Non-Hodgkin's lymphoma & primary biliary cirrhosis with Sjogren's syndrome

Sjogren's syndrome (SS) is an autoimmune disease characterized by a lymphoc ytic infiltration of the salivary and lacrimal glands leading to a progress ive destruction of these glands due to the production of autoantibodies. Th is disorder is either isolated (primary SS) or associated with other system ic diseases (secondary SS). The occurrence of B-cell non-Hodgkin's lymphoma (NHL) represents the major complication in the evolution of SS patients. T he risk of developing NHL, which is equivalent for both primary and seconda ry SS. was estimated to be 44 times greater than that observed in a compara ble normal population. NHLs in SS patients occur preferentially in the sali vary glands and in other mucosa-associated lymphoid tissues (MALT). However , it can also occur in the lymph nodes or bone marrow. We documented a case of low-grade B-cell lymphoma of MALT in the right eyel id and primary biliary cirrhosis (PBC) of a patient with SS. To the best of our knowledge, this is the first case reported in Korea.