發作性 夜間 血色素尿症 一例 報告
A Case of Paroxysmal Nocturnal Hemoglobinuria
소속 상세정보
최삼임(崔三任)/Sam-Im Choi
유주용(柳柱容)/Joo-Yong Yoo
KMID : 0357919810150040466
Abstract
Paroxysmal nocturnal hemoglouinuria(PNH) is a disorder of the hematopoietic stem
cell that results in the production of a clone of defective red blood cells in circulating
lines.
Clinical and laboratory observations were made in a case of PNH admitted to
Chonnam University Hospital. The patient was 16-year-old female who complained
general weakness and headache.
The peripheral blood revealed anemia with reticulocytosis. Whole blood screening test,
sucrose hemolysis test and Ham's test were positive. Serum bilirubin was moderately
increased. On urinalysis, marked hemoglotinuria and hemosiderinuria were shown. The
bone marrow aspiration revealed decreased cellularity with marked erythroid hyperplasia.
She was treated with transfusion of packed red cells and discharged with partial
alleviation of clinical manifestation.
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