MEQ Session 011016 Case 1 - Nigel Fong

M5MEQs201601/10/2016Case3:MsHuiBoGaohttp://tinyurl.com/jst7stf

WelcometotheamazingworldofhematologyJ

Ms Hui isa30yearsoldfemale.Shehasnopastmedicalhistory.ShewasreferredfromherGPwiththepresentationof4dayshistoryoffeverandarash.Shehasnootherbleedingmanifestations.Onexamination,hervitalswasT38.5BP100/62,HR80RR22SpO299%onRA.Shelookscomfortable.Examinationwasrevealingofarashoverthelimbsandtrunkwhichisnon-blanchable.

Question1.1:MsHuiBoGao

Q1.Whicharethe2mostimportanttestwillyouorderfirst?• Fullbloodcount• LiverPanel• RenalPanel• HIVTest• DengueSerology• PT/APTT• BloodCultures

1.1Question:MsHuiBoGao

Aclassicimageofpurpura

Q1.Whicharethe2mostimportanttestwillyouorderfirst?• Fullbloodcount– lookingforthrombocytopenia• LiverPanel• RenalPanel• HIVTest• DengueSerology– denguerashusuallyappearswhenthefeverlyses;thispt isstillfebrilewitharash

• PT/APTT– lookingforcoagulopathy• BloodCultures

Therestarenotwrongbutyouarebeingaskedtoprioritise!

1.1Question:MsHuiBoGao

Smalllesions(<0.5cm)• Macule– flat,cirumscribed, colored, nonpalpable• Papule– raised,solid andpalpable• Vesicle– raised, palpable,clearfluid-filled• Pustule– raised,palpable,pus filled(leukocytes orkeratin)Largelesions(>0.5cm)• Patch– largemacule(flatnon-palpable coloredarea)• Plaque– superficially raised,circumscribedsolid area• Nodule – distinctlargepapule• Bulla- largevesicle(blisters ifepidermallayercompletely sloughedoff)• Wheal– firmandedematous plaque(edemaofthedermis)Other• Plaque/scaley papule• Eschar• Erosion/ulcer• Purpura/petechia• Plaque/smooth papule

• Purpura isusedtodescriberedpurplelesionsthatresultfromtheextravasationofbloodintotheskinormucousmembranes

• Non-blanchable

• Purpuramaybepalpableornon-palpable(flat/macular)• Macularpurpura isdividedon2morphologiescharacterizedbysizeandtypicallynon-inflammatory– Petechiae – <3mm– Ecchymosis- >5mm

• Palpablepurpura:usuallyasignofvascularinflammation

EDbloodsshowsthefollowing:Hb 6.9TW50.2Plt 30N.myelocytes PresentPromyelocytes 90%Renalpanel:U7Na131K3.5Bicarb 20Cr60LiverPanelTBil 4ALP120AST55ALT42PT42APTT82

Youreviewthepatientintheward,vitalsT38.9BP94/70HR80.Sheisstillcomfortableanddoesnothaveanybleedingmanifestations.


EDbloodsshowsthefollowing:Hb 6.9TW50.2Plt 30N.myelocytes PresentPromyelocytes 90%Renalpanel:U7Na131K3.5Bicarb 20Cr60LiverPanelTBil 4ALP120AST55ALT42PT42APTT82

Youreviewthepatientintheward,vitalsT38.9BP94/70HR80.Sheisstillcomfortableanddoesnothaveanybleedingmanifestations.

TW20,maybeinfection.TW100,thinkleukaemia


Q2.Astheon-callHO,whichofthefollowingtherapieswouldyouinitiate?(Choose3of10)*• Transfuse1pintPCT• Transfuseplatelets• Referraltothehaematologyoncall• TransfertoMedicalICAformonitoring• Arrangeforaphresis• StartIVfluids2LNSover24H• Starthydroxyurea1gTDS• RepeattheFBC• Dobloodculturesandstartempiricalantibiotics• Startdexamethasone


Don’tknowwhatisgoingon….Alwaysgobacktobasics• Transfuse1pintPCT• Transfuseplatelets• Referraltothehaematologyoncall• TransfertoMedicalICAformonitoring• Arrangeforaphresis --- youwouldn’tstartthisyourself.Callhaem!• StartIVfluids2LNSover24H• Starthydroxyurea1gTDS --- youwouldn’tstartthisyourself.Callhaem!• RepeattheFBC• Dobloodculturesandstartempiricalantibiotics• Startdexamethasone--- bigno-noinacuteleukaemia cases!Cancause

tumorlysis.

