Hemoglobinuria of this type is diagnosed in young people of both sexes at the age of 20 to 40 years, less often in elderly patients. This hemolytic symptom is characterized by a permanent hemolysis of erythrocytes, which manifests paroxysmally (paroxysmally), is accompanied by thrombosis and leads to bone marrow hypoplasia. Paroxysmal nocturnal hemoglobinuria is a form of acquired anemia, which is extremely rare. Paroxysmal hemoglobinuria was first described by the Italian physician Marciafawa and his two colleagues - Mikeli and Strijbing, since this rare syndrome has become known in various countries as variable - nocturnal hemoglobinuria, paroxysmal nocturnal hemoglobinuria of Marietaafa-Mikeli and Striubing-Marietaafa disease. Paroxysmal nocturnal hemoglobinuria of Marietaafa-Mikeli Hemoglobinuria, the main signs and symptoms.Cold paroxysmal hemoglobinuria is the rarest form of autoimmune hemolysis.Marching hemoglobinuria, which is called "soldier" disease, since mechanical hemolysis of red blood cells is associated with an excessive, intense strain on the feet. Toxic hemoglobinuria is a pathological mass hemolysis that develops as a result of severe poisoning with chemical or natural poisonous substances (sulfonamides, fungi, bites of poisonous animals and insects).Paroxysmal nocturnal hemoglobinuria of Marietaafa-Mikeli (or Strubbing-Marietaafa disease) is a acquired hemolytic pathology in which a permanent intravascular decay of defective erythrocytes occurs.Such a volume of the blood pigment can not be adequately processed by a macrophage system (RES) and hemoglobin begins to enter the urine. Hemoglobinuria is characterized by a significant excess of all permissible standards, when the hemoglobin level reaches 200%. An increase in hemoglobin to 20-25% may indicate a congenital disorder of the structure of the protein (hemoglobinopathy) - beta-thalassemia, sickle cell anemia. In a healthy state, the blood plasma can also contain a small amount of hemoglobin - no more than 5% of the total plasma volume. The presence of hemoglobin in the urine is a direct evidence of the intense disintegration of red blood cells, which can be caused by both an internal disease (influenza, pneumonia, acute infection) and an external factor - hypothermia, excessive physical exertion, trauma, intoxication. Hemoglobinuria is one of the forms of hemolytic anemia (hemoglobinemia), more precisely a symptom of the pathological intravascular damage of red blood cells (erythrocytes), when hemoglobin is massively released into the circulatory environment and urine.
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