paroxysmal nocturnal hemoglobinuria, pnh…

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blood cells, pnh, paroxysmal nocturnal hemoglobinuria, rare, acquired disorder of blood cells in which a specific molecule on the surface, the cells leads to premature destruction, the cells, destruction is intermittent, paroxysmal, pnh, a defect, in the formation of a protein anchor, gpi, the lack of certain surface proteins, a number of surface proteins, unable to remain tethered, cell surface, the proteins cd55, decay accelerating factor, daf, cd59, membrane inhibitor of complement lysis, mirl, the loss of these cell-surface proteins is an enhanced sensitivity to complement-mediated cell destruction, complement, substance produced by the immune system, affect people of any age, red blood cell, white blood cell, platelet counts low, urine intermittently red, brown, signifying the breakdown of red blood cells, release of hemoglobin, circulation, urine, blood clots, form, people, arise in relation to aplastic anemia, progress to acute myelogenous leukemia, risk factors, except, prior aplastic anemia, not known, urine bloody, intermittent dark urine, abdominal pain, back pain, headache, shortness of breath, easy bruising, bleeding, in the skin, cbc, low white blood cell count, red blood cell count, platelets, sucrose hemolysis test is positive, ham's, acid hemolysin, test is positive, flow cytometry shows a lack of specific surface proteins, urinalysis shows hemosiderin, hemoglobin, serum hemoglobin results altered, steroids effective in suppressing hemolysis, cell destruction, blood transfusions required, anticoagulation therapy, prevent clot formation, bone marrow transplantation, cure this disease, outcome is variable, most people surviving approximately 10 years following diagnosis, in rare cases, abnormal cells, decrease over time, death, complications of blood clot formation, thrombosis, by infection, bleeding, due to a lack of normal blood cells, blood clots, anemia , acute myelogenous leukemia, iron deficiency anemia, death, find blood in urine, symptoms worsen, not improve, new symptoms develop, no known way, prevent this disorder.