Korean J Hematol 1997; 32(2):

Published online June 30, 1997

© The Korean Society of Hematology

동종 골수이식을 시행받은 중증 재생불량성 빈혈 환자에서 발생한 이차성 혈색소증 1례

김현수, 이석, 이상학, 정소영, 민유홍, 한지숙, 고윤웅

연세대학교 의과대학 내과학교실

A Case of Secondary Hemochromatosis after Allogeneic Bone Marrow Transplantation in Severe Aplastic Anemia

Hyun Soo Kim, Seok Lee, Sang Hak Lee, So Young Chong, Yoo Hong Min, Jee Sook Hahn, Yun Woong Ko

Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea

Abstract

Increasing number of successful bone marrow transplantation(BMT) for hematologic malignancies has focused attention on possible long-term consequences of the procedure. Liver disease after day 100 is usually due to chronic graft-versus-host disease, chronic HBV or HCV infection, drug, or other virus induced hepatitis. Iron overload is also an important cause of liver dysfunction after BMT, especially in multitransfused patients.
We observed a male patient diagnosed with secondary hemochromatosis after allogeneic BMT. His total red cell support had been only 14 units. He was admitted to
hospital due to jaundice and generalized weakness for two weeks. Previously he was diagnosed with hepatitis C and treated with α-interferon for six months, then his
serum became negative for anti-HCV and HCV RNA. On admission, studies for evaluation of liver function showed total protein, 6.4g/dL, albumin, 4.2g/dL, AST, 242IU/L, ALT, 144IU/L, total bilirubin, 15.6㎎/dL, direct bilirubin, 13.3㎎/dL, alkaline phosphatase, 223IU/L and prothrombin time, 10.5 sec(INR 0.76; 100%). His iron status
was serum iron 246 ㎍/dL, total iron binding capacity, 277 ㎍/dL, transferrin saturation, 88.8% and serum ferritin, 8,042ng/mL, and the liver biopsy showed extensive amounts of hemosiderin deposits in hepatocytes. After using deferoxamine to reduce iron overload, his liver function and iron status were substantially improved.
Thus the long-term follow-up of BMT patients should include analysis of HCV and iron status. This may prevent the development of clinically significant chronic liver
disease. The cause of iron overload in this patient may not simply due to transfusional overload and therefore further tests of intestinal iron absorption are warranted.

Keywords Secondary hemochromatosis; Aplastic anemia; Deferoxamine; Bone marrow transplantation;

Article

Korean J Hematol 1997; 32(2): 297-305

Published online June 30, 1997

Copyright © The Korean Society of Hematology.

동종 골수이식을 시행받은 중증 재생불량성 빈혈 환자에서 발생한 이차성 혈색소증 1례

김현수, 이석, 이상학, 정소영, 민유홍, 한지숙, 고윤웅

연세대학교 의과대학 내과학교실

A Case of Secondary Hemochromatosis after Allogeneic Bone Marrow Transplantation in Severe Aplastic Anemia

Hyun Soo Kim, Seok Lee, Sang Hak Lee, So Young Chong, Yoo Hong Min, Jee Sook Hahn, Yun Woong Ko

Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea

Abstract

Increasing number of successful bone marrow transplantation(BMT) for hematologic malignancies has focused attention on possible long-term consequences of the procedure. Liver disease after day 100 is usually due to chronic graft-versus-host disease, chronic HBV or HCV infection, drug, or other virus induced hepatitis. Iron overload is also an important cause of liver dysfunction after BMT, especially in multitransfused patients.
We observed a male patient diagnosed with secondary hemochromatosis after allogeneic BMT. His total red cell support had been only 14 units. He was admitted to
hospital due to jaundice and generalized weakness for two weeks. Previously he was diagnosed with hepatitis C and treated with α-interferon for six months, then his
serum became negative for anti-HCV and HCV RNA. On admission, studies for evaluation of liver function showed total protein, 6.4g/dL, albumin, 4.2g/dL, AST, 242IU/L, ALT, 144IU/L, total bilirubin, 15.6㎎/dL, direct bilirubin, 13.3㎎/dL, alkaline phosphatase, 223IU/L and prothrombin time, 10.5 sec(INR 0.76; 100%). His iron status
was serum iron 246 ㎍/dL, total iron binding capacity, 277 ㎍/dL, transferrin saturation, 88.8% and serum ferritin, 8,042ng/mL, and the liver biopsy showed extensive amounts of hemosiderin deposits in hepatocytes. After using deferoxamine to reduce iron overload, his liver function and iron status were substantially improved.
Thus the long-term follow-up of BMT patients should include analysis of HCV and iron status. This may prevent the development of clinically significant chronic liver
disease. The cause of iron overload in this patient may not simply due to transfusional overload and therefore further tests of intestinal iron absorption are warranted.

Keywords: Secondary hemochromatosis, Aplastic anemia, Deferoxamine, Bone marrow transplantation,

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