![]() ![]() Without this shield, they are vulnerable to attack and destruction by a part of your immune system known as the complement system. The abnormal red blood cells that develop in PNH - known as PNH red blood cells to differentiate them from your healthy red blood cells - lack a protective shield of proteins. Weakened bone marrow may occur due to aplastic anemia or another bone marrow disease, which may be mild or undiagnosed, according to the Aplastic Anemia and MDS International Foundation (AAMDSIF). Many experts believe that the mutation that causes PNH also happens in healthy people, and only leads to disease - with abnormal stem cells multiplying quickly - when people have underlying bone marrow dysfunction. This abnormal stem cell then divides over and over to produce many abnormal stem cells, which go on to become abnormal red blood cells. In PNH, a mutation occurs in one of these stem cells, in what’s known as the PIGA gene. These cells eventually grow and mature into red blood cells, white blood cells, and platelets. The PNH disease process starts in your bone marrow, which produces cells known as hematopoietic stem cells. This mutation typically occurs in adults and is not inherited from your parents or passed on to your children. ![]() PNH develops when a genetic mutation leads to the production of defective red blood cells. Many people with PNH don’t have discolored urine, which is just one of many possible symptoms of the condition. “Paroxysmal” means occurring in episodes, “nocturnal” means at night, and “hemoglobinuria” refers to the presence of the protein hemoglobin in your urine, which can turn it dark or reddish. PNH received its name long before scientists understood many aspects of the condition. But people with PNH still need to look out for dangerous complications, particularly blood clots that can affect many different areas of the body. Newer treatments for PNH have dramatically changed the outlook for people with the condition, who can now typically expect to live as long as people without PNH, according to the Cleveland Clinic. PNH is diagnosed based on your symptoms and a variety of blood tests, which can identify the consequences of red blood cell destruction as well as the actual abnormal red blood cells. It develops after birth and is not passed from parents to their children. PNH develops because of a genetic mutation that affects your body’s production of blood cells. This premature destruction of red blood cells can lead to symptoms including fatigue, headaches, and difficulty breathing. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder in which your body produces defective red blood cells that break apart in your bloodstream. ![]()
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |