Paroxysmal cold hemoglobinuria
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Paroxysmal cold hemoglobinuria Classification & external resources | |
ICD-10 | D59.6 |
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ICD-9 | 283.2 |
DiseasesDB | 9679 |
eMedicine | ped/512 |
Paroxysmal cold hemoglobinuria (PCH) (also known as Donath-Landsteiner syndrome) is a disease of humans that is characterized by the sudden presence of hemoglobin in the urine (called hemoglobinuria), typically after exposure to cold temperatures.
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[edit] Presentation
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.
[edit] Causes
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.
[edit] Treatment and prognosis
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.
[edit] Risk factors
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.
[edit] See also
[edit] References
This article does not cite any references or sources. (August 2007) Please help improve this article by adding citations to reliable sources. Unverifiable material may be challenged and removed. |
Thank you so much for the information!
ReplyDeleteI found another article that cross-checks well with the info on Wikipedia, and also has some more information. http://www.patient.co.uk/showdoc/40025291/
The thing that stuck out to me is that the acute form is described as "transient" and "self-limiting" and is more common in children. The chronic form is milder and mostly in adults. So hopefully this will be done after a couple of months of keeping warm.
And thank God that spring is coming!