Paroxysmal cold hemoglobinuria (PCH)

Definition

PCH is a rare blood disorder caused when a person is exposed to the cold, and the immune system makes proteins called antibodies that destroy red blood cells.

Alternative Names

Causes

Paroxysmal cold hemoglobinuria only occurs in the cold, and affects mainly the hands and feet. Antibodies are formed that attack one type of blood cell. These antibodies attach (bind) to red blood cells and to proteins in the blood (called complements) that destroy cells. The blood cells are destroyed when they move through the body and get rewarmed. As the cells are destroyed, the part of red blood cells, called hemoglobin, which carries oxygen is released into the blood and passed in the urine.

The disease has been linked to secondary syphilis, tertiary syphilis, and other infections (diseases caused by viruses or bacteria). Sometimes the cause is unknown.

People with this disease often get better quickly and do not have symptoms between episodes. Usually, the attacks end as soon as the affected cells stop moving through the body. The risk factors are infection with a virus and syphilis

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

• Donath-Landsteiner test is positive
• Serum hemoglobin is higher during attacks
• Urine hemoglobin is higher
• CBC shows anemia
• Coombs test is negative
• Bilirubin is high
• LDH is high

Treatment

The disease that caused the disorder can be treated. Sometimes, treatment that decreases the immune system (immunosuppressive therapy) is used. Cases caused by syphilis get better when the syphilis is treated.

Outlook (Prognosis)

Many cases will get better without treatment. In some people, the attacks may happen many times over the years.

Possible Complications

• Attacks continue
• Severe anemia
• Kidney failure

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

People who know they have this disease can prevent future attacks by staying out of the cold.

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Hoffman R, Benz E, Shattil S, Furie B, Cohen H. Hematology: Basic Principles and Practice. 4th ed. Philadelphia, Pa: Churchill Livingstone; 2004.

Roddr EG, Hillmen P, Schreiber AD. Immune-mediated hemolytic anemia. Hematology Am Soc Hematol Educ Program. January 2004;48-62.