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OXYGENATION:
HEMATOLOGIC
PATHOPHYSIOLOGYALLEN OCTAVIANO CUDIAMAT
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OUTLINE
ANEMIAS
POLYCYTHEMIA
ALTERATIONS OF PLATELET AND
COAGULATION
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anemia
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Normal hemaolo!i" #arameer$
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anemia reduction in the number of erythrocytes% in he
concentration of hemoglobin an&'or in he hematocrit a$
lon! a$ he oal (loo& )ol*me i$ normal+
"la$$i,i"aion a""or&in! o:
cell volume -mean "or#*$"*lar )ol*me% MCV.:
mi"ro"/i"% normo"/i"% or ma"ro"/i"
raio o, Hb concentration/erythrocyte count -mean
"or#*$"*lar hemo!lo(in% MCH.:
h/#o"hromi"% normo"hromi"% or h/#er"hromi"+
Paho!enei" &i)i$ion o, he anemia$ re,le"$ he
in&i)i&*al steps of erythropoiesis a$ 0ell a$ he life-span
o, he er/hro"/e$ "ir"*lain! in (loo& -hemolytic
anemia.+
a"*e or "hroni" blood loss "an al$o lea& o anemia+
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anemia
Causes of Disorders of erythropoiesis: 1. la"2 or a($en"e o, &i,,ereniaion o, #l*ri#oen% hemo#oiei"
$em "ell$ -aplastic anemia in #anm/elo#ah/ or a"*e m/eloi&
le*2emia.3
4. ran$ien -)iral in,e"ion. or "hroni" re&*"ion o, onl/ he
er/hro"/i" #re"*r$or "ell$ -isolated aplastic anemia. &*e oa*oani(o&ie$ a!ain$ er/hro#oiein or a!ain$ mem(rane
#roein$ o, he #re"*r$or "ell$3
5. er/hro#oiein &e,i"ien"/ in renal ,ail*re -renal anemia.3
6. "hroni" in,lammaion or *mor$ ha "an a"i)ae% amon!
oher$% er/hro#oie$i$7 inhi(iin! inerle*2in$ -secondaryanemia.3
8. a(normal "ell &i,,ereniaion -ine,,e"i)e er/hro#oie$i$.%
0hi"h in a&&iion o !ene &e,e"$ma/ mainl/ (e &*e o a
&e,i"ien"/ in ,oli" a"i& or )iamin 914 -megaloblastic anemia.3
. a(normal H( $/nhe$i$ -microcytic hypochromic anemia.+
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anemia
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anemia
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mani,e$aion$
COMMON:
;ea2ne$$% ,ai!*e% !eneral malai$e% AND #allor o, he$2in an& m*"o*$ mem(rane$ -$"lera% oral m*"o$a.+
in"rea$e& "ar&ia" 0or2loa&: a"h/"ar&ia% #al#iaion$%
&/$#nea% &i
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mani,e$aion$
PERNICIOUS
#eri#heral n*m(ne$$ an& #are$he$ia$% aa=ia% #oor"oor&inaion% an& "on,*$ion+
OTHERS:
>a*n&i"e 7 megao!asti" a#emia or hemoyti" a#emia$
Ton!*e i$ (ee,/ re& an& $ore 7 megao!asti" a#emia
an!*lar "heilo$i$ % "orner$ o, he mo*h ma/ (e
*l"erae& ? IDA& megao!asti"
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#ol/"/hemia
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POLYCYTHEMIA
in"rea$e& )ol*me o, R9C$+
hemao"ri i$ ele)ae& -o morehan 88@ in male$% more han
8@ in ,emale$.+
eiher #rimar/ -POLYCYTHEMIAVERA. or $e"on&ar/+
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POLYCYTHEMIA VERA #roli,erai)e &i$or&er in 0hi"h he m/eloi& $em "ell$ $eem o
ha)e e$"a#e& normal "onrol me"hani$m$+
The (one marro0 i$ h/#er"ell*lar% an& he R9C% ;9C% an&
#laele "o*n$ in he #eri#heral (loo& are ele)ae&+
R9C ele)aion i$ #re&ominan3 he hemao"ri "an e="ee& @+
Thi$ #ha$e "an la$ ,or an e=en&e& #erio& -1 /ear$ or lon!er.+
The $#leen re$*me$ i$ em(r/oni" ,*n"ion o, hemao#oie$i$ an&
enlar!e$+
O)er ime% he (one marro0 ma/ (e"ome ,i(roi"% 0ih a
re$*lan ina(ili/ o #ro&*"e a$ man/ "ell$-B(*rn o* or $#en#ha$e.+
The &i$ea$e e)ol)e$ ino m/eloi& mea#la$ia 0ih m/elo,i(ro$i$
or AML in a $i!ni,i"an #ro#orion o, #aien$3 hi$ ,orm o, AML i$
*$*all/ re,ra"or/ o $an&ar& reamen$
The me&ian $*r)i)al ime e="ee&$ 18 /ear$
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POLYCYTHEMIA VERA
MANI'ESTATIONS: r*&&/ "om#le=ion an& $#lenome!al/ -enlar!e&
$#leen.+
in"rea$e& (loo& )ol*me: hea&a"he% &i
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POLYCYTHEMIA VERA
COMP(ICATION: CVA -(rain aa"2% $ro2e.
