Platelet Disorders -2

8/18/2019 Platelet Disorders -2

1/42

LATELET DISORDERS

QUANTITATIVE ANDQUALITATIVE DISORDERS


2/42

QUALITATIVELATELET

DISORDERS• THROMBOCYTOPENIA

• THROMBOCYTOSIS


3/42

THROMBOCYTO ENIA

• MOST COMMON CAUSE OF ABNORMALBLEEDING AND GENERALLY

ATTTRIBUTED TO THE FF. CAUSES:1. Decrease p a!e e! pr"#$c!%"&'. Decrease# p a!e e! s$r(%(a !%)e #$e !" %&crease

#es!r$c!%"& a*"r c"&s$)p!%"&+. I&crease# p a!e e! se,$es!ra!%"& - !/e sp ee&0 2. D% $!%"& "3 !/e p a!e e! c"$&! - )$ !%p e - ""#

!ra&s3$s%"&s.


4/42

DECREASED LATELETRODUCTION

1. CONGENITAL HYPOPLASIA OF THEMEGA4ARYOCYTES IN THE BM

a5 FANCONI SYNDROME6 #*! pa&c !"pe&%a

-5 TAR SYNDROME6 !/r")-"c !"pe&%a 7* a-se&! ra#%%c5 NE8BORNS AS A RESULT OF INTRAUTERINE

E9POSURE TO DRUGS THIA;IDES5 AND VIRALINFECTIONS RUBELLA5

'. ACQUIRED HYPOPLASIA OF MEGA4ARYOCYTES) DUE TO THERAPEUTIC AGENT ACTIONSTHIA;IDE DIURETICS0 ESTROGEN HORMONE ANDALCOHOL SELECTIVELY DECREASESMEGA4AYOCYTE PRODUCTION


5/42

+. INFILTRATION OF THE BM BY MALIGNANT CELLST/r")-"c !"pe&%a ass"c%a!e# !" s$c/ "&c"


6/42

HEREDITAR !"#DITI"#$ A$$TD %& I#''E!TI(E )*ATE*ET )R"D+!TI"#

a Autosomal dominant thromboc-topeniab Ma- He//lin anomal-c %iscott Aldrich s-ndrome

0. DI$"RDER$ "' THE !"#TR"* "'THR"MB")"EI$I$

) N"! c"))"& Res$ ! 3r") a& %)pa%r)e&! %& !/e)ec/a&%s) !/a! c"&!r" p a!e e! pr"#$c!%"&.

) !-clic Thromboc-topenia %s a c"%!%"& %& 7/%c/!/r")-"c !"pe&%a a &"r)a p a!e e! c"$&!s a !er&a!ea! re


7/42

DECREASE LATELET SURVIVAL TIME

• I#!REA$E )*ATE*ET DE$TR+!TI"#1 IMM+#"*"2I!THR"MB"! T")E#IA

1. IDIOPATHIC THROMBOCYTOPENIC PURPURA ITP5THROMBOCYTOPENIA OCCURS IN THE ABSENCE OF ANY

DISEASE ASSOCIATED 8ITH DECREASE PLATELET ORTO9IN E9POSURE.

a5 Ac$!e ITP '6 ears " # a3!er rec"(er 3r") (%ra %&3ec!%"& se 3%)%!%&<

%. STAINED BLOOD SMEAR prese&!s: "$&


8/42

-5 C/r"&%c ITP6 a#$ ! )"s! ' 62 ears 7")e&%%. C%rc$ a!%&< p a!e e! are "$&< 7* s/"r! %3espa& a I


9/42

'. DRUG INDUCED IMMUNOLOGICTHROMBOCYTOPENIA

a5 A&!%-%"!%cs0 / p&"!%cs0 a&a


10/42

3. IMMUNOLOGICTHROMBOCYTOPENIA

•

C"%!%"& !/a! %s%%s!%&


11/42

6. I+c. )#&')'& c*+su/ &,*+ +*+-,//u+*)* ,c &2r*/(*c$&* '+,#

• T/r")-"!%c !/r")-"c !"pe&%cp$rp$ra TTP56 $&?&"7& e=ac!ca$se ser%"$s #se

a5 He)" !%c a&e)%a6 !ra$)a !"RBC

-5 C/a&


12/42

. NONIMMUNOLOGIC THROMBOCYTOPENIAT/r")-"c !"pe&%a )a -e prese&! %& a &$)-er "3r%c?e!!s%a 0 -ac!er%a 0 (%ra "r )a ar%ainfections due

to Increase consumption of platelets and less commonl-as a result of decrease production.T/r")-"c !"pe&%a re a!e# !" car#%"p$ )"&ar- pass ca& res$ ! 3r") DIC0 #% $!%"&0 se,$es!ra!%"&0

p a!e e! #es!r$c!%"& %& !/e "=


13/42

INCREASED LATELET SEQUESTRATION

• A& a-&"r)a #%s!r%-$!%"& "3 p a!e e!s )a a s"ca$se thromboc-topenia .

