Primary Sjögren’s syndrome (SS) is an autoimmune disease characterised by the presence of xerostomia and xerophthalmia without evidence of another systemic autoimmune disease. It has a wide clinical spectrum, extending from exocrinopathy to systemic autoimmune disease and to B cell lymphoma. The association of SS with aplastic anaemia (AA) has rarely been reported^1 2 and only in patients with lymphoma. We report here an exceptional case of primary SS and severe AA without lymphoma who had cytogenetic and immunological abnormalities, which might give clues to the pathogenesis of ‘idiopathic’ AA.

A 28 year old white man was referred in February 1990 for lymphadenopathies and pancytopenia. He complained of xerostomia and ocular burning. Xerophthalmia was confirmed by an abnormal Schirmer’s test (right eye 2 mm, left eye 1 mm after 10 mn) and a punctate keratitis on slit lamp examination after Rose-Bengal staining was observed. Labial salivary gland biopsy examination showed features of SS (grade 4 according to Chisholm’s focus score ³).

A polyclonal hypergammaglobulinaemia with a low level of IgA was present and fluorescent antinuclear antibodies were positive in a titre of 1: 640 with a …