I.

Introduction

Immune-mediated hemolytic anemia (IHA) or Autoimmune Hemolytic Anemia (AIHA) is a

condition where the immune system makes antibodies (proteins) that attack your red blood cells as
if they were substances foreign to the body. These disorders affect women more often than men.
About half of the time, the cause of autoimmune hemolytic anemia cannot be determined
(idiopathic autoimmune hemolytic anemia).

The presence of the following disease can increase one’s risk for IHA: Autoimmune diseases,
such as lupus, Chronic lymphocytic leukemia, Non-Hodgkin's lymphoma and other blood cancers,
Epstein-Barr virus, Cytomegalovirus, Mycoplasma pneumonia, Hepatitis, and HIV.

Destruction of red blood cells by auto antibodies may occur suddenly, or it may develop
gradually. In some people, the destruction may stop after a period of time. In other people, red
blood cell destruction persists and becomes chronic. There are two main types of autoimmune
hemolytic anemia: warm antibody hemolytic anemia and cold antibody hemolytic anemia. In the
warm antibody type, the autoantibodies attach to and destroy red blood cells at temperatures equal
to or in excess of normal body temperature. In the cold antibody type, the autoantibodies become
most active and attack red blood cells only at temperatures well below normal body temperature.

II. Etiology

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 temperatures ≥ 37° C. They may arise spontaneously or in association with certain
diseases (SLE, lymphoma, chronic lymphocytic leukemia). Some drugs stimulate production of
autoantibodies against Rh antigens. Other drugs stimulate production of autoantibodies against the
antibiotic–RBC-membrane complex as part of a transient hapten mechanism. In warm antibody
hemolytic anemia, hemolysis occurs primarily in the spleen. It is often severe and can be fatal. Most
of the autoantibodies in warm antibody hemolytic anemia are IgG. Most are panagglutinins and
have limited specificity.

Cold type Of IHA or Cold agglutinin disease (cold antibody disease) is caused by
autoantibodies that react at temperatures < 37° C. It sometimes occurs with infections (especially
mycoplasmal pneumonias or infectious mononucleosis) and lymphoproliferative states; about half
of cases are idiopathic, which is the common form in older adults. Infections tend to cause acute
disease, whereas idiopathic disease tends to be chronic. The hemolysis occurs largely in the
extravascular mononuclear phagocyte system of the liver. The anemia is usually mild (Hb > 7.5
g/dL). Autoantibodies in cold agglutinin disease are usually IgM. The higher the temperature (ie,
the closer to normal body temperature) at which these antibodies react with the RBC, the greater the
hemolysis.