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International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01
Paroxysmal cold hemoglobinuria is a very rare subtype of autoimmune haemolytic anaemia (AIHA), caused by the presence of cold-reacting autoantibodies in the blood and characterised by the sudden presence of hemoglobinuria, typically after exposure to cold temperatures. PCH is thought to account for at most 2-10% of cases of AIHA.