[Thrombotic thrombocytopenic purpura in childhood]
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[Thrombotic thrombocytopenic purpura in childhood]. / Hassenpflug, W-A; Angerhaus, D; Budde, U; Obser, Tobias; Schneppenheim, R.
In: HAMOSTASEOLOGIE, Vol. 24, No. 1, 1, 2004, p. 71-76.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - [Thrombotic thrombocytopenic purpura in childhood]
AU - Hassenpflug, W-A
AU - Angerhaus, D
AU - Budde, U
AU - Obser, Tobias
AU - Schneppenheim, R
PY - 2004
Y1 - 2004
N2 - 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.
AB - 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.
M3 - SCORING: Zeitschriftenaufsatz
VL - 24
SP - 71
EP - 76
JO - HAMOSTASEOLOGIE
JF - HAMOSTASEOLOGIE
SN - 0720-9355
IS - 1
M1 - 1
ER -
