Abstract
Paroxysmal nocturnal hemoglobinuria (PNH) is a disease characterized by hemoglobinuria caused by intravascular hemolysis and marked hemosiderosis in the tubular epithelium, and frequently results in renal failure. We reported an autopsy case of PNH, 57-year-old woman, having thrombosis of femoral vein and cavitated pulmonary tuberculosis with hematogenous generalizarion. The kidneys showed marked hemosiderosis in the proximal tubular epithelium and formation of small abscesses. Scarring bands with destruction of a few nephrons in the cortex, however, may be brought about by renal vasoconstriction due to repetition and prolongation of hemolytic crisis leading to chronic renal dysfunction. Proliferation of the lacis cells in the juxtaglomerular apparatus caused by chronic anemia was suspected as the probable cause for the production of erythropoietin and resultant erythroblastic formation of the bone marrow.
