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.
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