Lab Med Online.  2013 Jan;3(1):40-44. 10.3343/lmo.2013.3.1.40.

A Case of Extremely Elevated Ferritin Levels in a Patient with Fever of Unknown Etiology

• Kim H
• Jang YH
• Chung HJ
• Lee W
• Chun S
• Min WK

• KMID: 1845395
• DOI: http://doi.org/10.3343/lmo.2013.3.1.40

Abstract

Fever of unknown etiology that occurs along with highly elevated serum ferritin concentrations (>500 ng/mL) is mostly observed in hematologic malignancies and rheumatic diseases such as systemic lupus erythematosus, temporal arteritis, and adult-onset Still's disease (AOSD), among which AOSD is a type of systemic inflammatory disorder with unknown pathophysiology and has very low incidence. AOSD presents with various nonspecific symptoms and signs such as high spiking fever, joint pain, skin rash, and increased leukocytes. Because AOSD is diagnosed after excluding the possibility of other conditions such as neoplasms, infections, and inflammations, diagnosis and treatment are generally delayed. Patients with AOSD often have high serum ferritin levels than those with other conditions, although the underlying mechanism for this is not clearly understood. In addition, decreased proportion of glycosylated ferritin are observed in most patients with AOSD. Therefore a combination of high serum ferritin and a decreased proportion of glycosylated ferritin seems to be important for the differential diagnosis of AOSD that thus may allow early diagnosis of AOSD. Here we report a case of AOSD diagnosed via extremely high serum ferritin levels and decreased glycosylated ferritin proportion.

Keyword

Adult onset Still's disease; Ferritin; Glycosylated ferritin

MeSH Terms

Arthralgia
Diagnosis, Differential
Early Diagnosis
Exanthema
Ferritins
Fever
Giant Cell Arteritis
Hematologic Neoplasms
Humans
Incidence
Inflammation
Leukocytes
Lupus Erythematosus, Systemic
Rheumatic Diseases
Still's Disease, Adult-Onset
Ferritins

Figure

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  Fig. 1 A markedly increased number of granulocytes and hemophagocytes engulfing neutrophils are noted in the bone marrow aspirate (A, Wright stain, ×1,000) and biopsy specimens (B, H&E stain, ×400). No definite evidence of hemophagocytic lymphohistiocytosis is observed.

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  Fig. 2 Changes in body temperature, ferritin and C-reactive protein levels during the patient's clinical course.

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