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PCH DefinitionParoxysmal 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. CausesPCH 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. SymptomsSymptoms may include:
Exams and TestsLaboratory tests can help diagnose this condition.
TreatmentTreating 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 ComplicationsComplications may include:
When to Contact a Medical ProfessionalContact your health care provider if you have symptoms of this disorder. Your provider can check for other causes of the symptoms and decide whether you need treatment. PreventionPeople who have been diagnosed with this disease can prevent future attacks by staying out of the cold. ReferencesMichel M. Autoimmune and intravascular hemolytic anemias. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 146. Michel M, Jäger U. Autoimmune hemolytic anemia. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 47. | |
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Review Date: 3/31/2024 Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited. | |