Paroxysmal cold hemoglobinuria is a condition�in which antibodies are formed that attack a specific type of blood cell. These antibodies bind (attach) to red blood cells and to a cell-lysing (destroying)protein in the blood called complement. This only occurs in the cold, and usually affects only the hands and feet.

The cell destruction occurs when the blood cells become rewarmed by circulating through the body. As the cells are destroyed, hemoglobin is released into the blood and passed in the urine.

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

Recovery from the attacks is very quick, and people with this disease do not experience symptoms between episodes. Usually, the attacks end as soon as the affected cells are no longer in circulation. The risk factors are viral infection and syphilis. The disorder is rare.

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

• Positive Donath-Landsteiner test
• Serum hemoglobin increased during attacks
• Urine hemoglobin increased
• CBC shows anemia
• Coombs test is negative
• Bilirubin is elevated
• LDH is elevated

No specific treatment exists for cases when the cause is unknown. Cases caused by syphilis respond to treatment for the underlying disorder.

Many cases will resolve without treatment. In some people, the attacks may occur repeatedly for many years.

• Recurrence of attacks
• Severe anemia
• Kidney failure

Call your health provider if symptoms of this disorder develop (to rule out other causes of the symptoms, and to determine if treatment is needed).

People known to have this disease can prevent future attacks by limiting exposure to the cold.