Chronic neutropenia: possible association with Sjögren's syndrome

Three white males, ages 42-59, with chronic neutropenia are described. All 3 patients had splenomegaly, positive tests for rheumatoid factor, elevated erythrocyte sedimentation rates, hypergammaglobulinemia and bone marrow examinations which showed normal to increased cellularity. Two of the 3 patients had recurring arthralgias and positive tests for antinuclear antibodies. All 3 patients had evidence for decreased tearing suggestive of Sjogren's syndrome, but in only 1 patient was a positive lip biopsy obtained. One patient had repeated cutaneous infections which ceased after splenectomy resulted in an increased neutrophil count. Serum IgG neutrophil-binding and in vivo neutrophil kinetic studies on 1 patient were consistent with a humoral immune mechanism for his neutropenia.