Objectives To assess the efficacy of therapy with rituximab (RTX) and cyclophosphamide (CPH) in patients with parotid gland MALT lymphoma associated with primary Sjogren’s syndrome (pSS)
Methods 35 female patients with pSS, as determined by the revised American-European criteria, and parotid glands MALT lymphomas were treated with either RTX (13 patients) or CPH (7 patients). RTX in combination with CPH was used in 15 patients. RTX was administered at 375 mg/m2 weekly for 4-5 weeks. RTX in combination with 1000 mg CHP or 1000 mg CHP monotherapy were given every two weeks for a period 8-10 weeks.
In all patients an incision biopsy of the parotid gland was performed before treatment.
MALT lymphomas’s diagnosis was confirmed by histological and immunohistopathological analysis of parotid glands samples. Polymerase chain reaction (PCR) was used for the detection of monoclonal immunoglobulin heavy chain (IgH) gene rearrangements in parotid glands. MALT lymphoma was also localized in the lacrimal glands (n=4), submandibular glands (n=3), cervical lymph nodes (n=4).
Results Complete clinical response of MALT lymphoma was achieved in 11 out 13 patients (85%) in the RTX group, in 4 out 7 patients (57%) in the CPH group, in all 15 patients (100%) in the RTX with CPH group. The other patients had partial clinical response. An repeated incision biopsy of the parotid gland at month 3 after treatment was performed in 10 patients with RTX monotherapy and in 13 patients with combined therapy RTX and CPH. Complete disappearance of histological and immunohistopathological pattern of MALT lymphoma was shown in 70% of the RTX treated patients and in 85% of the patients with combined therapy. In parotid glands B-cell monoclonality (IgH-PCR) disappeared in 1 out 5 cases (20%) after RTX monotherapy and in 4 out 7 cases (57%) after RTX with CPH therapy.
Conclusions RTX monotherapy and RTX in combination with CPH are effective treatment for parotid gland MALT lymphomas in pSS.
Disclosure of Interest None Declared
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