• N"r)a !/e sp ee& p"" s appr"=%)a!e "&e6!/%r# "3 !/e !"!a sp ee& sp e&")e


14/42

&2 * / (* $&*. '+ ,#


15/42

DILUTION OF THE LATELET COUNT

Multiple transfusions


16/42

$plenic pool

Transfusion


17/42

THROMBOCYTOSIS• A p a!e e! c"$&! %&crease# a-"(e &"r)a 7% -e

3"$ as a res$ ! 3 a (ar%e! "3 c%rc$)s!a&ces.Reac!%(e !/r")-"c !"s%s


18/42

REACTIVE

THROMBOCYTOSIS• Ge&era resp"s 7/e& !/e %&<

#%s"r#er %s !rea!e#.• F" "7%&< sp e&ec!") 0 !/e p a!e e! c"$&!

7%


19/42

• T/r")-"c !"s%s 3" "7%&< )a "r s$r


20/42

AUTONOMOUSTHROMBOCYTOSIS

Mar?e# %&crease %& !/e p a!e e! c"$&!Ass"c%a!e# 7%!/ !/r")-"!%c a *"r /e)"rr/a


21/42

AUTONOMOUSTHROMBOCYTOSIS


22/42

Thrombocythemia

Middle agepatients (both

male andfemale)

bleeding or thrombosis with bleedingepisodes predominating

(Gastrointestinal hemorrhage)Bleedin

g inarterial

andvenous

circulation

Splenomegaly is

afrequentfinding


23/42

UALITATIVE

PLATELETDISORDER

Hereditary Qualitative PlateletDisorderAcquired Qualitative PlateletDisorder

FunctionalPlatelet Disorder

PlateletAdhesion

PlateletAggregation

PlateletSecretion

orReleaseReaction


24/42

PLATELET ADHESIONDEFECTS

Ber&ar#6S"$ %er S r")e I&/er%!e# as a& a$!"s")a recess%(e !ra%!Br$%s%&< a )"#era!e !" se(ere - ee#%&<

CHARACTERISTICS

G%a&! P a!e e!s ' $) %& #%a)e!er5C"arse


25/42

PL !"L"!

Lack glycoprotein 1b(GP1b)

Lack glycoprotein VAND V

Function asReceptor in

von!ille"rand#actor

$na"le to adherenor%ally tovascular

endotheliu%

Do not "indcoagulation#actor &nor%ally


26/42

CHARACTERISTICS

o MEGA4ARYOCYTE %& BM5 N"r)a !" s %


27/42

("&8% e-ra@s D%sease6 ABSENT "r ABNORMAL 3"r) "3

("&8% e-ra 3ac!"r %)pa%re# p a!e e! a#/es%"&6 NORMAL %& A


28/42

PLATELET AGGREGATIONDEFECTS

A& a


29/42

#iagnose$p a!e e! re!e&!%"& %s )ar?e# %&crease#

p a!e e! c"$&! %s


30/42

A s" ca e# G a&>)a&&@s !/r")-"s!/e&%a6%s )a "r %&/er%!e# - ee#%&< #%s"r#er c/arac!er%>e# - !/e

3a% $re "3 p a!e e!s !" a


31/42

LATELET SECRETION DEFECTS

A secre!%"& #%s"r#er %s 7/e& !/e #a)a


32/42

T7"


33/42

'. Asp%r%&6 %?e #e3ec!s

p a!e e!s /a(e &"r)a


34/42

!hree platelet function disorders involve p a!e e! secre!%"&:1. A p/a Gra&$ e De3%c%e&c 0 ca e# Gra P a!e e! S r")e0 !/ere

%s a ac? "3 %)p"r!a&! pr"!e%&s 7%!/%& !/e a p/a


35/42

HEREDITARY FORMS OF PLATELETDYSFUNCTION

- Ver ar


36/42

ACQUIRED QUALITATIVE

PLATELET DISORDERS- Ac,$%re# #%s"r#ers "3 p a!e e! 3$&c!%"& are ass"c%a!e#

7%!/ a &$)-er "3 c"%!%"&s 7%!/ !/e %&


37/42

• P a!e e! # s3$&c!%"& - ee#%&< #%s"r#ers 7%

-e prese&! %& !/e (ar%"$s parapr"!e%&e)%a.• M$ !%p e ) e ")a 8a #e&s!r")@s

)acr"< "-%&e)%a6a-&"r)a %!%es "3 !/e p a!e e! a


38/42

Ac$!e ) e "- as!%c e$?e)%a• Me


39/42

M e "pr" %3era!%(e #%s"r#ers• p" c !"!/e)%a (era0 c/r"&%c ) e "


40/42

• I&c. a)"$&!s "3 3%-r%&"


41/42

P a!e e! ass"c%a!e# a&!%-"#%es• I"#%"p/a!%c !/"r)-"c !"pe&%a p$rp$ra• A$!"%))$&e #%s"r#ers

6s s!e)e!%c $p$s er !/r")a!"s%s6 A&!%-"#%es /a(e -ee& s/"7& !" ca$se p a!e e! s%s0

p a!e e! a


42/42

Dr$

Platelet Disorders -2

Documents

Transcript of Platelet Disorders -2