Mantle Cell Lymphoma and Systemic Sclerosis

Systemic sclerosis (SSc) is a rare disease with an increased incidence of cancer, but the ocorrence of Non-Hodgkin lymphomas (NHL) is a very uncommon event. We report a case of A 76-year-old female addmited to the hematology clinic with long-term adenopathies and ocasional gastro-intestinal symptomatology. Progressive SYMMETRICAL swelling of hands was also noticed. Colonoscopy revealed multiple polyps and histopathology was consistent with Mantle-Cell (MCL) NHL. R-CHOP (rituximab, cyclophosphamide, doxorubicine, vincristine and prednisone) regimen was promptly iniated with complete response. Persistent swelling of both hands was observed, with thickening of the skin of both hands with proximal extension until the forearm. Biopsy confirmed the diagnosis of scleroderma. Symptomatic and rehabilitation treatment was initiated with mild improvement of symptoms. To our knowledge this is the first case of MCL ASSOCIATED WITH SYSTEMIC SCLEROSIS.