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8/16/2019 Anemia Hemolítica Autoinmunitaria.plus http://slidepdf.com/reader/full/anemia-hemolitica-autoinmunitariaplus 1/22  Anemia hemolítica autoinmunitaria UNIVERSIDAD NACIONAL AUTONOMA DE MÉXICO FACULTDAD DE MEDICINA HEMATOLOGÍA ALUMNA: LUNA MONTAÑO KAREN AIRETH GRUPO: 3921

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Page 1: Anemia Hemolítica Autoinmunitaria.plus

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 Anemia hemolítica

autoinmunitaria

UNIVERSIDAD NACIONAL AUTONOMA DE MÉXICOFACULTDAD DE MEDICINA

HEMATOLOGÍA

ALUMNA: LUNA MONTAÑO KAREN AIRETHGRUPO: 3921

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∗ Destrucción prematura de los eritrocitos yanemia causada por autoanticuerpos, que seunen a la superfcie del eritrocito con activacióndel complemento.

∗ Erir!"i!# #!$ $!r%&'(# ) (#r*"*r&' +,*$"i!$&'-. #i /i($ *$& "!$0i"i$ ((r$& 0( '!#(rir!"i!# r!4!"& 5(%'i#i# r(%&*r&6

DEFINICI7N

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FISIOPATOLOGÍA

EXTRAVASCULAR INTRAVASCULAR

Características

Er&4&#"*'&r I$r&4&#"*'&r6

I8G

L!# (rir!"i!# !#!$i&0!# !r"!%'(%($! ! &$i"*(r!# #!$,&8!"i&0!# !r "'*'&# 0('#i#(%& r(i"*'!($0!('i&' 0(5;8&0! + /&!6

I8M

E' (rir!"i! #( 0i#!"i& ($ '& '*0(' 4&#! + #* 5(%!8'!/i$& (#'i/(r&0& 0ir("&%($( &' !rr($(#&$8*;$(!6

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Extravascular Intravascular

H/ (# ,r&8%($&0& !r ($i%&#6Hi(rr! (# &'%&"($&0& ! #( r(8r(#&& '& MO &r& r(*i'i&r#(6Gr*!: H(% (# 0(#"!%*(#! &(r&irr!'(#

H/ ,r&8%($& ($ 0;%(r!# < +=H&!8'!/i$&6Gr*! H(% > A'/?%i$& +5(%!(i$&6

LDH. @I A*%($! 0( r(i"*'!"i!#6E#,(r!"i!# ($ (' ,r!i#6Pr*(/& 0( C!!%/# : >

LDH. @I A*%($! 0( r(i"*'!"i!#6E#B*i#!"i!# ($ ,r!i#Di#%i$*"i$ 0( 5&!8'!/i$&#6

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∗ L!# &*!&$i"*(r!# &&r("($ "!%! "!$#("*($"i&:∗ D(#r(8*'&"i$ 0(' #i#(%& i$%*$i&ri!∗ Pr0i0& 0( '& !'(r&$"i& i$%*$i&ri&

∗ E!#i"i$ & *$ &$;8($! #i%i'&r & *$ &$;8($! r!i!∗ N(!'&#i& 0( L"@6

∗ Gr&4(0&0 0( '& &$(%i&:∗ C&r&"(r;#i"&# 0(' &*!&$i"*(r!∗ F&"!r(# 0(' &"i($( )(0&0 + ($,(r%(0&0(# 0( /&#(-∗ C&&"i0&0 0( '& %0*'& #(& 0( "!%($#&r '&

5(%!'i#i#6

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I$,(""i!$(# 4;ri"&# + %i"!i"&#: AHA !r &$i"*(r!# ,r;!#6∗ S;'i# + M+"!'&#%&

∗ Fr%&"!#: A#!"i&0!# & AHA !r &$i"*(r!# "&'i($(#6∗ A',& %(i'0!&∗ C(,&'!#!ri$&#

∗ P($i"i'i$&∗ S*'/&"&%∗ C'&4*'&$&!∗ P'&i$!#

FACTORES DE RIESGO

5:6"($(("6#&'*068!/6%0(#"&r8&#8"C&

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∗ C&r&"(r;#i"&# 0(' &*!&$i"*(r! + ('%("&$i#%! 0( '& 5(%'i#i#:

