Causes

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 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.

Symptoms

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

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.
• Level of hemoglobin is increased during attacks.
• 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)

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.

Possible Complications

• 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 doctor can rule out other causes of the symptoms and decide whether you need treatment.

Prevention

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

Alternative Names

PCH

References

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

Update Date: 2/8/2012

Reviewed by: Todd Gersten, MD, Hematology/Oncology, Palm Beach Cancer Institute, West Palm Beach, FL. Review provided by VeriMed Healthcare Network; Linda J. Vorvick, MD, Medical Director and Director of Didactic Curriculum, MEDEX Northwest Division of Physician Assistant Studies, Department of Family Medicine, UW Medicine, School of Medicine, University of Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.