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Paroxysmal cold hemoglobinuria (PCH) |
| Definition: |
| PCH is a rare blood disorder caused by antibody formation that destroys the red blood cell after a person is exposed to the cold. |
| Alternative Names: |
| PCH |
| Causes, incidence, and risk factors: |
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Paroxysmal cold hemoglobinuria is a condition where antibodies are formed against a specific blood group. These antibodies bind the red blood cells and a cell-lysing blood protein called complement only in the cold, and usually in the extremities (hands and feet). When the blood cells are rewarmed in the body, they are lysed (broken down). The disease has been associated with secondary syphilis, tertiary syphilis, and viral infections. Sometimes the cause is unknown. Hemoglobin is released in the blood and passed in the urine as blood cells are broken down. Recovery from the attacks occurs very quickly, and people with this disease do not experience symptoms between episodes. Usually, the attacks end as soon as the sensitized cells are gone from the circulation. The risk factors are viral infection and syphilis. The disorder is rare. |
| Symptoms: |
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| Signs and tests: |
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| Treatment: |
| No specific treatment exists for cases when the cause is unknown. Cases caused by syphilis respond well to treatment for the underlying disorder. |
| Expectations (prognosis): |
| Many cases will resolve without treatment. In some people, the attacks may occur repeatedly for many years. |
| Complications: |
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| Calling your health care provider: |
| 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). |
| Prevention: |
| People known to have this disease can prevent future attacks by limiting exposure to the cold. |
Reviewed By: Rebecca Elstrom, M.D., Division of Hematology-Oncology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
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