CELL-MEDIATED IMMUNE-PANCYTOPENIA COMPLICATING PRIMARY SJOGRENS-SYNDROME

A 64-year-old woman with mild bilateral parotid gland swelling and bil ateral lower extremity purpura was admitted for evaluation of xerostom ia and pancytopenia. The patient had an increased erythrocyte sediment ation rate, pancytopenia, and positive tests for antibodies to nuclear antigen, SS-A, and SS-B. Impaired cell-mediated immunity was also pre sent. Bone marrow aspiration showed a hypoplastic marrow with an incre ased percentage of lymphocytes. A positive Schirmer's test and keratoc onjunctivitis were also noted. A diagnosis of primary Sjogren's syndro me was made by sialography and histological salivary gland findings. T herapy with prednisolone improved the pancytopenia. Addition of the pa tient's peripheral blood mononuclear cells to cultures of bone marrow mononuclear cells derived from a healthy volunteer dose dependently in hibited colony formation, including mixed hemopoietic colonies. On the other hand, addition of the patient's serum failed to inhibit colony formation by normal bone marrow mononuclear cells. These results sugge sted that one of the causes of pancytopenia in primary Sjogren's syndr ome might be mediated by the inhibition of mononuclear cells to the he mopoietic progenitors.