A variety of malignancies can present as rheumatic syndromes. Our aim was to describe a case of primary hepatic anaplastic large-cell lymphoma (ALCL) presenting as a rheumatic syndrome. We also describe cytokine production by the tumor.
A patient with ALCL presenting with arthralgia and systemic inflammation is described. Cytokine production by the resected tumor and adjacent normal liver was assayed by enzyme-linked immunosorbent assay following in vitro culture. Medline database search of the English literature between 1976 and August 2006 was performed.
A 44-year-old man was admitted with generalized arthralgias, fatigue, weight loss, and night sweats. Despite a high clinical suspicion of underlying malignancy, repeated radiologically guided biopsies of bone and liver abnormalities failed to demonstrate any malignant cells. The patient improved dramatically on empirical corticosteroids but symptoms recurred on attempting to reduce the steroid dosage. He ultimately underwent partial hepatectomy for an enlarging liver lesion, which was demonstrated to be primary hepatic ALCL on immunohistochemistry. There are a few reports of ALCL presenting as bone lesions, but to our knowledge this is the first report of hepatic ALCL presenting with a rheumatic syndrome. The clinical and biochemical inflammation resolved following resection of the tumor but returned on tumor recurrence. The tumor produced large quantities of the proinflammatory cytokines interleukin-6 (IL-6) and IL-8 but did not produce tumor necrosis factor-alpha (TNF-alpha), IL-1, or IL-4.
Repeat biopsies may be required to diagnose underlying tumor. The ALCL tissue in our patient produced large quantities of the IL-6, which we believe was associated with the patient's systemic inflammation.