Paroxysmal Nocturnal Hemoglobinuria Research Paper

Paroxysmal nocturnal hemoglobinuria, also known as (PNH), is a rare disorder in which the red blood cells we all carry in our bodies break apart prematurely. When this happens it is considered an acquired hematopoietic stem cell disorder. These stem sells are created in the bone marrow. These cells grow and develop into white blood cells (WBCs), platelets and red blood cells (RBCs). Hematopoietic stem cells in people with PNH are defective and produce defective blood cells. The defective red blood cells of paroxysmal nocturnal hemoglobinuria are susceptible to premature destruction by the complement system; a part of a person’s immune system that consists of a group of proteins that work together to destroy foreign invaders such as viruses and bacteria. These proteins normally protect red blood cells from destruction by the complement. People with PNH are missing two important complement-regulating proteins. Due to this missing protein, the red blood cells are prematurely destroyed, which leads to hemolytic anemia. Hemolysis by the complement system leads to episodes of hemoglobin in the urine, which would then be considered hemoglobinuria. Hemoglobin is an iron-containing …show more content…
It happens all the time and at a very fast pace that could lead to some serious heath issues. In paroxysmal nocturnal hemoglobinuria hemolysis happens all the time. Ongoing hemolysis happens constantly because of the red blood cells are missing a protective protein. The signs and symptoms are very much like other diseases. Everyone will have different reactions to this disease. Paroxysmal nocturnal hemoglobinuria is progressive to the point were it will get worse over a short period of time. All patients have had evidence of intravascular hemolysis; many have isolated or multiple cytopenias and a history of