Paroxysmal cold hemoglobinuria (PCH)

• Illustrations
• Alternative names
• Definition
• Causes, incidence, and risk factors
• Symptoms
• Signs and tests

• Treatment
• Expectations (prognosis)
• Complications
• Calling your health care provider
• Prevention

Alternative names    Return to top

Definition    Return to top

PCH is a rare blood disorder involving antibody formation that destroysВ red blood cells after an affectedВ person is exposed to the cold.

Causes, incidence, and risk factors    Return to top

Paroxysmal cold hemoglobinuria is a conditionВ in which antibodies are formed that attack a specific type of blood cell. These antibodies bind (attach) to red blood cells and to a cell-lysing (destroying)protein in the blood called complement. This only occurs in the cold, and usually affects only the hands and feet.

The cell destruction occurs when the blood cells become rewarmed by circulating through the body. As the cells are destroyed, hemoglobin is released into the blood and passed in the urine.

The disease has been associated with secondary syphilis, tertiary syphilis, and other infections (caused by viruses or bacteria). Sometimes the cause is unknown.

Recovery from the attacks is very quick, and people with this disease do not experience symptoms between episodes. Usually, the attacks end as soon as the affected cells are no longer in circulation. The risk factors are viral infection and syphilis. The disorder is rare.

Symptoms    Return to top

• Chills
• Fever
• Back pain
• Leg pain
• Abdominal pain
• Headache
• General discomfort, uneasiness, or ill feeling (malaise)
• Blood in the urine (red urine)

Signs and tests    Return to top

• Positive Donath-Landsteiner test
• Serum hemoglobin increased during attacks
• Urine hemoglobin increased
• CBC shows anemia
• Coombs test is negative
• Bilirubin is elevated
• LDH is elevated

Treatment    Return to top

No specific treatment exists for cases when the cause is unknown. Cases caused by syphilis respond to treatment for the underlying disorder.

Expectations (prognosis)    Return to top

Many cases will resolve without treatment. In some people, the attacks may occur repeatedly for many years.

Complications    Return to top

• Recurrence of attacks
• Severe anemia
• Kidney failure

Calling your health care provider    Return to top

Call your health provider if symptoms of this disorder develop (to rule out other causes of the symptoms, and to determine if treatment is needed).

Prevention    Return to top

People known to have this disease can prevent future attacks by limiting exposure to the cold.