Thrombotic Thrombocytopenic Purpura as an Initial Presentation of Primary Sjo¨gren’s Syndrome

A healthy woman presented with ecchymoses due to thrombocytopenia, with numerous bone marrow megakaryocytes, microangiopathic haemolytic anaemia, disorientation, irritability, and normal renal function. Thrombotic thrombocytopenic purpura (TTP) was diagnosed and treated successfully by plasma exchange therapy, both on presentation and during a further three relapses. The TTP was considered idiopathic until, 4 months later, definite primary Sjo¨gren’s syndrome (1°SS) was diagnosed following the appearance of sicca symptoms. Only four similar cases have been cited in the literature. TTP should be added to the varied haematological manifestations that may occur in patients with 1°SS. The possible presentation of 1°SS not with classic sicca symptoms but rather with haematological abnormalities, including TTP, should be recognised.