paroxysmal cold haemoglobinuria

Last reviewed 08/2021

Paroxysmal cold haemoglobinuria is a rare disease associated with intravascular haemolysis and haemoglobinuria following exposure to cold.

It is due to a specific antibody - the Donath-Landsteiner antibody - which is unusual in being an IgG antibody which reacts best at temperatures below 37 degrees centrigrade.

Traditionally, paroxysmal cold haemoglobinuria has been associated with congenital syphilis in which it follows a chronic, relapsing disease.

More commonly, it is a now an acute, transient condition which follows viral infections such as measles, mumps, glandular fever, or chicken pox, or is idiopathic.

The antibody sticks to the red blood cells in the cold and causes a complement-mediated lysis on rewarming.