while blood transfusions are commonly used for prophylaxis and treatment for acute chest syndromes and strokes in sickle cell patients , accumulation of excess iron resulting in secondary hemochromatosis remains a rare disease . chelation is the mainstay for preventing and treating iron overload to deter potential end - organ damages ; it is rare when therapy fails . a 52-year - old african american woman with chronic anemia secondary to sickle cell anemia and history of multiple blood transfusions presented with elevated serum ferritin ( 8000 ng / ml ) and bilirubin ( 16.8 mg / dl ) . she had no previous personal or family history of liver disease . a magnetic resonance cholangiopancreatography ( mrcp ) and a liver biopsy confirmed the secondary hemochromatosis with marked fibrosis and 4 + iron deposits , but since she was therapeutically on deferasirox , her treatment regimen involved only closer monitoring . her hemochromatosis led to readmission within a year for rapid progression of cardiac and hepatic failure . since chronically transfused sickle cell patients are at a significantly higher risk of mortality due to the secondary hemochromatosis and end - stage organ damage , knowledge of prophylactic iron chelation is important . minimizing unnecessary transfusions should be strongly emphasized to reduce the sequelae as iron burden remains a threat . the effectiveness of iron - chelating therapy is best monitored via periodic magnetic resonance imaging , liver transaminases , bilirubin , creatinine , ferritin , and cardiac function tests . despite the prophylactic treatment and quarterly blood work , in this case the initial presentation did not correlate with the severity of end - stage liver failure . the damage was not discovered until proven by liver biopsy and mrcp , too late to deter the sequelae and the mortality exactly 1 year after diagnosis of the secondary hemochromatosis . secondary hemochromatosis remains a rare , underdiagnosed disorder when presented atypically with  bronze diabetes  and cirrhosis in sickle cell patients . blood transfusions serve as both prophylaxis and treatment for strokes and acute chest syndromes.1,2 however , excess transfusional iron accumulates in tissues and forms reactive oxygen species resulting in end - organ damages . chelation is often initiated prophylactically in patients who have received greater than 10 units of packed red blood cells.3 in our case , despite prophylactic chelation and phlebotomy , our asymptomatic patient developed significant hepatocellular iron deposition with fibrosis leading to mortality within a year . a 52-year - old african american woman with chronic anemia due to sickle cell disease , and history of multiple transfusions , presented for routine outpatient blood work and found to have elevated serum ferritin ( 8000 ng / ml ) and bilirubin ( 16.8 mg / dl ) . her past medical history was significant only for cholecystectomy , with no previous personal or family history of liver disease . an magnetic resonance cholangiopancreatography ( mrcp ) ( figure 1 ) was done to rule out an obstruction . to everyone the biopsy later confirmed the presumptive diagnosis of the secondary hemochromatosis with marked fibrosis and 4 + iron deposits ( figure 2 ) . since the patient was therapeutically receiving phlebotomies every 45 months in addition to her iron chelator deferasirox for the last 10 years , her treatment regimen post discharge involved further monitoring every 3 months with imaging and laboratory tests , including ferritin , transferrin , and hepatic panel . laboratory investigations represented significant cirrhosis secondary to hemochromatosis along with evidence of renal , pulmonary , and cardiac failures , severe anemia , and supratherapeutic international normalized ratio resulting in her death within 7 days of admission . long - term transfusion therapy is now a routine , life saving treatment for patients with intractable anemia resulting from sickle cell disease , thalassemia , bone marrow failure , or aggressive treatment of cancer . repeated transfusion leads to rapid iron loading : each unit of blood contains 200250 mg of iron.4 the american association for the study of liver diseases recommends evaluating for hemochromatosis in patients with abnormal iron studies , even in the absence of symptoms , and in all patients with evidence of liver disease.5 they suggest diagnosing hemochromatosis if transferrin saturation is > 45% in combination with elevated ferritin levels . however a previous clinical trial suggested that serum ferritin ( range : 15003000 ng / ml ) is not always accurate to interpret the hepatic iron store concentrations because it does not directly correlate with the degree of cirrhosis.5 the effectiveness of iron - chelating therapy is best monitored via periodic magnetic resonance imaging ( mri ) , liver transaminases , bilirubin , creatinine , ferritin , and cardiac function tests . while this patient presented with an atypical , asymptomatic presentation , her routine labs conveyed only increased ferritin and bilirubin so hepatic failure was not considered . after the liver biopsy , a concern was raised for hemochromatosis even though she was prophylactically on iron chelator . this case raises important questions to consider : does initiating therapy early really help to increase the life expectancy of a patient with the secondary hemochromatosis ? despite chelation therapy , iron burden in chronically transfused patients remains a threat . since her chronic conditions were well - known , would a liver biopsy earlier in the course have indicated the degree of her serious hepatic and cardiac failures ? should routine quarterly mri and echocardiogram be used in asymptomatic patients to prevent some sequelae of end - organ damages ? since chronically transfused sickle cell patients are at significantly higher risk of mortality due to the secondary hemochromatosis and end - organ damages , knowledge of prophylactic iron chelation is of paramount importance.2,3 minimizing unnecessary transfusions should be strongly emphasized early . the effectiveness of iron - chelating therapy may be best monitored via periodic mri , liver transaminases , bilirubin , creatinine , ferritin , and cardiac function tests . in this atypical case , the initial presentation did not correlate with the severity of end - stage liver failure until proven by liver biopsy and mri , which still could not deter the sequelae and the mortality exactly 1 year after the diagnosis of secondary hemochromatosis .