Abstract
Background: Elevated serum ferritin is more commonly due to reactive causes such as infection, hepatic disorders, rheumatologic conditions, and malignancy than true iron overload. Extreme hyperferritinemia (>10,000 ng/mL), on the other hand, should prompt consideration of rare conditions such as adult-onset Still’s disease (AOSD) or hemophagocytic lymphohistiocytosis. This paper aims to present the case of the highest reported extreme hyperferritinemia (actual level 256,000 ng/mL) in a patient eventually diagnosed with adult onset Still’s Disease (AOSD). Case Presentation: A 55-year-old male, Filipino, was admitted due to acute onset fever and shortness of breath. He was initially assessed to have community-acquired pneumonia and a suspect for coronavirus disease 2019 (COVID-19), hence inflammatory markers were requested. Ferritin was notably elevated at 44,255 ng/mL. He eventually tested negative for COVID-19 RT-PCR. He was investigated for other causes of markedly elevated ferritin levels. His complete blood count (CBC) only showed leukocytosis with no peripheral blasts, iron level and liver function tests were normal, HIV immunoassay was negative, ANA was 1:80 speckled with normal complement level, rheumatoid factor negative, and positron emission tomography (PET) scan revealed presence of lymphadenopathies and did not show solid tumors. He was treated for urinary tract infection and pneumonia but still had intermittent fever and increasing ferritin trend, with the highest documented level at 256,000 ng/mL. Fulfilling the Yamaguchi criteria, he was managed as a case of severe AOSD and received tocilizumab. He had lysis of fever and decreasing trend of ferritin levels thereafter, with ferritin level of 34,184 ng/mL three weeks after tocilizumab infusion. He was discharged and improved with prednisone and methotrexate as home medications. Conclusion: To our knowledge, the highest level of extreme hyperferritinemia recorded in literature as of 2016 is 143,931 ng/mL, which was associated with hematologic malignancy. This case documents the highest noted ferritin level of 256,000 ng/mL associated with AOSD. AOSD remains a diagnosis of exclusion due to its nonspecific symptoms and absence of definitive tests. The treatment comprises NSAIDs, steroids, and immunosuppressives; however biological treatments such as tocilizumab can be considered in severe cases.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
More From: Open Journal of Rheumatology and Autoimmune Diseases
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.