HEMATOLOGY: CHAPTER 37 & 38 -
RODAKS 6TH EDITION QUESTIONS AND
ANSWERS
Glanzmannmthrombastheniam-mans-
Describedmasmambleedingmdisordermassociatedmwithmabnormalminmvitromclot
mretractionmandmnormalmplateletmcount
-
mNormalmpltmcount,mnormalmpltmmorphology,mlackmofmpltmaggregation,mredu
cedmclotmformation
Bernard-Souliermsyndromem-mans-
ismamraremdisordermofmplateletmadhesionmthatmusuallymmanifestsminminfancy
mormchildhoodmwithmhemorrhagemcharacteristicmofmdefectivemplateletmfunction
-mNormalmaggregation,mepinephrine,mcollagen,mandmarachidonicmacid
-
mDomnotmrespondmtomristocetinmandmhavemdiminishedmresponsemtomthrombi
n
DensemGranulemDeficienciesm-mans-
Thesemsyndromesmaremclassifiedmundermthemgroupmofm?
-mHermansky-Pudlakmsyndrome
-mChediak-Higashimsyndrome
-mWiskott-Aldrichmsyndrome
-mThrombocytopenia-absentmradiusm(TAR)msyndrome
a-GranulemDeficiencesm-mans-
Thesemsyndromemismclassifiedmundermthemgroupmof?
-mGraymplateletmsyndrome
Chediak-Higashimsyndromem-mans--
mRaremautosomalmrecessivemdisordermcharacterizedmbympartialmoculocutaneou
smalbinism,mfrequentmpyogenicmbacterialminfections,mgiantmlysosomalmgranules
minmcellsmofmhematologicmandmnonhematologicmorigin
Wiskott-Aldrichmsyndromem-mans-RaremX-
linkedmdiseasemcausedmbymmutationsminmthemWASmgenemonmthemshortmar
, mmofmthemXmchromosomemXp11.23mthatmencodesmformam502-
aminoacidmprotein
GraymPlateletmSyndromem-mans-
Plateletsmfrommthesempatientsmcontainmabsentmormmarkedlymreducedma-
granulemproteinsmincludingmPF4,mvWF,mfibronectin,mandmfactormV
Themmostmconsistentmlaboratorymabnormalitymhasmbeenmimpairmentminmthrom
bin-mediatedmaggregationmandmsecretion
150,000mtom450,000/uLm-mans-Normalmplateletmcount:
Thrombocytopeniam-mans-
ismthemmostmcommonmcausemofmclinicallymimportantmbleeding
Petechiaem-mans-
aremsmallmpinpointmhemorrhagesmaboutm1mmmminmdiameter
Ecchymosesm-mans--marem1mcmmormlargermandmusuallymirregularminmshape
-mlaymterm:mbruise
May-HegglinmAnomalym-mans-
autosomalmdominantmtraitmdisordermwheremneutrophilsmhavemblue-
stainingminclusionsmthatmresemblemDohlembodies;mthrombocytopeniamismalsom
presentmwithmgiantmabnormalmplatelets
MYH9m-mans-UndermMay-
HegglinmAnomaly,mtheremismammutationminmwhatmgene?
