November 20 fmc formacion medica continuada en atencion primaria. Pdf purpura trombocitopenica trombotica sin esquistocitos. Purpura trombocitopenica trombotica asociada a nefritis lupica an med mex 2017. Purpura trombocitopenica trombotica asociada a nefritis lupica. Pupura trombocitopenica trombotica e uma sindrome aguda descrita.
Purpura trombocitopenica trombotica, citologia youtube. Thrombotic thrombocytopenic purpura ttp is the most extensive and dangerous intravascular platelet clumping disorder. We also analyze the role of adamts levels for the management and diagnosis of this disease. Thrombotic thrombocytopenic purpura, atypical hemolytic uremic syndrome. A postoperative thrombotic thrombocytopenic purpura in a. Reunion cientifica sah aahi purpura trombotica trombocitopenica 2211 20 3ra parte. The etiopathogenic mechanisms may result from abnormalities in any of the three components of hemostasis. Purpura trombotica trombocitopenica y sindrome hemolitico uremico. The last 20 years have been marked by the connection between an old disease, the thrombotic thrombocytopenic purpura ttp, 1 and a young protein, adamts a disintegrin and metalloprotease with thrombospondin type 1 repeats, member. Esta enfermedad afecta a varones y a mujeres por igual. Purpura is a disease in which there is leakage of blood out of the blood vessels in the skin or mucosa, causing the presence of painless purplish spots, called petechiae small and large number stains or ecchymoses larger patches. Thrombotic thrombocytopenic purpura blood american. Susana avila martinez purpura trombocitopenica trombotica 2. Adamts, purpura trombotica trombocitopenica, sindrome uremico hemolitico atipico.
He proposed that a powerful poison with both agglutina. The new engl and journal of medicine 90 n engl j med 368. Volume 53, issue 1, januaryfebruary 20, pages 123126. Feb 08, 2016 purpura trombocitopenica trombotica 1. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext.
Purpura trombotica trombocitopenica medicina clinica. Adamts antibody depletion by bortezomib in thrombotic. Thrombotic thrombocitopenic purpura is a rare disease with a morphological expression featured of many microthrombi in the terminal arterioles of several vital structures. Response to plasma exchange and steroids as combined therapy for patients with thrombotic thrombocytopenic purpura. We report two patients diagnosed as having idiopathic thrombocytopenic purpura refractory to plasma exchange in which the use of additional treatment was necessary. This results in a low platelet count, low red blood cells due to their breakdown, and often kidneys, heart, and brain dysfunction. Moschcowitz was the first to inform multiple hyaline thrombi as the primordial finding of a partial autopsy case. In one case there was an adequate response to rituximab. For more than a halfcentury after its initial recognition, mortality was near 100% and the etiology totally obscure. Purpura results from the extravasation of blood cells into the skin andor mucous membranes giving rise to small purple coloured areas that do not disappear under pressure.
Adamts na purpura trombocitopenica trombotica ptt dpm. In its fullblown form, the disease consists of the following pentad. Purpura trombotica trombocitopenica ptt the oncology. Embolizacion parcial esplenica en purpura trombocitopenia. Eperto e bases del diagstico e hematologa 4 purpura trombotica trombocitopenica y sindrome hemolitico uremico figura 1. Protocolo clinico e diretrizes terapeuticas portaria sasms n. Since the frequency of earlyonset thrombocytopenia in postcardiac surgery patients is high, platelet concentrates are commonly transfused during postoperative management. Pdf purpura trombocitopenica trombotica asociada a. Em seu relatorio do primeiro paciente diagnosticado com purpura trombocitopenica trombotica ptt, moschcowitz descreveu trombos hialinos nas arteriolas terminais do rim e do musculo cardiaco. Thrombotic thrombocytopenic purpura ttp is a blood disorder that results in blood clots forming in small blood vessels throughout the body. Jun 05, 20 although thrombotic thrombocytopenic purpura ttp is a rare disease, when it develops in a postcardiac surgery patient, it may have a fatal outcome. A purpura trombocitopenica idiopatica pti, tambem conhecida como purpura trombocitopenica. Purpura trombocitopenica trombotica etiologia bmj best. Pdf purpura trombocitopenica trombotica asociada a embarazo.
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