불가피하게 반복적인 수혈을 받는 환자에서 수혈과 관련하여 이차성 혈색소 침착증이 발생할 수 있는데, 본 저자들은 재생불량성 빈혈로 10년간 약 400pints의 수혈을 받은 환자에서 피부 색소 침착, 저성선자극호르몬에 의한 성선기능 저하, 당뇨병, 간기능 장애, 확장성 심근병증에 의한 울혈성 심부전 등 여러 가지 심각한 합병증을 동반한 이차성 혈색소 침착증 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
불가피하게 반복적인 수혈을 받는 환자에서 수혈과 관련하여 이차성 혈색소 침착증이 발생할 수 있는데, 본 저자들은 재생불량성 빈혈로 10년간 약 400pints의 수혈을 받은 환자에서 피부 색소 침착, 저성선자극호르몬에 의한 성선기능 저하, 당뇨병, 간기능 장애, 확장성 심근병증에 의한 울혈성 심부전 등 여러 가지 심각한 합병증을 동반한 이차성 혈색소 침착증 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
We report a case of transfusion-associated hemochromatosis in a 38-year-old female who had severe aplastic anemia. She had undergone conservative treatment consisting of multiple transfusions because she had no suitable HLA-matched bone marrow donor. She had received 400 units of red blood cells (RB...
We report a case of transfusion-associated hemochromatosis in a 38-year-old female who had severe aplastic anemia. She had undergone conservative treatment consisting of multiple transfusions because she had no suitable HLA-matched bone marrow donor. She had received 400 units of red blood cells (RBCs) over 10 years. The transferrin saturation and serum ferritin were increased to 88.4% and 29,905 ng/mL, respectively. Abdominal computed tomography (CT) showed increased densities in the liver and myocardium, suggesting iron deposits in those organs. In general, transfusional iron overload results in primarily reticuloendothelial iron accumulation, which is considered to be less harmful than parenchymal iron accumulation. Nevertheless, our patient had many striking complications associated with hemochromatosis, such as skin pigmentation, hypogonadotropin hypogonadism, insulin-requiring diabetes mellitus, impaired liver function, and left ventricular dysfunction.
We report a case of transfusion-associated hemochromatosis in a 38-year-old female who had severe aplastic anemia. She had undergone conservative treatment consisting of multiple transfusions because she had no suitable HLA-matched bone marrow donor. She had received 400 units of red blood cells (RBCs) over 10 years. The transferrin saturation and serum ferritin were increased to 88.4% and 29,905 ng/mL, respectively. Abdominal computed tomography (CT) showed increased densities in the liver and myocardium, suggesting iron deposits in those organs. In general, transfusional iron overload results in primarily reticuloendothelial iron accumulation, which is considered to be less harmful than parenchymal iron accumulation. Nevertheless, our patient had many striking complications associated with hemochromatosis, such as skin pigmentation, hypogonadotropin hypogonadism, insulin-requiring diabetes mellitus, impaired liver function, and left ventricular dysfunction.
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