Autoimmune Hemolytic Anemia

Autoimmune hemolytic anemia (AIHA) (autoimmune

blood diseases)
(autoantibody)
/ (complements) direct
antiglobulin test (DAT) direct Coombs test

(classification) AIHA
AIHA
1. AIHA (primary idiopathic AIHA)
(secondary AIHA) AIHA
systemic lupus erythematosus (SLE), lymphoproliferative lymphoma, chronic
lymphocytic leukemia (CLL), angioimmunoblastic lymphadenopathy with dysglobulinemia (AILD)
mycoplasma ( 1)

1 autoimmune hemolytic anemia

Systemic lupus erythematosus

Immune thrombocytopenia (Evans syndrome)
Rheumatic disorders scleroderma
Rheumatoid arthritis
Sjogrens syndrome
Thyroiditis
Pernicious anemia
()
Chronic lymphocytic leukemia
Non Hodgkins lymphoma
Angioimmunoblastic lymphadenopathy with dysglobulinemia (AILD)

mycoplasma
Infectious mononucleosis
HIV


Hypogammaglobulinemia
Dysgammaglobulinemia
Wiskott-Aldrich Syndrome

Ovarian tumors cysts
Solid tumors
Ulcerative colitis
2.
(antigen)
2.1 Warm-reacting antibody type AIHA
37 o IgG
IgM IgA immunoglobulins
complements
2.2 Cold-reacting antibody type AIHA
37 o 4 o 2
Cold (hema) agglutinin disease (CHAD) IgM cold
agglutinin (agglutinate) AIHA
CHAD (primary idiopathic)
(secondary) lymphoproliferative disorders CLL, lymphoma
mycoplasma, infectious mononucleosis
Paroxysmal cold hemoglobinuria (PCH) Donathv
Landsteiner antibody IgG (specificity) P

PCH (syphilis)

AIHA warm autoantibody

warm autoantibody type AIHA warn autoantibody AIHA (
)

AIHA
AIHA IgG warm reacting
(immune adherence)
(phagocyte) (macrophage) reticuloendothelial ()

 Fc (gamma) receptor immune adherence

C3b C4b ( ) receptors
immune adherence (phagocytosis)
() (partial phagocytosis)
spherocytes spherocytes Billroths cord
AIHA spherocytes
(extravascular hemolysis)
AIHA
IgG

(intravascular hemolysis) AIHA
CHAD
IgM (periphery)
IgM C1

C3 C3b I C3b
receptor
extravascular hemolysis
intravascular hemolysis

1. AIHA 1
80,000
AIHA (idiopathic) 15
SLE lymphoma
AIHA AIHA
2. AIHA
AIHA 20-30
: 5 : 1 AIHA idiopathic secondary SLE
3. AIHA , ,
, (congestive heart failure)
(onset) 2-3
( 38 o) 39 o

,
AIHA
lymphoproliferative disease
AIHA SLE
AIHA (subacute onset)
2-3 (acute onset)

CHAD ,
(acrocyanosis) Raynauds phenomenon
(blanching phase) (reactive phase)
acrocyanosis (gangrene)

CBC : -
3 g/dl
(acute hemolysis) leucocytosis neutrophilia
Evan SLE
(polychromasia nucleated red cell)
spherocytes anisocytosis autoagglutination
30 AIHA
reticulocyte ( reticulocytosis) AIHA
reticulocyte (0%)

(serological test); -
AIHA
1. Saline agglutination test (NSS) 1
1 slide AIHA
complete antibody IgM CHAD
IgM AIHA warm antibody type mixed warm and cold
antibody type saline agglutination AIHA
2. direct antiglobulin test
(DAT) direct Coombstest incomplete antibody
antiglobulin reagent (antibody) (bridge) incomplete
autoantibody (agglutination) DAT AIHA

 warm antibody incomplete antibody IgG

antiglobulin reagent Ig
AIHA warm antibody Ig Ig
IgG Ig IgM
IgA IgM
3. AIHA
3.1 indirect antiglobulin test (IAT)
(donor erythrocyte) ( O) incubate
antiglobulin reagent IAT
alloantibody
. AIHA warm antibody
IAT
(spill over) IAT AIHA
DAT
3.2 cold agglutinin IgM autoantibody CHAD
complete antibody
I,i CHAD (titer) ()
1: 64 4-32 o
cold agglutinin warm antibody
30% AIHA (mixed cold and warm antibody AIHA) cold agglutinin
37 o
autoagglutination AIHA

AIHA 1) 2)

, , ,
,
()
AIHA

1.
1.1 Glucocorticoids glucocorticoids prednisolone
IgG antibodies prednisolone 1 ././
AIHA
1.2 (immunosuppressive drugs) cytotoxic
alkylating antimetabolic agents cyclophosphamide, azathioprine, chlorambucil
glucocorticoids AIHA 40-50%
glucocorticoids

1.3 Plasmapheresis IgM

IgG antibody IgG
AIHA IgG

1.4 (splenectomy) lymphoid organ

2.
2.1 (splenectomy) mononuclear phagocytes
IgG

2.2 Fc receptors (FcR) phagocyte

receptors
2.2.1 Glucocorticoids prednisone prednisolone

2.2.2 Intravenous immunoglobulin (IVIg) IgG IVIg FcR
receptors Fc
IVIg ITP AIHA FcR
2.2.3 Danazol anabolic agent FcR
ITP AIHA
2.2.4 FcR monoclonal antibody FcR

AIHA

1. Glucocorticoids prednisolone 1-2 ./

./ 60 ./ 100 ./
dexamethasone 5-10 .
6 prednislone
glucocorticoids AIHA warm acting antibody
(initial response) 70-90% 10%
20%

glucocorticoids 2-3
1
2/g/dl/
( 10 g/dl)
10 ./ 60 ./ 50 ./ 2030 ./ 5 ./ 10 ./ 36

prednisolone 40-60 ./

glucocorticoids CHAD
37 o AIHA mixed warm and cold antibody
glucocorticoids ( 20 ./
)

2. (Immunosuppressive drugs) azathioprine 2-2.5 ././,

cyclophosphamide 1-2 ././ AIHA
glucocorticoids
hemolytic crisis acute hemolytic relapse
hemolytic crisis (long-acting drug)
10-14 6
() glucocorticoids
prednisolone 20 ./
prednisolone

 (splenectomy) glucocorticoids
AIHA glucocorticoids
AIHA
IgG warm antibody type 50-70%
CHAD IgM
IVIg, plasmapheresis plasma exchange, anabolic hormone
danazol, vinca-loaded platelets AIHA plasmapheresis
CHAD
(blood transfusion)
AIHA

, (AIHA with
reticulocytopenia),

compatibility

pack red cells
dexamethasone 5 .
( 3-4 .)
hemolytic transfusion CHAD