Cold antibody hemolytic anemia
Cold Antibody Hemolytic Anemia Cold Antibody Hemolytic Anemia (CAHA) is a rare autoimmune disorder characterized by the premature destruction of red blood cells (hemolysis) due to the presence of cold-reacting autoantibodies. This condition is a type of autoimmune hemolytic anemia (AIHA) and is often associated with cold agglutinins, which are antibodies that cause red blood cells to clump together at low temperatures.
Pathophysiology[edit | edit source]
The pathophysiology of CAHA involves the production of IgM antibodies that bind to red blood cells at temperatures below normal body temperature, typically in the peripheral circulation. These antibodies activate the complement system, leading to the destruction of red blood cells. The hemolysis primarily occurs in the liver and spleen, where the complement-coated red blood cells are removed from circulation.
Symptoms[edit | edit source]
Patients with CAHA may experience a range of symptoms, including:
- Fatigue
- Pallor
- Jaundice
- Dark urine
- Cold-induced acrocyanosis (bluish discoloration of the extremities)
- Raynaud's phenomenon
Diagnosis[edit | edit source]
The diagnosis of CAHA is based on clinical presentation and laboratory findings. Key diagnostic tests include:
- Direct antiglobulin test (DAT), which is positive for complement (C3) and sometimes IgM.
- Cold agglutinin titer, which measures the level of cold agglutinins in the blood.
- Complete blood count (CBC) showing anemia and reticulocytosis.
- Peripheral blood smear showing agglutinated red blood cells.
Treatment[edit | edit source]
Treatment of CAHA focuses on managing symptoms and preventing hemolysis. Strategies include:
- Avoidance of cold exposure to prevent activation of cold agglutinins.
- Use of warm clothing and heated environments.
- In severe cases, immunosuppressive therapy such as rituximab may be used.
- Blood transfusions may be necessary in cases of severe anemia.
Prognosis[edit | edit source]
The prognosis of CAHA varies depending on the underlying cause and severity of the disease. Some patients may experience mild symptoms, while others may have significant hemolysis requiring medical intervention. The condition can be chronic or associated with other diseases such as infections or lymphoproliferative disorders.
See Also[edit | edit source]
External Links[edit | edit source]
- National Organization for Rare Disorders
- [MedlinePlus](https://medlineplus.gov)
NIH genetic and rare disease info[edit source]
Cold antibody hemolytic anemia is a rare disease.
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Contributors: Prab R. Tumpati, MD
