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

Background 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. Aims To examine conditions associated with extreme hyperferritinaemia greater than 3000 μg/ L in acutely ill adults at a quaternary care hospital. Methods 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. Results 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. Conclusions 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. [ABSTRACT FROM AUTHOR]