TARmSyndromem-mans-
Raremautosomalmrecessivemdisordermcharacterizedmbymseveremneonatalmthrom
bocytopeniamandmmutationminmthemRBM8Amgene
FanconimAnemiam-mans-
Associatedmwithmthrombocytopeniamandmothermabnormalitiesmandmaremmostly
mduemtommutationsminmonemofmthem3mgenes,mFANCA,mFANCC,mFANCG
NeonatalmThrombocytopeniam-mans-Plateletmcountmofm<150,000/
mLmandmismpresentminm1-5%mofminfantsmatmbirth
AcutemITPm-mans-
ismprimarilymamdisordermofmchildren,malthoughmamsimilarmconditionmismseen
moccasionallyminmadultsmandmismcharacterizedmbymabruptmonsetmofmbruising,
mpetechiae,mandmmucosalmbleeding
RODAKS 6TH EDITION QUESTIONS AND
ANSWERS
Glanzmannmthrombastheniam-mans-
Describedmasmambleedingmdisordermassociatedmwithmabnormalminmvitromclot
mretractionmandmnormalmplateletmcount
-
mNormalmpltmcount,mnormalmpltmmorphology,mlackmofmpltmaggregation,mredu
cedmclotmformation
Bernard-Souliermsyndromem-mans-
ismamraremdisordermofmplateletmadhesionmthatmusuallymmanifestsminminfancy
mormchildhoodmwithmhemorrhagemcharacteristicmofmdefectivemplateletmfunction
-mNormalmaggregation,mepinephrine,mcollagen,mandmarachidonicmacid
-
mDomnotmrespondmtomristocetinmandmhavemdiminishedmresponsemtomthrombi
n
DensemGranulemDeficienciesm-mans-
Thesemsyndromesmaremclassifiedmundermthemgroupmofm?
-mHermansky-Pudlakmsyndrome
-mChediak-Higashimsyndrome
-mWiskott-Aldrichmsyndrome
-mThrombocytopenia-absentmradiusm(TAR)msyndrome
a-GranulemDeficiencesm-mans-
Thesemsyndromemismclassifiedmundermthemgroupmof?
-mGraymplateletmsyndrome
Chediak-Higashimsyndromem-mans--
mRaremautosomalmrecessivemdisordermcharacterizedmbympartialmoculocutaneou
smalbinism,mfrequentmpyogenicmbacterialminfections,mgiantmlysosomalmgranules
minmcellsmofmhematologicmandmnonhematologicmorigin
Wiskott-Aldrichmsyndromem-mans-RaremX-
linkedmdiseasemcausedmbymmutationsminmthemWASmgenemonmthemshortmar
, mmofmthemXmchromosomemXp11.23mthatmencodesmformam502-
aminoacidmprotein
GraymPlateletmSyndromem-mans-
Plateletsmfrommthesempatientsmcontainmabsentmormmarkedlymreducedma-
granulemproteinsmincludingmPF4,mvWF,mfibronectin,mandmfactormV
Themmostmconsistentmlaboratorymabnormalitymhasmbeenmimpairmentminmthrom
bin-mediatedmaggregationmandmsecretion
150,000mtom450,000/uLm-mans-Normalmplateletmcount:
Thrombocytopeniam-mans-
ismthemmostmcommonmcausemofmclinicallymimportantmbleeding
Petechiaem-mans-
aremsmallmpinpointmhemorrhagesmaboutm1mmmminmdiameter
Ecchymosesm-mans--marem1mcmmormlargermandmusuallymirregularminmshape
-mlaymterm:mbruise
May-HegglinmAnomalym-mans-
autosomalmdominantmtraitmdisordermwheremneutrophilsmhavemblue-
stainingminclusionsmthatmresemblemDohlembodies;mthrombocytopeniamismalsom
presentmwithmgiantmabnormalmplatelets
MYH9m-mans-UndermMay-
HegglinmAnomaly,mtheremismammutationminmwhatmgene?
TARmSyndromem-mans-
Raremautosomalmrecessivemdisordermcharacterizedmbymseveremneonatalmthrom
bocytopeniamandmmutationminmthemRBM8Amgene
FanconimAnemiam-mans-
Associatedmwithmthrombocytopeniamandmothermabnormalitiesmandmaremmostly
mduemtommutationsminmonemofmthem3mgenes,mFANCA,mFANCC,mFANCG
NeonatalmThrombocytopeniam-mans-Plateletmcountmofm<150,000/
mLmandmismpresentminm1-5%mofminfantsmatmbirth
AcutemITPm-mans-
ismprimarilymamdisordermofmchildren,malthoughmamsimilarmconditionmismseen
moccasionallyminmadultsmandmismcharacterizedmbymabruptmonsetmofmbruising,
mpetechiae,mandmmucosalmbleeding