[Development of acute myelocytic leukemia in the course of Sjögren's syndrome]

A 53-year-old female, who had been having xerostomia and xerophthalmia for two years, was admitted in November 1984, because of subfever and malaise. Laboratory examination revealed pancytopenia with appearance of blasts, mild hypergammaglobulinemia, and elevated titers of ANA, anti SS-A and anti SS-B. Bone marrow aspirate showed increased myeloblasts. Schirmer's test and Rose Bengal's test were both positive, and sialography showed apple tree like appearance. Pathological findings of biopsied lip mucosa showed remarkable infiltration of lymphocytes. The patient was diagnosed acute myelocytic leukemia and Sjögren's syndrome. In spite of recovery of peripheral blood after combination chemotherapy (BHAC-DMP), a disturbance of consciousness appeared and she died of its progression. It was suspected clinically that she was suffered from progressive multifocal leukoencephalopathy (PML). It is sometimes reported that patients with Sjögren's syndrome develop malignant lymphoma, but it is very rare that Sjögren syndrome develop AML.