Paroxysmal cold hemoglobinuria (PCH)

Paroxysmal cold hemoglobinuria (PCH) is a rare blood disorder in which the body's immune system produces antibodies that destroy red blood cells when a person goes from cold to warm temperatures.

Causes of Paroxysmal cold hemoglobinuria (PCH)

Paroxysmal cold hemoglobinuria only occurs in the cold, and affects mainly the hands and feet. Antibodies attach (bind) to red blood cells, which allows other proteins in the blood (called complement) to also latch on. The antibodies destroy the red blood cells as they move through the body and get rewarmed. As the cells are destroyed, hemoglobin, the part of red blood cells that carries oxygen, is released into the blood and passed in the urine.

PCH has been linked to secondary syphilis, tertiary syphilis, and other viral or bacterial infections. Sometimes the cause is unknown.

The disorder is rare.

Paroxysmal cold hemoglobinuria (PCH) Symptoms

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

Tests and Exams

Laboratory tests can help diagnose this condition.

• Bilirubin levels are high in blood and urine
• 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.

Treatment of Paroxysmal cold hemoglobinuria (PCH)

Treating the underlying condition can help. For example, if PCH is caused by syphilis, symptoms may get better when the syphilis is treated.

In some cases, medicines that suppress the immune system are used.

Prognosis (Outlook)

Persons with this disease often get better quickly and do not have symptoms between episodes. Usually, the attacks end as soon as the damaged cells stop moving through the body.

Potential Complications

• Continued attacks
• Kidney failure
• Severe anemia

When to Contact a Health Professional

Call your health care provider if you have symptoms of this disorder. The doctor can rule out other causes of the symptoms and decide whether you need treatment.

Prevention of Paroxysmal cold hemoglobinuria (PCH)

Persons who have been diagnosed with this disease can prevent future attacks by staying out of the cold.

References

Schafer AI. Thrombotic disorders: hypercoagulable states. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 179.