Goodpasture's syndrome associated with thrombotic thrombocytopenic purpura secondary to an ADAMTS-13 deficit
| Autor: | Cristina Vega-Cabrera, Begoña Rivas-Becerra, Teresa Olea, Gloria del Peso, Jorge Martínez Ara, Rafael Selgas, Auxiliadora Bajo, M. Luz Picazo, Ana-Lidia Benitez |
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| Rok vydání: | 2012 |
| Předmět: |
Adult
Male Anti-Glomerular Basement Membrane Disease Urology medicine.medical_treatment Thrombotic thrombocytopenic purpura ADAMTS13 Protein Antibodies Monoclonal Murine-Derived Von Willebrand factor hemic and lymphatic diseases medicine Goodpasture's syndrome Humans Immunologic Factors biology Purpura Thrombotic Thrombocytopenic business.industry Platelet Count Acute kidney injury Microangiopathic hemolytic anemia Plasmapheresis medicine.disease Schistocyte ADAM Proteins Nephrology Immunoglobulin G Immunology biology.protein Rituximab business medicine.drug |
| Zdroj: | International urology and nephrology. 45(6) |
| ISSN: | 1573-2584 |
| Popis: | A 27-year-old man was hospitalized for acute kidney injury associated with antiglomerular basement membrane antibodies (anti-GBM). He underwent immunosuppression and plasma exchange therapy, without recovery of renal function. Later on, he was again admitted to the hospital with seizures. Evidence of microangiopathic hemolytic anemia, with schistocytes in peripheral blood, was present, as well as a persistent low platelet count and activity of von Willebrand factor from adherence to protease (ADAMTS-13) less than 1 %. The presence of IgG antibodies against ADAMTS-13 was documented, leading to a diagnosis of thrombotic thrombocytopenic purpura (TTP) in the context of Goodpasture's syndrome. The TTP was treated with rituximab and plasmapheresis with a good response. We conclude that early measurement of ADAMTS-13 activity dictated the most appropriate therapy and achieved excellent results in this patient. |
| Databáze: | OpenAIRE |
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