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Congenital Thrombotic Thrombocytopenic Purpura
Thrombotic Thrombocytopenic Purpura Familial

Presentation

One month afterwards, she returned presenting weakness and swollen face and legs, which had developed one day earlier. The ADAMTS13 activity was less than 5%, without presence of autoantibodies. [pesquisa.bvsalud.org]

Entire Body System

  • Fatigue

    It was only later that the doctors diagnosed her with idiopathic thrombocytopenic purpura, a platelet deficiency that causes fatigue, bleeding and speckly bruises called petechiae. [fr.bab.la]

  • Weakness

    One month afterwards, she returned presenting weakness and swollen face and legs, which had developed one day earlier. The ADAMTS13 activity was less than 5%, without presence of autoantibodies. [pesquisa.bvsalud.org]

Treatment

Pathogenesis and treatment of acquired idiopathic thrombotic thrombocytopenic purpura. Haematologica 2010; 95(9): 1444-47. Contreras E, de la Rubia J, del Río-Garma J, Díaz-Ricart M, GarcíaGala JM, Lozano M. [cimel.felsocem.net]

The patient evolved with increasing platelet counts, even without specific treatment, and she was discharged. One month afterwards, she returned presenting weakness and swollen face and legs, which had developed one day earlier. [pesquisa.bvsalud.org]

Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Engl J Med. 1991; 325(6):393-397. [medigraphic.com]

Epidemiology

Epidemiology. 2004; 15(2):208-15. George JN, Nester CM. Syndromes of Thrombotic Microangiopathy. N Engl J Med [Internet]. 2014;371(7):654–66. Available from: http:// www.ncbi.nlm.nih.gov/pubmed/25119611 Bitzan M, Bickford BB, Foster GH. [cimel.felsocem.net]

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