Thrombotic thrombocytopenic purpura as an initial presentation of primary Sjögren's syndrome

• Published in: Clinical rheumatology 2002-02, Vol.21 (1), p.57-59
• Main Authors: Schattner, A, Friedman, J, Klepfish, A
• Format: Article
• Language: English
• Subjects: Female; Humans; Middle Aged; Plasma Exchange; Purpura, Thrombotic Thrombocytopenic - etiology; Purpura, Thrombotic Thrombocytopenic - therapy; Sjogren's Syndrome - complications; Sjogren's Syndrome - diagnosis
• ISSN: 0770-3198
• DOI: 10.1007/s100670200013

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 Sjogren's syndrome (1 degree 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 degree SS. The possible presentation of 1 degree SS not with classic sicca symptoms but rather with haematological abnormalities, including TTP, should be recognised.