Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder of the red blood cells that causes the red blood cells to break prematurely. This is basically an acquired hematopoietic stem cell disorder. Some hematopoietic stem cells in individuals with PNH are defective and are hence quite susceptible to premature destruction by a person’s own immune system. The symptoms of the condition vary from person to person. While some people may have mild symptoms that remain stable for years, there may be others who may experience life threatening symptoms in a rather short span of time. Approximately 15-30 percent of individuals with PNH develop blood clots, especially in the veins and all patients of PNH have some type of bone marrow dysfunction. Anaemia is another common symptom.
HOW IS PAROXYSMAL NOCTURNAL HEMOGLOBINURIA DIAGNOSED?
A general physician will conduct a thorough clinical evaluation along with a variety of specialized tests. The main test for diagnosis of this condition is flow cytometry, a blood test that can identify PNH cells.
HOW IS PAROXYSMAL NOCTURNAL HEMOGLOBINURIA TREATED?
Treatment is based on the symptoms. Steroids can decrease the severity of hemolytic crises. Medications need to be given to correct anaemia and in some cases, transfusion therapy may be needed.
DID YOU KNOW?
Most people live 10 years or more after the initial diagnosis of Paroxysmal Nocturnal Hemoglobinuria.