Púrpura trombocitopénico trombótico con respuesta exitosa a vincristina / Successful treatment of thrombotic thrombocytopenic purpura with vincristine report of one case

Thrombotic thrombocytopenic purpura presents as a multisystemic disease with thrombocytopenia, microangiopathic hemolytic anemia, fever, neurological and renal involvement. We report a 24 years-old male presenting with purpura and a generalized seizure. His blood tests showed an hemolytic anemia, unconjungated hyperbilirubinemia, increased lactated dehydrogenase, thrombocytopenia and impairment of renal function. He was initially treated with daily plasmapheresis and steroids without improvement. Due to persistence of the disease, he was treated with two doses of intravenous vincristine in four days, with clinical and laboratory improvement. He was discharged 40 days after the last dose of vincristine, in good conditions.

Eficacia de la asociación rituximab-vindesina en el tratamiento de un caso de púrpura trombótico-trombopénico (PTT) adquirido idiopático refractario / Successful treatment of idiopathic acquired refractory thrombotic thrombocytopenic purpura with an association of rituximab - vindesine. Report of one case

We report a 23 years old female who presented a second episode of thrombotic thrombocytopenic purpura (TTP). She was treated with fresh frozen plasma infusions and 14 plasma exchange (PE) sessions without response. Therefore a second-line therapy was started, associating a weekly cycle administration of vindesine (Vds) 2 mg/m2 and rituximab (R) 375 mg/m2. Five cycles of this association plus one cycle of R exclusively, were administered. After the third course, biological signs of improvement were observed and complete normalization of blood cell counts and other specific parameters was seen after 8 weeks. From the beginning of her second relapse we detected a severe deficit (<5%) in von Willebrand-cleaving factor (ADAMTS13) associated to the presence of ADAMTS13 inhibitors. The combined treatment induced an improvement in ADAMTS13 values without detectable inhibitors. After 21 months of follow-up the patient was well, without signs of relapse but ADAMTS13 values were still under normal, which may be an unfavorable prognostic factor. PE is the treatment of choice for acquired idiopathic TTP, but for refractory cases or TTP cases with severe ADAMTS13 values/high inhibitor titers, PE associated to an immunosuppressive treatment should be considered.

Thrombotic thrombocytopenic purpura a case report and revivew of the literature

Thrombotic thrombocytopenic purpura [TTP] is a rare potentially fatal disease characterized by disseminated capillary fibrin thrombi The pathogencsis is complex and involves endothelial and platelet abnormalities.we describe a fatal case of TTP with a brief review of the literature

Púrpura trombocitopénica trombótica: informe de un caso que respondió a la infusión de plasma / Thrombocytopenic purpura: a case report responding to plasma infusions

Un niño inició cuadros de púrpura trombocitopénica a los dos años de edad. Se pudo descartar tanto una forma hereditaria, como una condición determinada por anticuerpos antiplaquetarios. A los ocho años de edad presentó parálisis facial de tipo central y se pudo demostrar en la biopsia de médula ósea y en la encía, la presencia de trombos hialinos en la microvasculatura. La aplicación de plasma ha determinado la remisión de todos los brotes observados hasta el mes de mayo de 1985