ABSTRACT
We report a case of a 61-year-old man with a history of CREST syndrome [calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia] who presented for evaluation of thrombocytopenia. He had evident cervical adenopathy and lymph node biopsy showed small lymphocytic lymphoma [SLL] with evident systemic adenopathy and bone marrow involvement. The patient achieved a complete remission with FCR [fludarabine/cyclophosphamide/rituximab] chemotherapy. About 30 cases of lymphomas are reported in the literature in association with systemic sclerosis. To our knowledge, there are no reports of a small lymphocytic lymphoma [SLL] in association with limited cutaneous systemic sclerosis with classic features of the CREST syndrome