positive CD33 negative

CD10 positive CD2 negative

CD79a positive CD3 negative

2 Whichlineageis thecurrentproblemin?

HAEMATOLOGY QUESTIONS

Case 14

A32-year-oldladypresentedtoA&Ewithbleedingper vaginam.Sheis 20weekspregnant.Onultrasoundthereis intrauterinegrowthretardation

(IUCR).ShehadaDVTaged30followingaroadtrafficaccident when she

fracturedher left femur

Ilerbloodresultswere asfollows:

ÿ A Factor VIII levels

I I? Lupusanticoagulantandanticardiolipin antibodies

ÿ K All ofthe above

ÿ L Noneoftheabove

HAFMATOLOGY- QULSTIONS

2 Whatotherhistorywould you liketoknow?(one answer)

ÿ A Pastobstetrichistory

ÿ B Family historyofthromboticepisodes

O C Bleedinghistory

ÿ D Family historyof earlypregnancy loss

ÿ E Familyhistory ofthrombocytopenia

ÿ F Weightloss

ÿ G Nightsweats

ÿ Fl Warfarin-induced skin necrosis

3 Whatdiagnosiswould youconsider?

HALMATOLOGY QUESTIONS Case 15

/A25-year-oldladywhois 34weekspregnant(para2)presentedto A&E

witha4-day historyofjaundice,easybruisinganda severeheadache.There hadbeennopriorproblemsinthepregnancy

Onexamination shewasjaundiced.HerBPwas 110/75mmHg.Her

temperaturewas 37°C Shehad severalsmall bruisesoverherabdomenandlegs Urinanalysisshowed protein+++• and blood+++.

Herbloodresultswere asfollows:

1 What isthemostlikely diagnosis?

ÿ A Thromboticthrombocytopenicpurpura(TTP)

ÿ B Haemolyticuraemicsyndrome(HUS)

HAEMATOLOCY QUESTIONS Case 1 6

A76-year-oldPolishgentlemanpresentedto A&fwitha 3-weekhistory of

lethargy,shortness of breath,confusionandbeing'offhislegs'

On examination hehada chaoticpulseol 140bpm.IlisBPwas 1 30/85mmHgand hisSaO>99% on air He wasjaundicedand had bibasalinspiratorycrepitationsonauscultation of hischest.Itwasimpossibleto

doafullneurologicalexamination onhimbecause hespokenoEnglish

andwascontused

Hisbloodsresults areshown below:

100U/L

2500 U/L

9pmol/L

0.2 mU/L

HAEMATOLOGY- QUESTIONS

The filmisshown below:

1 Whatarethetwodifferential diagnoses?

ÿ A Bi»orfolatedeficiency

D B Thromboticthrombocytopenicpurpura

D D High-gradenon-Hodgkin's lymphoma

ÿ E Hypothyroidism

ÿ F Hepatitis

ÿ G Myelodysplasia

ÿ H Autoimmunehaemolyticanaemia

2 What wouldyour nextinvestigationbe?

II AEMATO LOG Y - QUFSTIONS

3 Which(wo are (he correctmanagement?

HAEMATOLOGY- QUESTIONS

Case 1 7

A60-year-oldman isadmittedviahisCPtoyourcarewithstreptococcal

pneumonia andistreatedappropriately.He is asmoker,drinksabottleofvodkaper week and works inafishmarketduringthe week 10daysafterhisadmission his haemoglobindropsto9g/dLfrom 12g/dLonadmissionandhebecomes jaundiced.Hehadbeenstartedonibuprofenfor

Reticulocytes 6% (absolute count 300 x 1 07L)

The filmisshown below: