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

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 -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 mg/dL, direct bilirubin, 13.3mg/dL, alkaline phosphatase, 223IU/L and prothrombin time, 10.5 sec (INR 0.76; 100%). His iron status was serum iron 246microgram/dL, total iron binding capacity, 277microgram/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.