Fatal Blastoid Variant Mantle Cell Lymphoma in a Patient with Sj？gren's Syndrome

Primary Sj？gren's syndrome (pSS) is an autoimmune disorder of the exocrine glands presenting with progressive ocular and oral dryness, parotid gland enlargement, and often with extraglandular manifestations. In this group of patients the risk of development of non-Hodgkin's lymphoma is 16-fold compared to healthy population, mainly of the MALT lymphoma type. This case reports a 52-year-old woman with pSS developing a progressively growing mass at face and neck compatible with blastoid variant mantle cell lymphoma and a fatal outcome. 1. Introduction Primary Sj？gren's syndrome (pSS) is an autoimmune disease characterized by lymphocytic infiltration of exocrine glands together with polyclonal B-cell activation [1]. Patients have an increased risk of up to 6% per year for developing non-Hodgkin's (NHL) B-cell lymphomas, including mucosa-associated lymphoid tissue (MALT) lymphomas in 70% of cases, preferentially extranodal marginal zone (MZ), located mainly in the major salivary glands [2]. This complication is associated with excess in the overall mortality rate in pSS patients. The association between pSS and lymphoma has been recognized since 1964 [3]; thus, attention is drawn to regular control of patients with this disease. Several clinical and serological markers have been reported to predict the development of NHL in pSS in different series. Among these parameters, patients with pSS and splenomegaly, persistent enlargement of parotid glands, lymphadenopathy, palpable purpura, cryoglobulinemia, low levels of C4, neutropenia, or lymphocytopenia have more than 5-fold increased risk of NHL compared to patients without risk factors. This case reports a 52-year-old woman with pSS developing a progressively growing mass at face and neck compatible with blastoid variant mantle cell lymphoma and a fatal outcome. 2. Case Report A 52-year-old woman with a previous history of eight years of pSS (sicca syndrome, positive antinuclear antibodies, rheumatoid factor, and anti-Ro) was admitted to our hospital with the presence of a progressively growing mass on face and neck leading to swallowing and breathing difficulty. She reported chronic inflammatory arthralgias, xerostomia, and xerophthalmia. At physical examination on admission xerostomia and bilateral increase in size of parotid with dysphagia were observed (Figure 1). Cardiopulmonary, neurological, and osteoarticular systems were normal. On the biological analysis, low C4 and low C3 (65？mg/dL) levels were evidenced. High lactic dehydrogenase 777？U/L was also documented. Total leukocyte count was normal