Lymphoma Associated with Sjögren’s Syndrome

Abstract

To the Editor, A 50-year-old woman visited the outpatient rheumatology department presenting with heel spurs and radiating pain in the legs and the right hip. She had sicca symptoms and morning stiffness lasting for 15 min. Her physical examination was normal other than positive FABERE test of the right hip. Routine biochemical tests and complete blood count were normal. Serological tests were positive for anti-nuclear antibodies (+2) with a speckled pattern, and positive for both anti-SS-A/Ro and anti-SS-B/ La antibodies. The erythrocyte sedimentation rate and C-reactive protein level were normal. The rheumatoid factor was 17.4 IU/mL (reference range: 0-14 IU/mL). The sacroiliac magnetic resonance findings were compatible with trochanteric bursitis. The results of Schirmer’s test were <5 mm for both eyes and a minor salivary gland biopsy showed focal periductal lymphoid aggregate. With these findings, the present case apparently met the criteria for the diagnosis of Sjogren’s Syndrome (SS) [1]. The patient was treated with hydroxychloroquine and artificial tear drops. Eighteen months later, the patient was admitted with painless swelling of the parotid glands. There was generalized peripheral lymphadenopathy in the neck, axillary, and inguinal regions. Excisional biopsies were obtained from the right inguinal lymph node, which revealed a high-grade, B-cell nonHodgkin lymphoma (NHL). The tumor tissue was composed of atypical round cells with pleomorphic vesicular nuclei undergoing frequent mitosis and eosinophilic cytoplasm (Figure 1). Immunohistochemically, neoplastic cells were positive for CD20, and the Ki-67 proliferation index was around 70%. She received 3 cycles of rituximab plus cyclophosphamide, epirubicin, vincristine, and prednisolone. Because of cardiotoxicity, she received 5 cycles of rituximab, cyclophosphamide, and Oncovin. After 2 months, her symptoms were resolved and her CT scan revealed a remarkable decrease in the generalized swelling of the lymph nodes. Five months later, a PET scan showed no findings consistent with recurrence or