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Paroxysmal cold hemoglobinuria (PCH)

Definition

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.

Alternative Names

PCH

Causes

Paroxysmal cold hemoglobinuria 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.

  • Bilirubin levels are high in blood and urine.
  • Complete blood count (CBC) shows anemia.
  • Coombs test is negative.
  • Donath-Landsteiner test is positive.
  • Lactate dehydrogenase level is high.

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:

  • Continued attacks
  • Kidney failure
  • Severe anemia

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.

References

Jager U, Lechner K. Autoimmune hemolytic anemia. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 44.

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

Review Date:2/1/2016
Reviewed By:Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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