Paroxysmal nocturnal hemoglobinuria

Paroxysmal noctuRNAl hemoglobinuria (PNH) is a chronic intravascular hemolysis caused by acquired red blood cell membrane defects. It often worsens during sleep and can be accompanied by paroxysmal hemoglobinuria and pancytopenia. Although the disease is rare, the incidence has increased in recent years. North China is more than the South, and more than half of them occur in young adults aged 20-40, and some are younger than 10 and older than 70. More men than women. PNH has always been classified as hemolytic disease, but in addition to anemia is often accompanied by a decrease in neutrophils and / or platelets, and the molecular lesions of PNH involve various blood cells, so some authors in recent years regard PNH as a hematopoietic stem cell disease. The main cause of death is infection at home and vascular embolism abroad.