Nowcontextualized…• Transfuse1pintPCT• Transfuseplatelets• Referraltothehaematologyoncall• TransfertoMedicalICAformonitoring--- patientisstillwell• Arrangeforaphresis• StartIVfluids2LNSover24H• Starthydroxyurea1gTDS• RepeattheFBC--- youdon’tbelievethelabisit?• Dobloodculturesandstartempiricalantibiotics• Startdexamethasone

Somebasicmanagementprinciples• Transfuse1pintPCT• Transfuseplatelets• Referraltothehaematologyoncall• TransfertoMedicalICAformonitoring• Arrangeforaphresis• StartIVfluids2LNSover24H--- hydrationisrarelywrong• Starthydroxyurea1gTDS• RepeattheFBC• Dobloodculturesandstartempiricalantibiotics--- probablyquitesafe• Startdexamethasone

YoualsonoticedthattheAPTT/PTisprolonged.YourMOtellsyoutoorderotherinvestigationstoworkupthecause.Q3.Whatinvestigationwillyouordernextforthispatient(Choose2)• 50%correctionstudies• Directcoombstest• D-Dimer• Liverfunctiontest• Fibrinogen• Plateletfunctiontest• Bleedingtime


YoualsonoticedthattheAPTT/PTisprolonged.YourMOtellsyoutoorderotherinvestigationstoworkupthecause.Q3.Whatinvestigationwillyouordernextforthispatient(Choose2)• 50%correctionstudies• Directcoombstest• D-Dimer• Liverfunctiontest• Fibrinogen• Plateletfunctiontest• Bleedingtime


Thisquestion isaskingyou,whatdoyouthinkisthecauseofprolongedaPTT/PT?Theinvestigationyouchooseistoconfirmyoursuspicion.

Inthiscasecoagulopathy isduetoDIVC(happens inacuteleukaemia orvsickpt)…notduetoliverdiseaseorfactorinhibitors orautoimmune dx.

InvestigationsinDIC:• Prolongedoftheprothrombin time(PT)

– Reflectsreducedactivityofthecomponentsoftheextrinsic andcommonpathways

– IncludefactorsVII,X,V,andprothrombin,whicharethemostfrequentlydecreasedclottingproteinsinDIC

• ProlongedActivatedpartialthromboplastin time(aPTT)– Measurestheintrinsicandcommonpathwaysofcoagulation– IssensitivetodeficienciesoffactorsXII,XI,IXandVIII– LesssensitivethanthePTtodeficienciesofcomponentsofthecommonpathway

InvestigationsinDIC:• Thrombocytopenia:Consumptive• Plasmafibrinogen:Usuallylow inacutedecompensatedDIC– Cavet:Maybeelevatedasanacutephasereactant

• D-Dimer:high - indicativeoffibrinolysis• PeripheralBloodFilm

– Thrombocytopenia– Largeplatelets– Fragmentedredcells(schistocytes).

Thehaematology reviewsthebloodfilmtoconfirmthediagnosis.


Q4.Whatisthemostlikelydiagnosis?*• Acutemyeloidleukemia• Burkitts Lymphoma• Acutepromyelocytic leukemia• Acutelymphoblasticleukemia• Chronicmyeloidleukemia


Q4.Whatisthemostlikelydiagnosis?*• Acutemyeloidleukemia• Burkitts Lymphoma• Acutepromyelocytic leukemia• Acutelymphoblasticleukemia• Chronicmyeloidleukemia


Theanswerwasalreadyinthestem!Promyelocytes:VerygranularWeirdlooking bignucleiAuerrods

Thediagnosisofacutepromyelocytic leukemiawasmade.Thehaematology registrarsproceedstoordertheappropriatetreatment.Q5.Whattreatmentwasordered?(Choose1)*• Alltransretinoicacid• Dexamethasone4gTDS• Hyper-CVAD• IA3+7• Allogenictransplant


Thediagnosisofacutepromyelocytic leukemiawasmade.Thehaematology registrarsproceedstoordertheappropriatetreatment.Q5.Whattreatmentwasordered?(Choose1)*• Alltransretinoicacid• Dexamethasone4gTDS• Hyper-CVAD• IA3+7• Allogenictransplant


ManagementofAPML• Medicalemergency:duetotheriskofalifethreateningbleed• Treatmentisstartedonclinicalsuspicion• ATRA(Alltrans-retinotic acid)akavitaminA• Supportivemanagementforbleedingcomplications

ThenextdayyouwerecalledtoseeMs Tanforhemoptysis.Thenursesinformedyouthatshehadcoughedoutblood6timesinthelast1hour,eachtimefillingupaspecimenblood(50ml).Onreviewofthepatient,Ms Tanwastalkinginfullsentences.Shedoesnotcomplainofanybreathlessnessorchestpain.Onexamination,RR24SpO297%onRA.Heartsoundsareheardwithnoadditionmurmursorheartsounds.Lungshavereducedairentryatthebases,noadditionalsoundswereheard.


