Cold paroxysmal hemoglobinuria (CPH) is a rare autoimmune disorder characterized by the destruction of red blood cells (hemolysis) triggered by exposure to cold temperatures. It is a type of hemolytic anemia that occurs episodically, and its symptoms typically manifest when an affected individual is exposed to cold environments.
During a cold paroxysmal hemoglobinuria episode, the immune system mistakenly launches an attack on red blood cells, leading to their destruction and subsequent release of hemoglobin into the bloodstream. This can result in symptoms such as fatigue, dark urine that may contain visible blood (hemoglobinuria), pale skin (pallor), and jaundice. The severity and duration of these episodes can vary among individuals.
The underlying cause of cold paroxysmal hemoglobinuria is the presence of an abnormal protein called Donath-Landsteiner antibody. This antibody binds to red blood cells when exposed to cold temperatures, activating the immune system to initiate hemolysis.
Diagnosis of cold paroxysmal hemoglobinuria involves a combination of physical examination, medical history review, laboratory tests (such as a complete blood count and Coombs test), and specialized tests to detect the presence of Donath-Landsteiner antibody.
Treatment for cold paroxysmal hemoglobinuria usually involves keeping the affected individual warm to prevent episodes, as well as managing symptoms during an episode with rest and, in severe cases, blood transfusions. In some cases, immunosuppressive medication may be necessary to manage the underlying autoimmune response.
