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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%Hi(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$
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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"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$,(r%(0&0 !r "ri!&8'*i$ 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&$( & Q #(%&$
• Ri*i%&/: 0!#i# #(%&$&'(# 0( 3%8%2 0(#*# 0( '&4&"*$&"i$ &$i$(*%!""i"& + &$i5(%!5i'*#6
5:6"($(("6#&'*068!/6%0(#"&r8"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( %!$(0
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Tr&&%i($!
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"($(("6#&'*068!/6%0(#"&r8"C&
∗ 5:
6%(0i8r&5i"6"!%0,#i%##i%2i%#1860,
∗ &i%( Pr( 6C6 (ematolo&ía. 2a san&re y susen)ermedades. E0i!ri&' M" Gr& Hi''6 3W (0Mi"!6
∗ M(J;& MH6 Anemias hemolíticas autoinmunes.R(4 IMSS3 )1-:226 1Q. "!$#*'&0! ($'& (/ )11-:
@i/'i!8r&,;&6
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