Paroxysmal cold hemoglobinuria (PCH)
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Paroxysmal cold hemoglobinuria (PCH)

Definition

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

Causes, incidence, and risk factors

Paroxysmal cold hemoglobinuria is a condition 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

Signs and tests

Treatment

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

Expectations (prognosis)

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

Complications

  • Recurrence of attacks
  • Severe anemia
  • Kidney failure

Calling your health care provider

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

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

Illustrations

Blood cells
Blood cells

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