sîngele alb

8/18/2019 Sîngele Alb

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FIZIOPATOLOGIA SÂNGELUI ALB


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SCHEMA DIFERENŢIERII CELULELOR STEM

Celula-stem hamat!"et"#$ % mult"!te&t$

Celula '(mata(e )e #l&"" !lu("!te&t$ CFC m"el")e CFC l"m'")e

Celula '(&ata(e )e #l&"" u&"!te&t$ *+la,t". - me/a#a("+last - e("t(+last - m"el+last - m&+last - N0 - #elule )e&)("t"#e


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MULTIPLICAREA CELULELOR % STEMA1 Mult"!l"#a(ea #u )"'e(e&2"e(e.

Celula-stem

Cel-stem Celule )"'e(e&2"ate

A1 Mult"!l"#a(ea #u #l&a(e

Celule- stem

Celule-stem


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$% &$'N$* L+CC$TL

Celula stem hematopoietică

Predecesorul comun mieloid

-C.' / factorul stimulator de colonii granulocitar

neutrofile

eo0inofile

ba0ofile

1-C.' / factorul stimulator de colonii monocitar

monocite

2

Predecesorul comun limfocitar3

L-C.' / factorul stimulator de colonii limfocitar

Predecesorul 4-limf Predecesorul T-limf4- limfocite T- limfocite

/


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LEUCOCITELE CIRCULANTE

Conţinutul de leucocite în sânge-

4-9х109/L (4-9 mii/mm3)LEUCOGRAMA.

Neutrofile metmielocite ! 1"

nesegmentte - 4" segmentte - #0"

$o%inofile - 4"

&%ofile - 1"

Limfocite - '"

onocite - "


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NUM3RUL ABSOLUT DE LEUCOCITE

E4em!lu. 56789 :; *5888 :mm


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PATOLOGIA SÂNGELUI ALB

71 P(#ese se#u&)a(e *(ea#t"e (ea#2"a(/a&el( hemat"!"et"#e la !(#ese )"&alte s"steme )e(e/la(ea !(l"'e($(""

?1 P(#ese !("ma(e % !(#ese l#al"ate &(/a&ele hemat!$"et"#e

)e(e/la(ea )"'e(e&2"e("" ," !(l"'e($(""


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71 RROCESE REACTIE

71 Leu##"te?1 Leu##"t!e&""


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71 LEUCOCITOZELE

Leu##"t$ % #(e,te(ea &um$(ulu" )eleu##"te &t(- u&"tate )e lum )e s&/e

Leu##"tele a)e$(ate *a+slute %#(e,te(ea #&2"&utulu" )e leu##"te !este96789 :L #m"te&t #u #(e,te(ea#&2"&utulu" ttal )e leu##"te & (/a&"sm

Pat/e&"a - st"mula(ea !()u#2"e" )e leu##"te


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Leu##"tele 'alse *(elat"e % #(e,te(ea#&2"&utulu" )e leu##"te !este

96789

:L #u !$st(a(ea #&2"&utulu" ttal )eleu##"te & (/a&"sm la &"el &(mal

Pat/e&"a. hem#e&t(a2"a (e)"st("+u"(ea leu##"tel( & !atul as#ula(

m)"'"#a(ea (a!(tulu" )"'e("t( '(me )eleu##"te & s&/e *leu#/(am$ %#(e,te(ea !(#e&tulu" u&e" '(me !e seama

s#$)e("" !(#e&tulu" alte" '(me )e leu##"te


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J1 K& 'u"e )e su(sa )e !(l"'e(a(e a leu##"tel( ,"a '(me" )m"&a&te )e+ leu##"t.

