- Paroxysmal cold hemoglobinuria
Paroxysmal cold hemoglobinuria Classification and external resources ICD-10 D59.6 ICD-9 283.2 DiseasesDB 9679 eMedicine ped/512 MeSH D006457
Paroxysmal cold hemoglobinuria (PCH) (also known as Donath-Landsteiner syndrome) is a disease of humans that is characterized by the sudden presence of hemoglobin in the urine (called hemoglobinuria), typically after exposure to cold temperatures.
In people with PCH, a polyclonal IgG anti-P autoantibody binds to red blood cell surface antigens in the cold. This can occur in a susceptible individual as blood passes through cold extremities in cold weather. When the blood returns to the warmer central circulation, the red blood cells are lysed with complement, causing intravascular hemolysis. Hemoglobinuria and anemia can then occur. The anemia may be mild or severe.
PCH is a rare autoimmune haemolytic anaemia that can occur following an infection, when a microorganism triggers the formation of antibodies that cross-react with the P antigen on the red blood cell membrane. Viral infections that can cause PCH include measles, mumps, influenza, adenovirus, chickenpox, cytomegalovirus, and Epstein-Barr virus. Bacterial infections that can cause PCH include syphilis, Haemophilus influenzae and Mycoplasma pneumoniae. PCH can also be a side effect of some vaccinations. Chronic idiopathic (of unknown cause) PCH also occurs, but it is rare.
Treatment and prognosis
Acute PCH tends to be transient and self-limited, particularly in children. Chronic PCH associated with syphilis resolves after the syphilis is treated with appropriate antibiotics. Chronic idiopathic PCH is usually mild.
People with PCH are sometimes advised to avoid exposure to cold temperatures. If anemia is severe, blood transfusion may be needed. Careful compatibility testing by the blood bank is necessary because autoantibodies may interfere with blood typing. Prednisone may be used in individuals with PCH and severe anemia.
- ^ Ogose T, Wakata Y, Kaneko M, Shinahara K, Takechi T, Kotani H (October 2007). "A case of recurrent paroxysmal cold hemoglobinuria with the different temperature thresholds of Donath-Landsteiner antibodies". J. Pediatr. Hematol. Oncol. 29 (10): 716–9. doi:10.1097/MPH.0b013e31814d6845. PMID 17921855. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?an=00043426-200710000-00009.
- ^ Sokol RJ, Booker DJ, Stamps R (1999). "Erythropoiesis: Paroxysmal Cold Haemoglobinuria: A Clinico-Pathological Study of Patients with a Positive Donath-Landsteiner Test". Hematology 4 (2): 137–164. PMID 11399560.
Pathology: hematology · hematologic diseases of RBCs and megakaryocytes / MEP (D50-69,74, 280-287) Red
Wikimedia Foundation. 2010.
Look at other dictionaries:
paroxysmal cold hemoglobinuria — an autoimmune or postviral disease marked by episodes of hemoglobinemia and hemoglobinuria after exposure to cold, caused by complement dependent hemolysis due to IgG antibody (Donath Landsteiner antibody) directed against the P blood group… … Medical dictionary
Paroxysmal hemoglobinuria — can refer to: * Paroxysmal nocturnal hemoglobinuria * Paroxysmal cold hemoglobinuria … Wikipedia
cold agglutinin — n any of several agglutinins sometimes present in the blood (as that of many patients with primary atypical pneumonia) that at low temperatures agglutinate compatible as well as incompatible red blood cells, including the patient s own called… … Medical dictionary
Cold antibody hemolytic anemia — can refer to: Cold agglutinin disease Paroxysmal cold hemoglobinuria This disambiguation page lists articles associated with the same title. If an internal link led yo … Wikipedia
Hemoglobinuria — The presence of free hemoglobin in the urine, an abnormal finding, that may make the urine look dark. Hemoglobin is the protein in the red blood cells which carries oxygen from the lungs to the tissues of the body and returns carbon dioxide from… … Medical dictionary
PCH — paroxysmal cold hemoglobinuria; patient care hours; polycyclic hydrocarbon … Medical dictionary
PCH — • paroxysmal cold hemoglobinuria; • patient care hours; • polycyclic hydrocarbon … Dictionary of medical acronyms & abbreviations
Hemolytic anemia — ICD9|283, ICD9|773 ICDO = OMIM = MedlinePlus = 000571 eMedicineSubj = med eMedicineTopic = 979 MeshID = D000743Hemolytic anemia is anemia due to hemolysis, the abnormal breakdown of red blood cells (RBCs) either in the blood vessels… … Wikipedia
Hematologic disease — Infobox Disease Name = PAGENAME Caption = DiseasesDB = ICD10 = ICD9 = ICDO = OMIM = MedlinePlus = eMedicineSubj = eMedicineTopic = MeshID = D006402 Hematologic diseases are disorders which primarily affect the blood.Classification of hematologic… … Wikipedia
List of diseases (P) — A list of diseases in the English Wikipedia.DiseasesTOC PaPac Pal* Pachydermoperiostosis * Pachygyria * Pachyonychia congenita Jackson Lawler type * Pacman dysplasia * Paes Whelan Modi syndrome * Paget disease extramammary * Paget disease… … Wikipedia