16 AHA !r &$i"*(r!# B*( r(&""i!$&$ &' "&'!r626 AHA !r &$i"*(r!# B*( r(&""i$ &' ,r;!6

16 E$,(r%(0&0 !r "ri!&8'*i$&#26 H(%!8'!/i$*ri& &r!;#i"& !r ,r;!

36  Ti! %i!6

C'&#i"&"i$

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∗ M# ,r("*($(6∗ A0*'!# %&+!r(# 0( &!#6∗ M*J(r(#6∗

A$i"*(r! I8G ) I8G 1. I83-∗  S($#i/i'i&$ &' (rir!"i! & 3 C

A$i"*(r!# B*( r(&""i!$&$ &'"&'!r6

Idiopática Secundaria

 Tr&#!r$!#'i$,!r!'i,(r&i4!#)'(*"(%i& 'i$,!";i"&"r$i"&. 'i$,!%& 0( L"@- Tr&#!r$!#&*!i$%*$i&ri!#: LES.&rrii# r(*%&!i0(6

M(J;& MH6 Anemias hemolíticas autoinmunes. R(4 IMSS3 )1-:2261Q. "!$#*'&0! ($ '& (/ )11-:

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∗ I$i"i! #*('( #(r i$#i0i!#!6∗ C*r#! *(0( #(r &8*0! ! "r$i"!. r(%i#i!$(#

+ (&"(r/&"i!$(#6∗

S;$!%& 0( &$(%i& )"&$#&$"i!. %&r(!#.0i#$(&-6∗ Fi(/r(∗ I"(ri"i&∗

E#'($!%(8&'i& + 5(&!%(8&'i&6

C*&0r! "';$i"!:

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Pr*(/&# 0( '&/!r&!ri!Resultado

PAD )Pr*(/& 0( &$i8'!/*'i$&0ir("&-

>

@i'irr*/i$& i$0ir("& E'(4&0&

V('!"i0&0 0( #(0i%($&"i$

8'!/*'&r

E'(4&0&

D(#5i0r!8($&#& '"i"& E'(4&0&

Pr(#($"i& 0( *r!/i'i$& ($ 5("(#+ !ri$&

>

Fr!i# 0( #&$8r( P!'i"r!%&#i&. (#B*i#!"i!# +

esferocitosPr*(/& 0( C!!%/# 0ir("! >

M(J;& MH6 Anemias hemolíticasautoinmunes. R(4 IMSS3 )1-:226 1Q. "!$#*'&0! ($ '& (/)11-:

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∗ Síndrome de Evans: Anemia se acompaña de púrpura tromocitopenia inmunitaria ydisminución de recuentos de plaquetas.

Tratamiento:• Pr(0i$i#!$&: 12 %8 80;& 0*r&$( & Q #(%&$&#6

• Ri*i%&/: 0!#i# #(%&$&'(# 0( 3%8%2 0(#*# 0( '&4&"*$&"i$ &$i$(*%!""i"& + &$i5(%!5i'*#6

5:6"($(("6#&'*068!/6%0(#"&r8&#8"C&

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AHA !r "*(r!#,r;!#

A$i"*(r!# IgM B*( #( J&$ &'"!%'(%($! + r(&""i!$&$i%&%($( & *$& (%r(r&*r&32C

S;$0r!%( 0( A8'*i$i$&#,r;&# ! "ri!8'*i$i$&#

H(%!8'!/i$*ri&&r!;#i"& &' ,r;!66

C*&0r! "';$i"!:

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∗ E# (' %# 0( '!# 0!# "*&0r!# "';$i"!#6∗ 33 0( !0!# '!# "&#!# 0( AHAI6

∗ R('&"i$ "!$:∗ !ycoplasma pneumoniae.

"E#∗ $!" 

∗ C*&0r! C';$i"!:∗ P&"i($(# 0( Q &!#∗ Trastornos del ujo vascular o emolisis:

∗ !edos "crocianosis

∗ #rejas

∗ $unta de la narí%&

∗ $olineuropatía

∗ Sx& 'iperviscosidad locali%ada&

E$,(r%(0&0 !r

"ri!&8'*i$i$&#

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D&!# 0( '&/!r&!ri!Resultado

PAD )Pr*(/& 0( &$i8'!/*'i$&0ir("&-

>

@i'irr*/i$& i$0ir("& N!r%'& E'(4&0&

V('!"i0&0 0( #(0i%($&"i$

8'!/*'&r

E'(4&0&

D(#5i0r!8($&#& '"i"& E'(4&0&

H(%!8'!/i$*ri& >

Pr*(/& 0( C!!%/# 0ir("! >

H(%!#i0(ri$*ri& >

Fr!i#: Es(uistocitos. (#,(r!"i!# +,!r%&"i$ 0( i'&# 0( %!$(0&#6

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 Tr&&%i($!

