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Paroxysmal cold hemoglobinuria (PCH)

PCH

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

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Causes

PCH only occurs in the cold, and affects mainly the hands and feet. Antibodies attach (bind) to red blood cells. This 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. 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.

Symptoms

Symptoms may include:

Exams and Tests

Laboratory tests can help diagnose this condition.

Treatment

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.

Outlook (Prognosis)

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

Possible Complications

Complications may include:

When to Contact a Medical Professional

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

Prevention

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

Related Information

Antibody
Hemoglobin

References

Michel M. Autoimmune and intravascular hemolytic anemias. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 160.

Win N, Richards SJ. Acquired haemolytic anaemias. In: Bain BJ, Bates I, Laffan MA, eds. Dacie and Lewis Practical Haematology. 12th ed. Philadelphia, PA: Elsevier; 2017:chap 13.

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Review Date: 1/19/2018  

Reviewed By: Richard LoCicero, MD, private practice specializing in hematology and medical oncology, Longstreet Cancer Center, Gainesville, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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