Q6.Whatwillyoudonextinthemanagementofthispatient?• Putpatientonintranasaloxygen• Considerelectiveintubation• Patientisclinicallystable,askthenursetoinformyouiffurtherepisodesoccur

• Arrangeforrespiratoryreviewandbronchoscopy• UrgentthoraximagingwithaCTthorax


Q6.Whatwillyoudonextinthemanagementofthispatient?• Putpatientonintranasaloxygen• Considerelectiveintubation• Patientisclinicallystable,askthenursetoinformyouiffurtherepisodesoccur

• Arrangeforrespiratoryreviewandbronchoscopy• UrgentthoraximagingwithaCTthorax


Massivehemoptysis• Nocleardefinition:500mlover24Hor150mlover1h• Thinkoftheanatomicaldeadspace- thevolumethatislifethreateningbyvirtueofairwayobstruction(anatomicaldeadspaceisonly100-200mL)

• Medicalemergencyandshouldbeaddressedimmediately.• Initialprioritiesarestabilisationofthepatientandprotectionofthenon-bleedinglung

• ABCfirst– maintainairwaypatency:Asphyxiationisthemostfrequentcomplicationofmassivehemoptysis

• Assessment:History,PhysicalExamination,investigationsInvestigation:FBC/PT/APTT/CXR

• InitialManagement:– Oxygenation– Lateralposition(ifsiteofbleedisknown- affectedlungindependentposition)

– IVaccess– Correctcontributingcoagulopathy/thrombocytopenia

• Localizethesourceofthebleeding:Bronchoscpoy

PatientwaselectivelyintubatedandtransferredtoICU.Abronchoscopywasdonewhichshoweddiffusebleeding.Shewassupportedwithbloodproductsincludingplatelets,freshfrozenplasmaandcryoprecipitate.ThebleedingfinallystopsandtheICUteamconsidersextubation.


Q7.WhichofthefollowingregardingbloodtransfusionsisINCORRECT• ThetypicaleffectivedoseofFFPis10to15mL/kg• FFPiseffectiveattreatingmildcoagulopathiescharacterizedbyINRoflessthan1.85

• CryoprecipitatecontainsfactorVIII,vonWillebrand factor,factorXIII,fibronectin,andfibrinogen

• Theriskforbacterialinfectionandsepsisisincreasedinpatientsreceivingplatelettransfusionscomparedwithotherbloodproducts

• FFPisanacellular product


Q7.WhichofthefollowingregardingbloodtransfusionsisINCORRECT• ThetypicaleffectivedoseofFFPis10to15mL/kg• FFPiseffectiveattreatingmildcoagulopathiescharacterizedbyINRoflessthan1.85

• CryoprecipitatecontainsfactorVIII,vonWillebrand factor,factorXIII,fibronectin,andfibrinogen

• Theriskforbacterialinfectionandsepsisisincreasedinpatientsreceivingplatelettransfusionscomparedwithotherbloodproducts

• FFPisanacellular product


Whatcanyoutransfuse• Cellularproducts:RBC,platelets• Plasmaproducts:FFP,Cyroprecipitate,plasmaderivedproducts

Approach• Knowyourindicationfortransfusion• Weightheriskandbenefits

Remember:Bloodproductsisamedicationandisapublicresource(donated)

Redbloodcells• Indication:improvetheoxygen-carryingcapacityofblood.• Humansdisplaymanycompensatoryresponsestomaintainoxygendelivery,andnosinglehemoglobintriggerexists

• Generalguidelines:Hb >7(Adjustbaseonpatient’sphysiologicalstate)

• Targets:Typically250to300mLwithanexpectedincreaseinthehemoglobinof1g/dL (10g/L)inanonbleedingadult.

Platelets• Indication:prophylacticandtherapeuticforhemorrhageinpatientswiththrombocytopenia

• Optionsavailable:pooledorsingledonor1unitfromasingledonor=approx 4-5pooleddonors

• Targets:usuallyunittransfusionshouldbringupplateletcountbyatleast20-30k

• Note:Storedatroomtemperature– thewarmerstorageconditionsallowforproliferationofanycontaminatingbacteria

FreshFrozenPlasma• Indication:Prophylacticortherapeuticinpatientswithmajorcoagulopathy,warfarinreversal,massivetransfusion

• Containsallthebloodclottingfactors• Targets:FFPvolume200-300ml;effectivedosing10-15ml/kg• Note:CorrectionwithFFPisnotalinearphenomenonIUnlikePCT– 1packPCTincreasesHb ~1gTheamountofFFPrequiredtobringINRfrom10to9ismuchlessthanthatrequiredtobringINRfrom3to2.

•Haematologyisfun•Haematologyisnotscary!!•Haematologyiseasy!!!

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MEQ Session 011016 Case 1 - Nigel Fong