Conditions associated with extreme hyperferritinaemia (>3000 μg/L) in adults

The clinical significance of extreme hyperferritinaemia has come under scrutiny with the increasing recognition of haemophagocytic lymphohistiocytosis (HLH) in adults. Most studies of hyperferritinaemia have focused on serum ferritin greater than 1000 μg/L, often in ambulatory patients. The conditions associated with more extreme hyperferritinaemia are poorly understood.

To examine conditions associated with extreme hyperferritinaemia greater than 3000 μg/L in acutely ill adults at a quaternary care hospital.

Patients with serum ferritin greater than 3000 μg/L at Vancouver General Hospital between 1 August 2011 and 1 August 2012 were identified. Those over 18 years of age and with clinical data available were included in the study.

Eighty-three subjects were identified. Twenty-one cases (25%) were due to transfusional iron overload, 16 (19%) due to liver disease and 15 (18%) due to mixed factors. Haemophagocytic lymphohistiocytosis (HLH) was diagnosed in six of 83 patients (7%) with ferritin greater than 3000 μg/L, but six of eight patients (75%) with ferritin greater than 20 000 μg/L.

Extreme hyperferritinaemia greater than 3000 μg/L is uncommon in adult patients. The highest serum ferritin values are seen in HLH, but the differential diagnosis for serum ferritin greater than 3000 μg/L remains broad with iron overload and liver disease being the most common causes.