cases in hematopathology
TRANSCRIPT
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CASEDISCUSSIONS
IN
HAEMATOPATHOLOGY
KGayathriIAPP.Pune.May4th2014
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Infant4months,withprogressiveanemiaandjaundice.
Received BloodTransfusionfrommother(70ml)at7daysageforHb7.5g/dl.
Case:1
gaya3k
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Thereisamorphologiccluetodiagnosis. What wouldyoudotoestablishdiagnosis?
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BABY KEERTHI PRIYA OSMOTIC FRAGILITY H -
064\03
0102030
405060708090
100
0 1 2 3 4 5 6 7 8
gm\L NaCl
%
OFLYSIS
T pre incu.
C pre incu
Tpost incu
Cpost incu
FragilitycurveshowssignificantshiftindicatingearlyRBClysisingradedsolutionsofbufferedsaline.
Willthisgivethediagnosis?
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Md. RAFI OSMOTIC FRAGILITY H 563 \ O3
0102030405060708090
100
0 1 2 3 4 5 6 7 8 9 10
gm /L NaC l
%oflysis T pre incu.
C pre incu
Tpost incu
Cpost incu
Red cell lysis at pH 7.4, 20C in g/L NaCl
Starts at : C 5.0 T 8.0Complete at: C 3.0 T 6.0MOF : C 4.5 T 7.0
Comments :Fragility Curve shows increase red cell fragility.
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Whatdoesthistelluswithregardtodiagnosis?
Whatimportantmessagecanwesharewithlaypersons,basedonthisfinding?
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M d. RA FI OSMOT IC FR AGILITY H 563 \ O3
0
102030405060708090
100
0 1 2 3 4 5 6 7 8 9 10
g m /L N aC l
%oflysis T p re incu.
C p re incu
Tp ost incu
Cp ost incu
Osmotic Fragility
FragilitycurveshowssignificantshiftindicatingearlyRBClysisingradedsolutionsofbufferedsaline.
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BABY KEERTHI PRIYA OSMOTIC FRAGILITY H
064\03
0102030
405060708090
100
0 1 2 3 4 5 6 7 8
gm\L NaCl
%
OFLYSIS
T pre incu.
C pre incu
Tpost incu
Cpost incu
Md. RAFI OSMOTIC FRAGILITY H 563 \ O3
0102030405060708090100
0 1 2 3 4 5 6 7 8 9 10
g m /L N aC l
%oflysis T pre incu.
C pre incu
Tpost incu
Cpost incu
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Whatdoesthistelluswithregardtodiagnosis?
Sample standing next to the cell counter
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Samplestandingnexttothecellcounter
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Whatimportantmessagecanwesharewithlaypersons,basedonthisfinding?
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Girl12years,detectedwithtransfusion
dependantanemiasincetheageof4years.
Onexaminationshortandsmallorage;no
lymphadenopathyororganomegaly.
Case:2
a a3k
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Case02Girl12years(si).BMB
Receivedbloodtransfusionssincetheageof4years Onexamination:
shortinstatureandsmallforherage nopalpablelymphnodes
noorganomegaly
hyperpigmentationperioral
Alertandintelligent
Clinicaldiagnosis?
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Hb5.6g/dl;TLC3600/cumm;Platelets80000/cumm
Peripheralsmear:
RBCS:NN¯ocytic
WBCs:Relativelymphocytosis
Platelets:Decreased.Nogiantforms
Reticulocytecount:
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Whatareyourobservations?Whatnext?
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Thereisamorphologicdiagnosis.Isthereanymoreinformationthatcanmakeadifferenceinthemanagement?
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Dicentric Telomere
Chromosomal aberrations seen in
in the current case & comparison with literature
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25%riskofFAin
everychild
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Whymustweestablishtheetiologicdiagnosis?
WhatistherecommendedmanagementforFA?
Whatisourrolebeyondestablishingdiagnosis?
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Cancers
Transformation>30%
Hematologicalmalignancies.
OthercancersOralcavity,breastandgynecologic tumors
ScreeningFamily
Chromosomefragility. HLAtyping
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Girl9years,admittedwithintermittentfever
of1year.Onexaminationwithpallorandmildicterus;
hepatosplenomegaly.Hb4.7g/dl;TLC2300/
cumm;Platelets20000/cumm.
