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Thrombocytopenia
What we will cover today:Normal platelet production and survival
Thrombocytopenic bleedingApproach to thrombocytopeniaITP
DICCases
April 16, 2 1, Dr! D!"ee
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Thrombocytopoiesis
PLURIPOTENTSTEM CELL
COMMITTEDPROGENITOR
CELL
RECOGNIZABLEBONE MARROW
PRECURSOR CELL
MATUREBLOODCELL
myeloblastmonoblast
pronormoblast red cellneutrophilmonocyte
basophil
platelet
CFU-Baso
CFU-Eos
CFU-GMBFU-E/CFU-E
eosinophil
pre-T
pre-B
myeloidproge itor
!ell
lymphoid
lymphoblast
lymphoblast
T-cell
B-cell& plasma cell
MI"EDPROGENITOR
CELL
C#U$Meg mega%aryo!ytepl&ripote t'tem !ell
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Platelet formation
megakaryocyte formation ofdemarcationmembranes
platelets
(pro)platelets
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Platelets in the circulation:Influx, efflux, and redistribution
CIRCULATINPLAT!L!T"
"PL!!N
#!"TRUCTI$Nor R!%$&AL
PR$#UCTI$N
platelet count( ) thrombopoietin
!"#
$"#
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Thrombopoietin
increased
normal
t(rom)o!ytope ia
*TPO+ ,ree
*TPO+total normal
normal
ormal platelet !o& t
platelet
T%
plasma
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Thrombocytopenic bleedin'
#is$ o% bleedingplatelet countcause o% thrombocytopenia
comorbid diseasedrugs
Clinical mani%estationspetechiaepurpura, ecchymoses
mucosal bleedingmenorrhagiaintracranial bleeding
Ris( of thrombocytopenichemorrha'e in A%L
)
*)
+)
)
-)
) +) -) .) /) *))
Platelet count 0x *)123L4
5 d a y s 6
i t h ' r o s s
h e m o r r
h a ' e
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Approach to thrombocytopenia
' aplasia' in iltration' ine ecti e me*a+aryopoiesis e*, M .' selecti e impairment o plateletproduction
Causes o splenome*aly' in ection' in lammation' con*estion' mali*ancy' red cell disorders' stora*e diseases
' immune auto-immune ( T%0 .1E dru*s in ections allo-immune' non-immune sepsis C0 TT%0 2U. hypertensi e disorders o pre*nancy
loo+ or splenome*aly bone marro3 in esti*ationre ie3 meds
loo+ or underlyin* disordersre ie3 meds
T-ROMBOC.TOPENIA
r&le o&t p'e&dot(rom)o!ytope ia
SE/UESTRATION PRODUCTION DESTRUCTION
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ITP
idiopathic autoimmune plateletdestruction
&1 cause o% isolated thrombocytopenia inother'ise healthy young personsa diagnosis o% e(clusion
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ITP: Clinical features
occurs in any age or se(, but typicallyyoung %emale
can be preceeded by viral in%ectionsigns and symptoms depend on plateletcountonset usually insidious
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ITP: Laboratory features
ITP I) A DIA*N+)I) + -.C"/)I+Nno sensitive and speci0c test %or ITP
isolated thrombocytopeniaincreased Pnormal PT, PTT
bone marro' investigation not essential instraight%or'ard cases
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ITP: TreatmentPatient is not bleedin'
plt 3 4 5 #( not indicatedplt 2 4 5 #( usually not needed, monitor
closelyplt 7 2 5 #( indicated 'ith one or more o%5prednisoneI I*
anti D i% #h possplenectomy i% relapsing severe ITP
8No role %or prophylactic platelet trans%usion, even i% plt 9 :
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ITP: TreatmentPatient is bleedin'
or serious bleeding 8eg! CN),retroperitoneal, *I:
Prednisone and I I* Trans%use plateletsconsider urgent splenectomyProvide other supportive;resuscitative care as needed
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ITP: Pro'nosis
Children5 usually permanent remission
Adults5 usually relapsing 8chronic ITP:, butcourse is relatively benign!
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#isseminated Intra7ascularCoa'ulation 0#IC4
DIC is characteri
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Causes of #IC
In%ection bacterial sepsis, viralin%ections
Neoplasm A ", adenocarcinoma+bstetrical disorders retained dead %etus,abruption, etc
Trauma;surgery brain in=ury, crush, burns, etc!
+thers acute hemolytic trans%usionreaction, etc!
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depletion of clotting factorsprolonged PT, PTT
increased FDP and D-dimer
thromboctyopenia (consumption)
LA8$RAT$R9%ANI !"TATI$N"
microangiopathic hemolytic anemia
decreased fibrinogen
depletion of physiologic anticoagulants
tissue factor release
activation of intrinsicpathway of coagulation
(systemic thrombingeneration)
generali ed intravascular fibrin deposition
underlying disorder
activation offibrinolytic system(systemic plasmin
generation)
PAT;$P;9"I$L$ IC!&!NT"
Pathophysiolo'y of #IC
hemorrhage
thrombosis!infarction
CLINICAL%ANI !"TATI$N"
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Treatment of #IC
treat the underlying diseasereplacement therapy
cryoprecipitatePplatelet concentratepac$ed red cells
consider additional pharmacologic therapycontroversial or investigational agents> AT, APC, PC concentrate, heparin, anti0brinolytic
agents!
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The most important stuff toremember:
Approach to thrombocytopeniaITP diagnosis and management
?hen to 'orry about a lo' platelet count
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Thrombocytopenia: Case *
A previously healthy 23 year old female competitive lacrosse player presents to your office with a three day history of increased ruisin! and petechiae" #er only medications are napro$en and anoral contraceptive" %hysical e$am shows petchiae on the le!s andseveral small ruises on the e$tensor surfaces"
&eu'ocytes ($ 10 9)&* 6"8 +4"0 , 11"0-#emo!lo in (!)&* 130 +120,160-
./ (f&* 87 +80 , 100-%latelet count ($ 10 9)&* 11 +150 , 450-.% (f&* 12.5 +7"4 ,10"4-
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Thrombocytopenia: Case *
#ow do you approach this pro lem dia!nostically
#ow you mana!e this patient and what do you advise hera out her activities and medications
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Thrombocytopenia: Case +A / / comes ac' on a 75 year old man who is new to your practice"
&eu'ocytes ($ 10 9)&* 3.6 +4"0 , 11"0-#emo!lo in (!)&* 127 +140 , 180-./ (f&* 101.5 +80 , 100-%latelet count ($ 10 9)&* 56 +150 , 450-.% (f&* 8"1 +7"4 ,10"4-
eticulocytes ($ 10 9)&* 86 +18 , 94-
eutrophils ($ 10 9)&* 1.3 +2,7"5"0-
&ymphocytes ($ 10 9)&* 1"6 +1"5,4"0-.onocytes ($ 10 9)&* 0"7 +0"2,0"8-
osinophils ($ 10 9)&* 0 +0,0"7-asophils ($ 10 9)&* 0 +0,0"1-
o platelet clumps are seen on the peripheral lood film"
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Thrombocytopenia: Case +What is the differential diagnosis?
12 folate deficiencyhypersplenismalcoholmedicationsmyelodysplasiaother one marrow patholo!y
How do you sort this out?
tain a history and e$amine the patient"ltrasound of the a domen (spleen si e*"
:erum 12 / folate one marrow investi!ation"
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Thrombocytopenia
What we hopefully have covered today:Normal platelet production and survival
Thrombocytopenic bleedingApproach to thrombocytopeniaITP
DICCases