Recomendaciones

M&$($(r '& (%(r&*r& !r

&rri/& 0( '& "*&' ('&*!&$i"*(r! #( J&

I$%*$!#*r(#!r(# Ci"'!,!#,&%i0&C'!r&%/*"i'!I$(r,(r$ &',&

"i0! ,'i"! S!#i($( '& (rir!!+(#i#"!%($#&!ri&6Pr(4i($( *$& "ri#i# &'#i"&6

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∗ S( 4i$"*'& "!$ '& #i'i# "!$8($i& ! &0B*iri0&6∗ El autoanticuerpo involucrado es el Donath-Lantsteiner .

∗ %&'.

∗ (emolisina muy poderosa y i)asíca * se f+a a los

eritrocitos a -$/ y al complemento a 01$.$ausando hemolisis súita.

H(%!8'!/i$*ri& &r!;#i"& !r

,r;!6

5:6%(0i8r&5i"6"!%0,#i%##i%2i%#1860, 

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∗ Ni!# "!$ &$("(0($ 0( ($,(r%(0&0 4ir&'r(4i&6

∗ Pr*(/& 0( C!!%/#: >∗

H(%!8'!/i$*ri& 0(#*# 0( '& (!#i"i$ &',r;!6

C*&0r! "';$i"!6

H/ 81%'

P'&#%& R!Ji!

Fr!i# Erir!,&8!"i!#i#.

%i"r!(#,(r!"i!#@H L(*"!($i& #(8*i0& !r

'(*"!"i!#i# $(*r!,;'i"&6

Pr*(/& 0( D!$&5L&$#(i$(r6

 >

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∗ Tratamiento:

∗ E4i&r (' ,r;!6∗ E$ "&#!# &8*0!#:

∗ S*('( &*!'i%i&r#(  R("*(r&"i$ ($ 0;&#∗  Tr&$#,*#i$ 0( #&$8r(

 &i%( Pr( 6C6 (ematolo&ía. 2a san&re y sus en)ermedades. E0i!ri&' M" Gr&Hi''6 3W (0 Mi"!

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∗ R(r(#($& (' 0( '!# "&#!#∗ I8G  3C∗ I8M Fr;!∗ N&*r&'(& i0i!&i"&

∗ )2- #( &#!"i&$ & LES + LNH + LH6

Mi&

 &i%( Pr( 6C6 (ematolo&ía. 2a san&re y sus en)ermedades. E0i!ri&'M" Gr& Hi''6 3W (0 Mi"!

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Pr!0*""i$0(

&$i"*(r!

4# ,r%&"!

A$i"*(r!#( *$( &',r%&"!

C!%'(J!i$%*$( #(

*$( &'

(rir!"i!

AHI &0B*iri0& !r ,r%&"!#:

H(%!'i#i# i$r&4&#"*'&r6A"!%&& 0( ,&''& r($&'6)uinidina&

C!%'(J!# i$%*$(#:

M(J;& MH6 Anemias hemolíticas autoinmunes. R(4 IMSS3 )1-:226 1Q."!$#*'&0! ($ '& (/ )11-:

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Fr%&"! #(*$( &'

(rir!"i!

Pr!0*""i$0( *$

&$i"*(r!0iri8i0! &',r%&"!

H(%!'i#i#0('

(rir!"i!6

$enicilina* cefalosporinaH(%!'i#i# (r&4&#"*'&r6

HAPTENO:

AUTOANTICUERPO

"lfametildopaA$i"*(r!# #!$ "'&#( I8G "!$ (#("i"i0&0 &$i#i#(%&R56

M(J;& MH6 Anemias hemolíticas autoinmunes. R(4 IMSS3 )1-:226 1Q. "!$#*'&0! ($ '& (/)11-:

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∗ 5:6"($(("6#&'*068!/6%0(#"&r8&#8"C&

∗ 5:

6%(0i8r&5i"6"!%0,#i%##i%2i%#1860, 

∗ &i%( Pr( 6C6 (ematolo&ía. 2a san&re y susen)ermedades. E0i!ri&' M" Gr& Hi''6 3W (0Mi"!6

∗ M(J;& MH6 Anemias hemolíticas autoinmunes.R(4 IMSS3 )1-:226 1Q. "!$#*'&0! ($'& (/ )11-:

@i/'i!8r&,;&6