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Understanding Mixed Autoimmune Hemolytic Anemia

Mixed autoimmune hemolytic anemia is a rare type of autoimmune hemolytic anemia defined by the presence of both warm and cold autoantibodies that can damage red blood cells at different temperatures. It can be idiopathic or associated with conditions like systemic lupus erythematosus or lymphoma. Diagnosis is based on evidence of hemolytic anemia and detection of both IgG and IgM autoantibodies. Treatment focuses on corticosteroids and splenectomy, but patients also need to avoid cold temperatures to prevent further hemolysis. Mixed AIHA can occur at any age but presents abruptly with severe anemia.
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0% found this document useful (0 votes)
54 views2 pages

Understanding Mixed Autoimmune Hemolytic Anemia

Mixed autoimmune hemolytic anemia is a rare type of autoimmune hemolytic anemia defined by the presence of both warm and cold autoantibodies that can damage red blood cells at different temperatures. It can be idiopathic or associated with conditions like systemic lupus erythematosus or lymphoma. Diagnosis is based on evidence of hemolytic anemia and detection of both IgG and IgM autoantibodies. Treatment focuses on corticosteroids and splenectomy, but patients also need to avoid cold temperatures to prevent further hemolysis. Mixed AIHA can occur at any age but presents abruptly with severe anemia.
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MIXED-TYPE AUTOIMMUNE (HAND OUT)

RENEGADO JANINE P.
Mixed autoimmune hemolytic anemia is a type of autoimmune hemolytic anemia (AIHA;
see this term) defined by the presence of both warm and cold autoantibodies, which
have a deleterious effect on red blood cells at either body temperature or at lower
temperatures.

Etiology

Mixed AIHA can be idiopathic or secondary, mainly associated with systemic lupus
erythematosus (SLE) and lymphoma.

Epidemiology

Mixed AIHA occurs in less than 10% of cases of AIHA, whose annual incidence is
between 1/35,000-1/80,000 in North America and Western Europe.

Laboratory Diagnosis

Diagnosis is based on clinical or laboratory evidence of hemolytic anemia and the


detection of autoantibodies, usually both IgG and IgM, with the direct anti-globulin test
(DAT) showing a pattern of IgG with complement C3, and the presence of cold
agglutinins (IgM) in the serum at a significant titer.

Erroneous diagnosis of mixed AIHA is sometimes made on the basis of inadequate


serologic studies as a large proportion of patients with warm AIHA also have cold
autoantibodies that are measured but are clinically insignificant. Unless a cold
autoantibody with a high thermal amplitude (>30 degrees C) is observed in association
with a warm autoantibody, a diagnosis of mixed AIHA is not warranted.

Treatment

The principles of management are comparable to that of warm AIHA, including


corticosteroids and, if these are ineffective, splenectomy, but avoidance of cold must
also be considered. Cautious transfusion is possible in cases with severe anemia.
However, the best compatible or least incompatible packed red blood cell units must be
identified by the blood center to avoid post-transfusional hemolysis
Clinical Significant

Mixed AIHA can occur at any age but is rare in children. Patients with mixed AIHA
usually present with abrupt onset severe hemolysis and anemia.

Pathophysiology

There are two mechanisms of hemolysis. Intravascular hemolysis is the destruction of


red blood cells in the circulation with the release of cell contents into the plasma.
Mechanical trauma from a damaged endothelium, complement fixation and activation
on the cell surface, and infectious agents may cause direct membrane degradation and
cell destruction.

The more common extravascular hemolysis is the removal and destruction of red blood
cells with membrane alterations by the macrophages of the spleen and liver. Circulating
blood is filtered continuously through thin-walled splenic cords into the splenic sinusoids
(with fenestrated basement membranes), a spongelike labyrinth of macrophages with
long dendritic processes. A normal 8-micron red blood cell can deform itself and pass
through the 3-micron openings in the splenic cords. Red blood cells with structural
alterations of the membrane surface (including antibodies) are unable to traverse this
network and are phagocytosed and destroyed by macrophages.

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