Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired defect in the myeloid stem cell lineage and can be seen as a rare, chronic, morbid disorder. Formally known as Marchiafava-Micheli syndrome, it received its current name as a descriptive term for the disease. Individuals afflicted with the disease wake up to dark, “cola” colored urine due to RBC breakdown and release of hemoglobin in the ...

A previously healthy 16-yr-old girl was found to have pancytopenia, low reticulocyte count, a cellular bone marrow, and a negative Coombs test, all coincident with clinical and laboratory evidence of infectious mononucleosis. Symptoms and signs of infectious mononucleosis subsided, but pancytopenia and hemolytic anemia persisted. Sucrose hemolysis and acid hemolysis tests supported a diagnosis ...

casional patients survive for much longer periods of time. Two of the earliest cases identified in the United States were reported from the Johns Hopkins Hospital in 1936 by Hamburger and Bernstein.13 Their paper was the first in the English language in which the disease was specifically recognized. One of their patients, a man, died of uremia in 1949, with kidneys appearing like the terminal s...

Danazol was administered to two patients with paroxysmal nocturnal hemoglobinuria (PNH) with a dramatic effect on the hematological findings. The patients, 31- and 41-year-old females, were initially diagnosed as having aplastic anemia, and were initially treated with anabolic steroid and immunosuppressive therapy, respectively. Sugar water and Ham tests turned positive at the start of danazol ...

Three patients with paroxysmal nocturnal hemoglobinuria accompanied by chronic renal lesions were studied. All the cases had histories of severe hemolytic anemia and repeated hemoglobinuria. The biopsy specimen of the kidney of two patients (Case 1 and Case 2) showed interstitial nephritis. Renal glucosuria, tubular proteinuria, increased urate clearance (Case 2) and reduced tubular reabsorptio...

On June 24, 2023, an Expert Council was held in St. Petersburg, during which leading experts the field of hematology discussed current achievements and answered a number unresolved issues targeted therapy paroxysmal nocturnal hemoglobinuria (APG) order to further improve treatment results Russia. During Council, following aspects APG were considered: • criteria for suboptimal response patients ...