hear aa"2 -MI.
hrom(oi" "om#li"aion$ are he mo$,re*en "a*$e o, &eah+
9lee&in! 7 #laele$ -o,en )er/ lar!e. are
$ome0ha &/$,*n"ional+ no$e(lee&$% *l"er$% an& ,ran2 !a$roine$inal
(lee&in!+
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SECONDARY POLYCYTHEMIA
e="e$$i)e #ro&*"ion o, er/hro#oiein+CAUSES: re&*"e& amo*n o, o=/!en -h/#o=i" $im*l*$.:
"i!aree $mo2in!% "hroni" o($r*"i)e #*lmonar/
&i$ea$e% or "/anoi" hear &i$ea$e
non#aholo!i" "on&iion$ $*"h a$ hi!h ali*&e+
"erain hemo!lo(ino#ahie$ in 0hi"h he
hemo!lo(in ha$ an a(normall/ hi!h a,,ini/ ,or
o=/!en -e!% hemo!lo(in Che$a#ea2e.+
Neo#la$m$ -e!% renal "ell "ar"inoma. ha
$im*lae er/hro#oiein #ro&*"ion+
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aleraion$ o, #laele an&
"oa!*laion
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Plaele an& "oa!*laion &i$or&er$
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hrom(o"/o#enia Lo0 #laele "o*n
MANI'ESTATIONS:
9lee&in! an& #ee"hiae *$*all/ &o no o""*r 0ih #laele
"o*n$ !reaer han 8%'mm5
e="e$$i)e (lee&in! "an ,ollo0 $*r!er/ or oher ra*ma+
#laele "o*n (elo0 4%'mm5% #ee"hiae "an a##ear% alon!
0ih no$e an& !in!i)al (lee&in!% e="e$$i)e men$r*al (lee&in!%
an& e="e$$i)e (lee&in! a,er $*r!er/ or &enal e=ra"ion$+
#laele "o*n i$ le$$ han 8'mm5: $#onaneo*$% #oeniall/
,aal "enral ner)o*$ $/$em or !a$roine$inal hemorrha!e "ano""*r+
I, he #laele$ are &/$,*n"ional &*e o &i$ea$e -e!% MDS. or
me&i"aion$ -e!% a$#irin.% he ri$2 o, (lee&in! ma/ (e m*"h
!reaer e)en 0hen he a"*al #laele "o*n i$ no $i!ni,i"anl/
re&*"e&+
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idiopathi" throm!o"ytope#i"purpura )ITP*
#re"i$e "a*$e: *n2no0n
)iral in,e"ion$ $omeime$ #re"e&e ITP in "hil&ren+
me&i"aion$ $*"h a$ $*l,a &r*!$ "an in&*"e ITP+
S/$emi" l*#*$ er/hemao$*$ -SLE. or #re!nan"/% "an
al$o in&*"e ITP+
Ani7#laele a*oani(o&ie$ ha (in& o he
#aien$ #laele$ are ,o*n& in he (loo& o, #aien$
0ih ITP+ ;hen he #laele$ are (o*n& (/ he
ani(o&ie$% he RES or i$$*e ma"ro#ha!e $/$em
in!e$$ he #laele$% &e$ro/in! hem+ The (o&/
aem#$ o "om#en$ae ,or hi$ &e$r*"ion (/
in"rea$in! #laele #ro&*"ion 0ihin he marro0+
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MANI'ESTATIONS
are ea$/ (r*i$in!% hea)/ men$e$% an& #ee"hiae on he e=remiie$
or r*n2+
DRY PURPURA: $im#le (r*i$in! or #ee"hiae
en& o ha)e ,e0er "om#li"aion$
;ET PURPURA:
(lee&in! ,rom m*"o$al $*r,a"e$% $*"h a$ he !a$roine$inal ra"
-in"l*&in! he mo*h. an& #*lmonar/ $/$em -e!% hemo#/$i$.
