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The Korean Journal of Pathology 1982;16(3): 470-475.
발작성 야간 혈색소뇨증 3예
Paroxysmal Nocturnal Hemoglobinuria -3 Cases-
Three cases of paroxysmal nocturnal hemoglobinuria(PNH) were investigated clinically and in Laboratory. PNH was chronic hemolytic anemia characterized by an abnormal sensitivity of blood cells to the lytic action of serum component. The results were summarized as below; 1) All three were male and age distribution was between 28 to 47. 2) The chief compaints were anemic symptoms, hemolytic manifestation, and hemorrhage. 3) The diagnosis made before PNH were aplastic anemia in 2 cases and iron deficiency anemia in 1. 4) Hematologic data were revealed as; Anemia (Hgb. below 12gm%), Reticulocytosis (over 3%), Leukopenia (below 6.000/㎣), Thrombocytopenia (below 20,000/㎣). 5) The urinalysis were showed hemoglobinuria in all cases and hemosiderinuria in 2 cases. 6) Ham's test were positive in all cases and sucrose hemolysis test were positive in 2 cases. 7) Increased cellurarity with erythroid hyperplasia and decreased iron storage were disclosed in bone marrow smear. 8) Two patients were improved with clinical management and one was expired.
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