Extreme hyperferritinemia is uncommon and can be caused by a spectrum of disorders, some of which overlap.

Patient selection: serum ferritin concentration > 10,000 ng/mL


Differential diagnosis:

(1) chronic transfusion with iron overload

(2) hematologic malignancy

(3) acute or chronic liver disease, including hemochromatosis and alcoholism

(4) hemoglobinopathy

(5) solid malignancy

(6) infection (tuberculosis, HIV, viral hepatitis, babesiosis, other)

(7) macrophage activation syndrome (MAS)

(8) hemophagocytic lymphohistiocytosis (HLH)

(8) Still's disease



• Hemoglobinopathies may require chronic transfusion.

• MAS can be seen in juvenile Still's disease.

• HLH can be seen with infection and hematologic malignancies.

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