!reaer ri$2 ,or inra"ranial (lee&in!
De$#ie lo0 #laele "o*n$% he #laele$ are /o*n! an& )er/
,*n"ional+ The/ a&here o en&ohelial $*r,a"e$ an& o one anoher%
$o $#onaneo*$ (lee&in! &oe$ no al0a/$ o""*r+
idiopathi" throm!o"ytope#i"
purpura )ITP*
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HEMOPHILIA
Hemo#hilia A i$ "a*$e& (/ a !enei" &e,e" ha re$*l$ in &e,i"ienor &e,e"i)e ,a"or VIII3
Hemo#hilia 9 -Chri$ma$ &i$ea$e. $em$ ,rom a !enei" &e,e" ha
"a*$e$ &e,i"ien or &e,e"i)e ,a"or IX+
Hemo#hilia i$ a relai)el/ rare &i$ea$e3 hemo#hilia A i$ hree ime$
more "ommon han hemo#hilia 9+ 9oh /#e$ o, hemo#hilia are inherie& a$ X7lin2e& rai$% $o almo$
all a,,e"e& #eo#le are male$3 ,emale$ "an (e "arrier$ (* are
almo$ al0a/$ a$/m#omai"+
The &i$ea$e i$ re"o!ni
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HEMOPHILIA
Hemorrha!e$ ino )ario*$ #ar$ o, he (o&/+ The ,re*en"/ an& $e)eri/ o, he (lee&in! &e#en&
on he &e!ree o, ,a"or &e,i"ien"/ a$ 0ell a$ he
inen$i/ o, he #re"i#iain! ra*ma+
>OINT 9LEEDING ? @ o, #aien$
"hroni" #ain or an2/lo$i$ -,i=aion. o, he oin
Hemaoma$
Inra"ranial (lee&$
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Di$$eminae& inra)a$"*lar
"oa!*laion -DIC. normal hemo$ai" me"hani$m$ are
alere& $o ha a ma$$i)e amo*n o, in/
"lo$ ,orm$ in he mi"ro"ir"*laion+ Iniiall/% he "oa!*laion ime i$ normal+
Ho0e)er% a$ he #laele$ an& "loin!
,a"or$ are "on$*me& o ,orm he
mi"rohrom(i% "oa!*laion ,ail$+ Th*$% he #ara&o=i"al re$*l o, e="e$$i)e
"loin! i$ (lee&in!+
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DIC
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DIC
PATHOPHYSIOLOGY
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DIC
MANI'ESTATIONS: (lee& ,rom m*"o*$ mem(rane$%
)eni#*n"*re $ie$% an& he
!a$roine$inal an& *rinar/ ra"$+
ran!e ,rom minimal o""*l inernal
(lee&in! o #ro,*$e hemorrha!e
or!an &/$,*n"ion% $*"h a$ renal ,ail*re
an& #*lmonar/ an& m*li,o"al "enral
ner)o*$ $/$em in,ar"ion$ a$ a re$*l o,
mi"rohrom(o$e$% ma"rohrom(o$e$% or
hemorrha!e$+
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DIC Re"o!ni
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DIC
Re"o!ni
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DIC
COMP(ICATIONS:
Renal ,ail*re
Gan!rene P*lmonar/ em(oli$m or hemorrha!e
Alere& le)el o, "on$"io*$ne$$
A"*e re$#iraor/ &i$re$$ $/n&rome Sro2e
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&an2e