07 hematology

8/16/2019 07 Hematology

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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

07 hematology

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