Paroxysmal cold hemoglobinuria
Paroxysmal cold hemoglobinuria
Description, Causes and Risk Factors:
Paroxysmal cold hemoglobinuria is a rare blood disorder in which the body's immune system produced antibodies that destroy red blood cells when they go from cold to warm temperatures.
PCH is an autoimmune disorder - a disorder in which the body's natural defenses against invading organisms destroy healthy tissue for unknown reasons. In PCH, antibodies mistakenly attack red blood cells causing the cells to breakdown prematurely, a condition called (hemolysis). When antibodies attack healthy tissue, they are referred to as autoantibodies.
In most children, PCH occurs following a viral infection such as measles, mumps or chickenpox and spontaneously resolves once the infection is treated. Usually, PCH does not recur (self-limited) in children, but recurrent cases have been reported in the medical literature. In most adults the cause of PCH is unknown (idiopathic). In the past, most cases of PCH in adults were associated with syphilis. With the dramatic decline in the prevalence of syphilis, PCH in adults has declined, and the current understanding of the adult form of the disease is less clear.
Antibodies (which are also known as immunoglobulins) are specialized proteins that bind to invading organisms and bring about their destruction. There are five main classes of antibodies - IgA, IgD, IgE, IgG, and IgM. In PCH, a specific autoantibody known as the Donath-Landsteiner autoantibody is produced often in response to a viral infection. This autoantibody binds to red blood cells during exposure to cold temperatures. The Donath-Landsteiner autoantibody is a type of IgG antibody.
Acute cases of the disease are characterized by an abrupt onset with features of severe intravascular hemolysis including high fever, chills, back and/or leg pain. Other symptoms may include nausea, headache, vomiting and diarrhea. Typically hemoglobinuria occurs, producing dark red to black urine. Hemolysis can be severe and even life-threatening and results from exposure to cold, which may even be localized (e.g. from drinking cold water, from washing hands in cold water). Chronic forms of PCH are characterized by recurrent episodes of hemolysis precipitated by cold exposure.
A majority of cases of paroxysmal cold hemoglobinuria recorded in the early medical literature were associated with late syphilis or congenital syphilis. In the early 1900s over 90 percent of patients with chronic PCH had a positive test for syphilis and approximately 30 percent showed clinical evidence of the disease. With the effective treatment of syphilis and the virtual elimination of the congenital form, "classical" syphilitic PCH is now an extremely rare disorder, as is chronic PCH. In modern times, PCH is almost always encountered as an acute transient syndrome in young children with a recent history of a viral illness, so that paroxysms resulting from cold exposure are rarely encountered.
PCH affects males and females in equal numbers. The disorder is rare in the United States and the prevalence and incidence rates are unknown.
Symptoms may include:
General discomfort, uneasiness, or ill feeling (malaise).
Blood in the urine.
A diagnosis is made based upon a thorough clinical evaluation, a detailed patient history, identification of characteristic symptoms and a variety of specialized tests:
Complete blood count (CBC) shows anemia.
Coombs test is negative.
Donath-Landsteiner test is positive.
Level of hemoglobin is increased during attacks.
Lactate dehydrogenase level is high.
Bilirubin levels are high in blood and urine.
The treatment of PCH has received relatively little recent attention. The rarity of the disease makes controlled therapeutic trails with adequate numbers of patients difficult. In addition, prophylactic avoidance of cold is sufficient to control paroxysmal attacks and correct anemia in most cases of idiopathic PCH. PCH associated with syphilis generally responds to penicillin.
Fisher recently reported an inconclusive trial of combined immunosuppressive therapy (steroids and azathioprine) in a patient with non-syphilitic PCH. Splenectomy is not usually of value in PCH, although occasional long-term remission has been reported.
In some cases, severe anemia may require a red blood cell transfusion. In such cases, a transfusion should not be delayed. Use of a blood warmer during transfusion is particularly important.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
A new research from the University of Edinburgh, UK, suggests that cheap cilostazol tablets may reduce damage to arteries, which lead to blood clots, resulting in strokes and cognitive decline. The researchers plan to assess the medications’ ability to cut the risk of...
A new study from the US discovers that flavonoids, natural compounds found in fruits and vegetables, may help preserve the lung function, which tends to decline with age. For the study, a team of researchers looked at data from 463 adults from Norway and England whose...
Quiz about this article
0 of 2 questions completed
Please answer on few questions to make our service more useful
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
0 of 2 questions answered correctly
Time has elapsed
You have reached 0 of 0 points, (0)
Question 1 of 2
Was this article useful for you?Correct
Thanks for your feedback!Incorrect
Thanks for your feedback!
Question 2 of 2
What else information about this disease you want to know ?Correct
Thanks for feedback!Incorrect
Thanks for feedback!
Good weather is the best reason to do outdoor sports, which will help not only lose weight, but also will strengthen health. Bicycle The sun dries out the local paths, so you can safely sit on your favorite bike and confidently twist the pedals, where the eyes look....read more
First aid for injuries consists of simple rules that need to be clearly implemented. If this is a closed injury, you need to immobilize the injured limb, otherwise the person may lose consciousness from a painful shock. If you need to get to the emergency room...read more
Many people spontaneously decide starting to do sport, while others weigh all the pros and cons for a log time. But almost all of them make the same mistakes, listening to the advice of non experts. There are 10 anti-tips for those who want to do plan to do some sport...read more