&eut('"le e"&'"le

+a'"le l"m'#"te m&#"te


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.emnifica5ia biologică a leucocito0elor3

Leu##"tele &eut('"le % "mu&"tatea &es!e#"'"#$

a&t"m"#(+"a&$ *"&'e#2"a ##"#$

Leu##"tele e"&'"le %"mu&"tatea &es!e#"'"#$ a&t"!a(a"ta($

L"m'#"tele %

"mu&"tatea s!e#"'"#$ a&t"m"#(+"a&$ M&#"tele % (ea#2"a s"stemulu" ma#('a/al

leu#em"a m&#"ta($ m&&u#lea

"&'lama2"a & 'aa !(l"'e(at"$


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?1 LEUCOCITOPENIILELEUCOCITOPENIE % m"#,(a(ea &um$(ulu" )e

leu##"te &t(- u&"tate )e lum )e s&/e

LEUCOCITOPENIILE a)e$(ate *a+slute %

m"#,(a(ea #&2"&utulu" )e leu##"te & s&/esu+ >6789 :L #m"te&t #u m"#,(a(ea#&2"&utulu" ttal )e leu##"te & (/a&"sm

Pat/e&"a - su!(es"a leu##"t!"ee" )"st(u#2"a leu##"tel( & s&/e

!"e()e(ea leu##"tel(


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Leu##"t!e&""le 'alse *(elat"e %m"#,(a(ea &um$(ulu" )e leu##"te su+

>6789 :L #u !$st(a(ea #&2"&utulu" ttal )eleu##"te & (/a&"sm la &"el &(mal Pat/e&"a.

hem)"lu2"a (e)"st("+u"(ea leu##"tel( & !atul as#ula( m)"'"#a(ea (a!(tulu" )e leu##"te &

leu#/(am$ - s#$)e(ea !(#e&tulu" u&e"'(me !e seama #(e,te("" !(#e&tulu" alte"'(me )e leu##"te


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K& 'u"e )e m"#,(a(ea '"e#$(e" '(me )e leu##"t. Neut(!e&"" E"&!e&""

G(a&ul#"t!e&"" *a/(a&ul#"te L"m'#"t!e&"" M&#"t!e&""


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Sem&"'"#a2"a +"l/"#$ a leu##"t!e&""l(

Neut(!e&""le % "mu&)e'"#"e&2$ &es!e#"'"#$a&t"m"#(+"a&$ *'a/#"ta!(e)"s!"2"e la "&'e#2"a ##"#$

E"&!e&""le % "&)"#$ st(esul h"!e(#(t"#"smul L"m'#"t!e&""le % l"!sa l"m'#"tel( B %

"mu&)e'"#"e&2$ um(al$l"!sa l"m'#"tel( T % "mu&)e'"#"e&2$ #elula($

l"!sa am+el( '(me % "mu&)e'"#"e&2$ m"4t$


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?1 P(#ese !("ma(e DEREGLAREA DIFERENŢIERII I PROLIFER3RII

Hem+lastele % tum(" mal"/&e !(e&"te )"&.

Celulele-stem !lu("!temt$CFC-GEMM - !(e)e#es(ul #mu& m"el")CFC- L"BT - !(e)e#es(ul #mu& l"m'")

CFC u&"!te&te % Me/a- E E BG N#elulele matu(e


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CLASIFICAREA HEMOBLASTOZELOR

5emo6lsto%e

Leucemii Limfome

7cute Cronice


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CLASIFICAREA HEMOBLASTOZELOR71 Leu#em"" % hem+laste #a(e se )elt$ )"&

#elulele hemat!"et"#e ale m$)ue" asel(. a1 Leu#em"" a#ute % hem+laste #a(e se)elt$ )"& #elulele +last"#e ale m$)ue"asel(.

l1a1 L"m'+last"#$ l1a1 M"el+last"#$ l1a1 M&+last"#$

l1a1 E("t(+last"#$ l-a- me/a#a("+last"#$ l1a1 Plasm+last"#$ et#1


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+1 Leu#em"" #(&"#e % hem+laste #a(e se)elt$ )"& #elulele !lu("!te&te alem$)ue" asel( *!(e)e#es(ul #mu&m"el") ," l"m'").

