[Thrombotic thrombocytopenic purpura in childhood].
Thrombotic thrombocytopenic purpura (TTP) is a micro-angiopathic disease due to deficiency of the specific VWF cleaving protease (VWF-CP) ADAMTS13. The acquired form is caused by autoantibodies against VWF-CP, whereas mutations of the ADAMTS13 gene are responsible for inherited TTP. In childhood both forms exist with predominance of inherited TTP. The phenotype of TTP in childhood can be rather variable. Besides the classical clinical picture, abortive forms may occur that can delay the identification of patients at risk. The patients are frequently assumed to suffer from idiopathic thrombocytopenia (ITP) or Evans syndrome. Further efforts are necessary to accelerate correct diagnosis and to establish a risk-adapted prophylactic therapy.
Hassenpflug
W-A
WA
Universitätsklinikum Hamburg-Eppendorf, Zentrum für Frauen-, Kinder- und Jugendmedizin, Klinik für Pädiatrische Hämatologie und Onkologie, Martinistrasse 52, 20246 Hamburg. hassenpflug@uke.uni-hamburg.de
Angerhaus
D
D
Budde
U
U
Obser
T
T
Schneppenheim
R
R
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D006461:Hemolysis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008666:Metalloendopeptidases / Q000235:genetics; D010641:Phenotype; D011379:Prognosis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000235:genetics*
D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human