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Autoimmune

Hemolytic Anemias

autoimmune hemolytic anemia is


caused by autoantibodies that
react with RBCs at
temperatures37C (warm
antibody hemolytic anemia)
or<37C (cold agglutinin
disease).
Hemolysis is usually
extravascular.

Classification of
AIHAs
Warm Antibody AIHA (warm AIHA)

Cold Antibody AIHA (cold agglutinin


syndrome; CAS)
Paroxysmal Cold Hemoglobinuria (PCH)
Combined Cold and Warm (Mixed AIHA)

Warm antibody hemolytic


anemia

Warm antibody hemolytic anemia is


the most common form of
autoimmune hemolytic anemia
(AIHA); it is more common among
women. Autoantibodies in warm
antibody hemolytic anemia generally
react at temperatures37C.

etiology

Spontaneously
In association with certain disorders
(SLE, lymphoma, chronic
lymphocytic leukemia)
After use of certain drugs :
methyldopa,levodopa,quinidine,
sulfonamide

Cold agglutinin disease

Cold agglutinin disease (cold


antibody disease) is caused by
autoantibodies that react at
temperatures<37C

etiology

Infections (especially mycoplasmal


pneumonias or infectious
mononucleosis)
Lymphoproliferative disorders
Idiopathic (about half of cases)

Paroxysmal cold
hemoglobinuria

Paroxysmal cold hemoglobinuria (PCH;


Donath-Landsteiner syndrome) is a rare
type of cold agglutinin disease. Hemolysis
results from exposure to cold, which may
even be localized (eg, from drinking cold
water, from washing hands in cold water).
An IgG autohemolysin binds to RBCs at
low temperatures and causes
intravascular hemolysis after warming.

Assays for hemolytic anemia (eg,


peripheral smear, reticulocyte count;
sometimes urinary hemosiderin,
serum haptoglobin)
Direct antiglobulin test

Immune Hemolytic Anemias


DIRECT ANTIGLOBULIN
TEST

Test for IgG and/or complement coating the patients red blood cells

Treatment

For drug-induced warm antibody hemolytic


anemia, drug withdrawal, sometimes IV
immune globulin
For idiopathic warm antibody hemolytic
anemia, corticosteroids (eg,prednisone1
mg/kg po once/day or higher doses) are
the treatment of choice in idiopathic warm
antibody AIHA
For cold agglutinin disease, avoidance of
cold

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