Donath–Landsteiner hemolytic anemia

Donath–Landsteiner hemolytic anemia is a rare form of hemolytic anemia characterized by the presence of Donath-Landsteiner antibodies, which are autoantibodies that react with red blood cells at cold temperatures and cause their destruction upon warming. This condition is also known as paroxysmal cold hemoglobinuria (PCH).

Etiology

Donath–Landsteiner hemolytic anemia is typically caused by the production of Donath-Landsteiner antibodies, which are IgG autoantibodies. These antibodies bind to red blood cells in cold temperatures and cause hemolysis, or destruction of the red blood cells, when the body is warmed. The exact cause of the production of these antibodies is unknown, but it is often associated with viral infections or autoimmune diseases.

Symptoms

The symptoms of Donath–Landsteiner hemolytic anemia can vary, but they often include fatigue, pallor, jaundice, and dark urine. In severe cases, patients may experience hemoglobinuria, or the presence of hemoglobin in the urine, and hemolytic crisis, a rapid decrease in red blood cells leading to severe anemia.

Diagnosis

Diagnosis of Donath–Landsteiner hemolytic anemia is typically made through a combination of clinical history, physical examination, and laboratory testing. The Donath-Landsteiner test, which involves exposing a patient's blood to cold and then warming it, is used to detect the presence of Donath-Landsteiner antibodies.

Treatment

Treatment for Donath–Landsteiner hemolytic anemia is primarily supportive and may include blood transfusions, corticosteroids, and immunosuppressive therapy. In severe cases, plasmapheresis may be used to remove the Donath-Landsteiner antibodies from the blood.

Prognosis

The prognosis for Donath–Landsteiner hemolytic anemia is generally good, with most patients experiencing a complete recovery. However, in some cases, the condition can be chronic and require long-term management.

See also

• Hemolytic anemia
• Autoimmune hemolytic anemia
• Cold agglutinin disease