secondary hemochromatosis in sickle cell disease

What is it about?

Secondary hemochromatosis remains an even 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 ten units of packed red blood cells. In our case, despite prophylactic chelation and phlebotomy, our asymptomatic patient developed significant hepatocellular iron deposition with fibrosis leading to mortality within a year.

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Why is it important?

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 one year after diagnosis of secondary hemochromatosis.