Paroxysmal cold haemoglobinuria

Description

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.

Index terms

Paroxysmal cold haemoglobinuria
Donath-Landsteiner syndrome

Exclusions from above levels

04 – Diseases of the immune system
05 – Endocrine, nutritional or metabolic diseases
18 – Complications of pregnancy, childbirth or the puerperium

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19 – Certain conditions originating in the perinatal period
20 – Congenital malformations, deformations or chromosomal abnormalities
22 – Injury, poisoning or certain other consequences of external causes
http://id.who.int/icd/entity/1000704511 – Human immunodeficiency virus disease
JB64.1 – Other diseases of the blood or blood-forming organs or certain disorders involving the immune mechanism complicating pregnancy, childbirth or the puerperium

Sibling codes

3A20.0 – Autoimmune haemolytic anaemia, warm type
3A20.1 – Autoimmune haemolytic anaemia, cold type
3A20.2 – Autoimmune haemolytic anaemia, mixed type, cold and warm
3A20.4 – Alloimmune haemolytic anaemia
3A20.5 – Evans syndrome
3A20.Y – Other specified acquired haemolytic anaemia, immune
3A20.Z – Acquired haemolytic anaemia, immune, unspecified

Foundation entity

Paroxysmal cold haemoglobinuria – http://id.who.int/icd/entity/715111132

Part of: 3A20: Acquired haemolytic anaemia, immune › 03: Diseases of the blood or blood-forming organs