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Rheumatology 2001; 40: 940-942
© 2001 British Society for Rheumatology

Paediatric Rheumatology

Acquired deficiency of von Willebrand factor-cleaving protease in a patient suffering from acute systemic lupus erythematosus

Paediatric Rheumatology/Series Editor: P. Woo

Letters to the Editor

T. Güngör, M. Furlan1, B. Lämmle1, F. Kuhn and R. A. Seger

University Children's Hospital, Division of Paediatric Immunology/Haematology, Zürich, and
1 Central Haematology Laboratory, University Hospital, Inselspital, Bern, Switzerland

SIR, Thrombotic thrombocytopenic purpura (TTP) is a well-known complication in established rheumatic diseases, especially in patients with systemic lupus erythematosus (SLE) (estimated incidence 1–4%). Post-mortem examination of SLE patients suggests that this association may be present in a significantly higher percentage of SLE patients succumbing to multi-organ system failure [1, 2].

TTP is a syndrome characterized by the pentad of thrombocytopenia, haemolytic microangiopathy, fluctuating neurological deficits, renal dysfunction and fever [3]. However, it has been observed that many patients with TTP may lack one or more of these criteria. The complete clinical pentad, characteristic of TTP, is entirely present in only about 30–40% of patients [4]. TTP may precede the onset of . . . [Full Text of this Article]

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