Case:3
gaya3k
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Case03Girl9years(jy).BMA
IntermittentfeverXabout4months
Onexamination:
sicklooking
pallor
mildicterushepatosplenomegaly
Hb4.7g/dl;TLC2300/cumm;Plt20000/cumm
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Ismorphologicdiagnosisadequateforthemanagementofthischild?
HLH Di ti G id li
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HLHDiagnosticGuidelines
InternationalHLHstudygroup2004
Either1or2offollowing
1) MoleculardiagnosisconsistentwithHLH
2) Diagnosticcriteriafulfilled(5outof8)
Diagnosticcriteria:
ClinicalFever,Splenomegaly Lab
Cytopenia(Minimum
Bicytopenia) TriglyceridesorFibrinogen
MorphologicevidenceofHaemophagocytosisinBMA,
Lymphnode,Liver,Splenicpulp
NewCriteria
Ferrin, SolubleCD25,NKcellacvity
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Courseofillness
Receivedimmunosuppresiontherapy
Respondedinitially (steroids+cyclosporin)
Succumbedhoweverafter4months
Planearlytransplantinabsenceofresponse
Case:4
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Boy8years,withjointpains,fever.
Onexaminationsmallbuilt,palewithmild
icterus
gaya3k
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Simple techniquesCan yield remarkableresults
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Hb type Fa Mo Sib Pro
F 20.6 - 1.0 25.4
A0 2.5 48.5 48.1 2.6
A2 1.8 3.1 3.4 2.0
S 74.8 43.3 42.6 69.6
C/o painful joints.
Extended Family screen:
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y
Hb type 13M 32M 15F 9M
F - - - -
A0 89.0 88.2 88.1 51.7
A2 3.1 2.9 3.0 4.0
S - - - 39.1
-------------------------------------------
Relevant history:
Family with H/o consanguinity
With 8yrM - Sickle Homozygous
Sibs of both parents & progeny,
screened
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WhymustweevaluatefurtheronceSicklecellsareseen?
Whatisourrolebeyondestablishingdiagnosis?
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Parentsof6y.Fwithjaundiceandjointpains,
detectedwithsicklecells
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Roleincommunityawarenessandprevention
Boy5years,withcomplaintofpassingbloodinCase:5
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y y , p p g
urinesincedaypriortopresentation.Also
yelloweyessincethen,withpainabdomen
andvomiting.Hadsimilarepisodeatage3andreceivedbloodtransfusion.Remainedwell
betweentheseepisodes
gaya3k
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Whatdoesthemorphologysuggest?
C 05 B 5 ( ) PS
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Case05Boy5years(ma).PS
C/Opassingbloodinurinedayprior
Yellownessofeyes
Painabdomenandvomiting
Similarepisodeatage3years
Receivedbloodtransfusion Remainedwellintheinterval
Clinicaldiagnosis?
Peripheral blood
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Peripheralblood
Hb7.0g/dl;HCT16.4%;RBC2.45mill/cumm;MCV
67fl;MCH28.5pg;MCHC42.5g/dl;RDW16.2%
TLC20100/cumm;Plt3.87lac/cumm
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Whatnext?Whatinvestigations?
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M5yrtreatedforfebrileillness
brought witholiguriaandhigh
coloredurine.
G6PDScreening:Enzymeassay
NoDeficiencynoted
PlasmaHb:39mg/l
UrineHaemosiderin: Negative
UrineHb:Positive
(28mg/l)
Comments:
Haematologicprofileisconsistentwith
Intravascular Hemolysis.
Other parameters
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Otherparameters
Reticulocytecount(corrected):15%
Sicklingtest:negative
DAT:negative;IAT:negative
SerumLDH:3825U/L
HbHPLC:HbF HbA2.5HbA079.1% G6PDenzymeassay:432u/10.12redcells
Whatnext?
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Whatiscriticalinestablishingdiagnosis?
Follow up
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Followup
RepeatHb(weeks):11.4g/dl
G6PDenzymeassay:142U/10.12redcells
Evaluationduringstableperiod
IfnotdiagnosedwithG6PDdeficiencyplanotherenzymeassaysPK
Casper is here to guide you
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Casperisheretoguideyou
Sample collection principles :
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* Vacutainer collection / Prepared vials(Children)
* Choice of Anticoagulant : Hemogram K3 EDTA
Coagulation parameters - Citrate
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Peripheral Smear Examination
Probably contributes more to the diagnosis of
Haematologic disorders, than any other single
laboratory parameter (Wintrobe)
What is the value of an investigation ??
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Life itself .