l1#1 G(a&ul#"ta($ l1#1 L"m'#"ta($ l1#1 M&#"ta($ l1#1 E("t(#"ta($

*e("t(em"e l1#1 Me/a#a("#"ta($

l1#- !lasm#"ta($

*m"elm mult"!lu


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?1 L"m'ame % hem+laste #a(e se

)elt$ )"& #elulele hemat!"et"#ee4t(ame)ula(e *l"m'#"te matu(e.

a1 Mala)"a H)/"&

+1 L"m'amele &&-H)/"&


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ETIOLOGIA HEMOBLASTOZELOR

71 Raele "&"a&te

?1 "(u,"


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PATOGENIA GENERAL3 AHEMOBLASTOZELOR

71 Ce!tul #l&al %tuma(ea(e!(e"&t$

ttal"tatea )e#elule mal"/&e!(e&"te )"&t(-s"&/u($ #elul$

muta&t$ % #l&atum(al$ *tuma(em&#l&al$

? C " t 2 l "


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?1 Ce4"ste&2a #l&e"tum(ale#m"te&t #u

!!ula2"a!(e&"t$ )"&#elulele &(male

Clon s.n.tos.

Clon mlign.


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>1 Deta,a(ea #elulel(tum(ale )"&'#a(ul !("ma()"sem"&a(ea#elulel( tum(ale," "&'"lt(a(ea

(/a&el(e4t(a'#ale*e4t(ame)ula(e - metastaa(e

M$)ua asel(

A!a("2"a !("& muta2"e


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1 A!a("2"a !("& muta2"ea su+#l&el(tum(ale *tuma(e

!l"#l&al$ %!(/(es"a tum(al$1 P(/(es"a tum(al$

t(a&s'(m$hem+lasta#(&"#$ & '(maa#ut$

Cl&a I Su+#l&a

F(ma F(ma C(&"#$ a#ut$


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PATOGENIA GENERAL3 A LEUCEMIILOR ACUTE

Leu#em""le a#ute % tum(" mal"/&e )"&#elulele +last"#e ale m$)ue" (,"".e("t(+last m"el+last me/a#a("+last

I&"2"al se mal"/&"ea$ 7 #elul$ #a(e )$

&a,te(e u&e" #l&e mal"/&e Cl&a mal"/&$ se )"sem"&ea$ ,"

#l&"ea$ m$)ua (,"e su+st"tu"&)

2esutul hemat!"et"# &(mal


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La u& a&um"t /(a) )e !(l"'e(a(e mal"/&$ se

"&h"+$ e("t(!"ea *s"&)(mul a&em"#/(a&ul#"t!"ea *s"&)(mul "&'e#2"st(m+#"t!e&"a *s"&)(mul hem(a/"#

Cm"te&t #e4"st$ #elulehemat!"et"#e &(male ," mal"/&e


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Celu"lele mal"/&e a!a( & s&/ele !e("'e("# %+lastem"a

Celulele +last"#e )"& s&/e &'"lt(ea$(/a&ele e4t(ame)ula(e. /a&/l"&" l"m'at"#"s!l"&a '"#atul SNC et#1



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Celula stem hematopoietică (67 linii)

Progenitorul comun mieloid Progenitorul comun limfoid

eritroblast T- limfoblast B-Limfoblast

monoblast

mieloblast

(N,B,)!

megacarioblast

eritroblaşti l i m f o b l a ş t i

monoblaştimieloblaşti

(N,B,)!

megacarioblaşti


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LEUCEMIILE CRONICE

8umori mielo2rolifertie #u !$st(a(ea#a!a#"t$2""u )e )"'e(e&2"e(e mutţi lnielul celulei 2luri2otente ! 2redecesorul

comun mieloid su limfoid cu 2redominre 2rolifer.rii unei serii

LEUCEMIA CRONIC3 GRANULOCITAR3


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LEUCEMIA CRONIC3 GRANULOCITAR3

