Chronic relapsing thrombotic thrombocytopenic purpura: three case reports and update of the pathogenesis and therapeutic modalities

Abstract

Chronic relapsing thrombotic thrombocytopenic purpura (CRTTP) is the rarest type of TTP, usually presenting in childhood. The aetiology is still not fully explained. The disorder is associated with the presence in plasma of unusually large von Willebrand factor (UlvWF) multimers that are especially prominent between episodes. CRTTP can be prevented by periodic plasma transfusions. We report three children with congenital CRTTP who have been successfully treated and maintained in prolonged remission by the prophylactic use of fresh frozen plasma without concurrent plasmapheresis. Two of the patients are sibs.

New strategies in diagnosis and treatment of thrombotic thrombocytopenic purpura: case report and review

“…Regular infusions of FFP in patients with hereditary de®ciency of vWFcleaving protease may prevent further attacks of the disease. Although the biological half-life of the protease is about 2 to 4 days [12], prophylactic plasma therapy at intervals of 2 to 4 weeks appears to prevent relapses of TTP [18,20]. Maes et al [20] reported three children with CRTTP who were treated with regular FFP infusions every 2 weeks.…”

“…Although the biological half-life of the protease is about 2 to 4 days [12], prophylactic plasma therapy at intervals of 2 to 4 weeks appears to prevent relapses of TTP [18,20]. Maes et al [20] reported three children with CRTTP who were treated with regular FFP infusions every 2 weeks. Using this schedule, the patients were maintained in remission for over 3 years [20].…”

“…Maes et al [20] reported three children with CRTTP who were treated with regular FFP infusions every 2 weeks. Using this schedule, the patients were maintained in remission for over 3 years [20]. Commercially prepared FFP in which viruses had been inactivated by the solvent-detergent procedure or by the methylene blue method contain normal levels of enzyme activity [11,38].…”

“…In intermittent TTP the disease takes a relapsing course at occasional irregular intervals. The rarest form is chronic relapsing TTP (CRTTP) which often begins in childhood [3,20]. This type of TTP is characterized by frequent episodes at irregular intervals.…”

Thrombotisch-thrombozytopenische Purpura (TTP) – Fallbericht, Pathogenese und Therapieoptionen

“…1). Anhand des Auftretens der einzelnen Krankheitsepisoden können des Weiteren akute einzelne Episoden von intermittierend rezidivierenden oder auch chronisch rezidivierenden Formen unterschieden werden (20).…”