Paroxysmal cold hemoglobinuria (PCH)
PCHParoxysmal 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.
Antibodies
An antibody is a protein produced by the body's immune system when it detects harmful substances, called antigens. Examples of antigens include micr...
Causes
PCH 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.
Symptoms
Symptoms may include:
- Chills
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Fever
Fever
Fever is the temporary increase in the body's temperature in response to a disease or illness. A child has a fever when the temperature is at or abov...
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Leg pain
Leg pain
Leg pain is a common problem. It can be due to a cramp, injury, or other cause.
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Abdominal pain
Abdominal pain
Abdominal pain is pain that you feel anywhere between your chest and groin. This is often referred to as the stomach region or belly.
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Headache
Headache
A headache is pain or discomfort in the head, scalp, or neck. Serious causes of headaches are rare. Most people with headaches can feel much better...
ImageRead Article Now Book Mark Article - General discomfort, uneasiness, or ill feeling (malaise)
Malaise
Malaise is a general feeling of discomfort, illness, or lack of well-being.
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Blood in the urine (red urine)
Blood in the urine
Blood in your urine is called hematuria. The amount may be very small and only detected with urine tests or under a microscope. In other cases, the...
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Exams and Tests
Laboratory tests can help diagnose this condition.
- Bilirubin levels are high in blood and urine.
-
Complete blood count (CBC) shows anemia.
Complete blood count
A complete blood count (CBC) test measures the following:The number of white blood cells (WBC count)The number of red blood cells (RBC count)The numb...
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Coombs test is negative.
Coombs test
The Coombs test looks for antibodies that may stick to your red blood cells and cause red blood cells to die too early.
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Donath-Landsteiner test is positive.
Donath-Landsteiner test
The Donath-Landsteiner test is a blood test to detect harmful antibodies related to a rare disorder called paroxysmal cold hemoglobinuria. These ant...
Read Article Now Book Mark Article - 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)
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 Complications
Complications may include:
- Continued attacks
- Kidney failure
-
Severe anemia
Severe anemia
The Coombs test looks for antibodies that may stick to your red blood cells and cause red blood cells to die too early.
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When to Contact a Medical Professional
Contact 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.
Prevention
People who have been diagnosed with this disease can prevent future attacks by staying out of the cold.
References
Michel 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.
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.
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