Haematological manifestations of primary Sjögren's syndrome: a clinicopathological study

Clinically significant cytopenias are thought to be uncommon in primary Sjögren's syndrome: only a few cases have been reported in the literature. Over a 3-year period we identified haematological abnormalities in 11 of 27 patients with Sjögren's syndrome. Six patients had a positive direct antiglobulin test, including one patient with all the features of autoimmune haemolytic anaemia and two others with some features of this condition. Four patients had immune thrombocytopenia and two patients had myelodysplastic syndrome. Neutropenia was noted in two patients, one patient had aplastic anaemia and one had pure red cell aplasia. Haematological disorders were found to be common in patients with Sjögren's syndrome (40 per cent). Accordingly, we suggest that patients with immune cytopenia(s) should be screened for Sjögren's syndrome using sensitive assays for anti-SS.A and anti-SS.B antibodies, and that patients with Sjögren's syndrome should be periodically monitored, with a full blood count to rule out any haematological abnormality.