Paroxysmal cold hemoglobinuria
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Donath-Landsteiner syndrome; PCH
Paroxysmal cold hemoglobinuria is a rare disease that is associated with intravascular hemolysis and hemoglobinuria after exposure to cold. The Donath-Landsteiner antibody sticks to the red blood cells in the cold which causes a complement-mediated lysis upon rewarming.
In people with PCH, a polyclonal IgG autoantibody binds to red blood cell surface antigens in the cold. This can occur in a susceptible individual as blood passes through cold extremities in cold weather. When the blood returns to the warmer central circulation, the red blood cells are lysed with complement, causing intravascular hemolysis. Hemoglobinuria and anemia can then occur. The anemia may be mild or severe.
PCH can be an after effect of an infection, when a microorganism triggers the formation of antibodies that cross-react with the P antigen on the red blood cell membrane. Viral infections that can cause PCH include measles, mumps, influenza, adenovirus, chickenpox, cytomegalovirus, and Epstein-Barr virus. Bacterial infections that can cause PCH include syphilis, Haemophilus influenzae and Mycoplasma pneumoniae. PCH can also be a side effect of some vaccinations. Chronic idiopathic (of unknown cause) PCH also occurs, but it is rare.
Acute PCH tends to be transient and self-limited, particularly in children. Chronic PCH associated with syphilis resolves after the syphilis is treated with appropriate antibiotics. Chronic idiopathic PCH is usually mild.
People with PCH should avoid exposure to cold temperatures. If anemia is severe, blood transfusion may be needed. Careful compatibility testing by the blood bank is necessary because autoantibodies may interfere with blood typing. Prednisone may be used in individuals with PCH and severe anemia.
Differential Diagnosis of Causes of Paroxysmal cold hemoglobinuria
In alphabetical order: [1] [2]
- Anemia
- Chicken Pox
- Congenital Syphillis
- Glandular fever
- Measles
- Mumps
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