Thrombotic thrombocytopenic purpura in childhood |
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Authors: | Maria C. Bouw MD Natasja Dors MD Heleen van Ommen MD PhD Nicole L. Ramakers‐van Woerden MD PhD |
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Affiliation: | 1. Department of Paediatrics, Meander Medical Centre, Amersfoort, The Netherlands;2. Department of Paediatric Haematology, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands |
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Abstract: | Thrombotic thrombocytopenic purpura (TTP) is a rare disease, especially in childhood, and has a high mortality rate in the absence of appropriate treatment. It is characterised by microangiopathic haemolytic anaemia and consumptive thrombocytopenia. TTP may be difficult to distinguish from haemolytic uraemic syndrome (HUS) because of similar clinical manifestations and laboratory findings. In the past, TTP and HUS have often been considered to represent variable expressions of a single entity. Our increased understanding of the pathogenesis of TTP has in turn resulted in significant improvements in its treatment and outcomes. Several immunomodulating agents are currently being used with variable outcomes. Pediatr Blood Cancer 2009;53:537–542. © 2009 Wiley‐Liss, Inc. |
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Keywords: | ADAMTS13 haemolytic uraemic syndrome thrombotic microangiopathy thrombotic thrombocytopenic purpura von Willebrand factor cleaving protease |
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