ICD-11 classes
03 Diseases of the blood or blood-forming organs
Anaemias or other erythrocyte disorders
Haemolytic anaemias
Acquired haemolytic anaemia
3A20 — Acquired haemolytic anaemia, immune
3A20.3 — Paroxysmal cold haemoglobinuria

ICD-11 3A20.3 — Paroxysmal cold haemoglobinuria

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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.