Journal of Clinical Oncology, Vol 21, Issue 22
(November), 2003: 4251-4252
© 2003 American Society for Clinical Oncology
Uncommon Hematologic Malignancies
CASE 3. PAROTID SWELLING DURING TREATMENT FOR TRANSFORMED MYCOSIS FUNGOIDES
Brian R. Bird,
Peter A. Daly
St. James’s Hospital and Trinity College, Dublin, Ireland
A 49-year-old male with stage IV mycosis fungoides which transformed to a large T-cell lymphoma (Fig 1 ) did not respond to treatment with alemtuzumab (anti-CD52, campath-1H) and proceeded to methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisolone, and bleomycin (MACOP-B) chemotherapy. He developed bilateral parotid enlargement and facial lipodystrophy (Fig 2) as well as insulin-resistant diabetes mellitus and severe hypertriglyceridemia (Fig 3). A magnetic resonance image of his parotids (Fig 4) showed enhanced T1 signal which disappeared on fat suppression. Fine needle aspiration cytology showed normal parotid cells. The parotid swelling resolved with aggressive treatment of his dyslipidemia with insulin, oral hypoglycemics, and fenofibrate.
Pseudo-Sjogren’s syndrome, where parotid enlargement and xerostomia are associated with hyperlipidemia, has previously been described.1 Some cases resolve on treatment of the hyperlipidemia. Our patient had a mild, pre-existing hypertriglyceridemia, which was exacerbated by the high-dose steroids he received with MACOP-B (prednisolone 75 mg/d x 10 weeks, tapered off over 2 weeks). Facial lipodystrophy is caused by complement activation2 and immune destruction of facial fat. Alemtuzamab, a monoclonal antibody to CD52, is known to cause complement activation. Lipodystrophy in HIV-positive patients treated with protease inhibitors is associated with a high cortisol:dehydroepiandrosterone ratio and high interferon alfa levels.3
This case of iatrogenic pseudo-Sjogren’s syndrome highlights the importance of considering fatty infiltration as a cause of parotid enlargement. We believe that the cytokine production associated with bulky, high-grade T-cell lymphoma may also have contributed to his lipodystrophy.
AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST
The authors indicated no potential conflicts of interest.
REFERENCES
1. Sheikh JS, Sharma M, Kunath A, et al: Reversible parotid enlargement and pseudo-Sjogren’s syndrome secondary to hypertriglyceridemia. J Rheumatol 23:1288–1291, 1996[Medline]
2. Sissons JG, West RJ, Fallows J, et al: The complement abnormalities of lipodystrophy. N Engl J Med 294:461–465, 1976[Abstract]
3. Christeff N, De Truchis P, Melchior JC, et al; Longitudinal evolution of HIV-1-associated lipodystrophy is correlated to serum cortisol:DHEA ratio and IFN-alpha. Eur J Clin Invest 32:775–784, 2002[Medline]
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