Tuma(e m"el!(l"'e(at"$ #u !$st(a(ea#a!a#"t$2""u )e )"'e(e&2"e(e

se )elt$ !("& muta2"a la &"elul #elule"

stem !lu("!te&te *!(e)e#es(ul #mu&muel")

a!a(e #l&a mal"/&$ a #elule" stem!lu("!te&te )"& #a(e !("& tate #elulele)es#e&)e&te


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tate /e&e(a2""le #elule" stem !lu("!te&tea!a(2"& #l&e" tum(ale

Muta2"a se ma&"'est$ !("& h"!e(!(l"'e(a(e&e#&t(lat$ )a( #u !$st(a(ea #a!a#"t$2"" )e

)"'e(e&2"e(e !&$ la #elulele matu(e

Celulele mal"/&e su&t "&se&s"+"le la

sem&alele a!!tt"#e ," !"e() a)e""tatea lamat("#ea "&te(#elula($


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Ma(e(ul #l&el( tum(ale este#(mmul Ph"la)el!h"a

Ce4"ste&2a #elulel( mal"/&e ," #el(&(male

Celulele mal"/&e !(l"'e(ea$ ," #l&"ea$m$)ua (,"e

La u& a&um"t /(a) )e !(l"'e(a(e #elulele


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La u& a&um"t /(a) )e !(l"'e(a(e #elulelemal"/&e a!a( & s&/e ," #l&"ea$(/a&ele e4t(ame)ula(e

Ca!a#"tatea &alt$ )e !(l"'e(a(e #&)u#e laa!a("2"a su+#l&el( mal"/&e % su("&etuma(ea !l"#l&al$ ," a#ut"a(ea!(#esulu" tum(al

PATOGENIA LEUCEMIEI CRONICE GRANULOCITARE


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PATOGENIA LEUCEMIEI CRONICE GRANULOCITARE




monoblast

mieloblast

(N,B,)!

megacarioblast

eritrocit T- limfocit B-Limfocit

monocitgranulocit

(N,B,)!

trombocit


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PATOGENIA LEUCEMIEI CRONICE LIMFOCITARE

Leucemi cronic. limfocitr. - 2roces

limfo2roliferti tumore monoclonl. mutţi re loc l nielul celulei 2redecesore

limfo2oie%ei din m.du oselor Celuil mutnt. mlign. d. n:tere clonului de

celule cu diferenţiere 2ân. l limfocite mture

;n 9" este &-celulr. în " - 8-celulr.


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Celulele mal"/&e au muta2"a /e&e" !<*)"m"&ua(ea a!!te" ," e4!(es"a s4#es"$

a BCL-? % a&t"a!!tt"#$

Cl&a mal"/&$ su+st"tu"e alte se("" me)ula(e.

e("t(#"ta( *s"&)(mul a&em"# me/a#a("#"ta( *s"&)(mul hem(a/"# /(a&ul#"ta( *s"&)(mul "&'e#2"s

Celulele mal"/&e "es & #"(#ula2"a sa&/u"&$," "&'"lt(ea$ (/a&ele e$t(ame)ula(e



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monoblast

mieloblast

(N,B,)!

megacarioblast


monocit 89 :89 granulocit

(N,B,)!

trombocit


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PATOGENIA LIMFOAMELOR

Limfomele ! tumori mligne< cre se de%olt.

din celulele limfoide etrmedulre=Limfomele 5odg>in ! ?0" din &-limfocitele

mture '0" - din 8-limfocitele mture le

gnglionilor limfticiLimfomele non-5odg>in ! tumori din limfocitele

& :i 8 din gnglionii limftici cu 6locul l diferite

et2e de 6lsttrnsformre



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monoblast

mieloblast

(N,B,)!

megacarioblast


monocitgranulocit

(N,B,)!

trombocit

sîngele alb

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