Diagnosis of thrombotic thrombocytopenic purpura
| Autor: | E E Klebanova, G M Galstyan |
|---|---|
| Jazyk: | ruština |
| Rok vydání: | 2020 |
| Předmět: |
History
mutations of the adamts13 gene Endocrinology Diabetes and Metabolism Thrombotic thrombocytopenic purpura 030204 cardiovascular system & hematology Pathogenesis 03 medical and health sciences 0302 clinical medicine Von Willebrand factor hemic and lymphatic diseases medicine thrombotic thrombocytopenic purpura metalloproteinase adamts13 biology business.industry Organ dysfunction Autoantibody General Medicine Microangiopathic hemolytic anemia medicine.disease ADAMTS13 von willebrand factor inhibitor Immunology biology.protein Medicine medicine.symptom Antibody Family Practice business 030215 immunology |
| Zdroj: | Терапевтический архив, Vol 92, Iss 12, Pp 207-217 (2020) |
| ISSN: | 2309-5342 0040-3660 |
| Popis: | Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease, disease, characterised by microangiopathic hemolytic anaemia, consumption thrombocytopenia, and organ dysfunction. The pathogenesis of TTP is attributed to the deficiency in the activity of the metalloproteinase ADAMTS13, specific von Willebrand factor cleaving protease. TTP is suspected when detecting microangiopathic hemolytic anemia, thrombocytopenia, damage to various organs. Diagnosis of TTP is confirmed by the detection of ADAMTS13 activity in plasma less than 10%. Plasma samples for the study of ADAMTS13 activity should be taken before the start of plasma transfusions or plasma exchange. In patients with severe ADAMTS-13 deficiency autoantibodies anti-ADAMTS13 and inhibitor ADAMTS13 should be investigated. Anti-ADAMTS13 antibodies belonging to IgG not always have inhibitory effects. The inhibitory effect of anti-ADAMTS13 antibodies is confirmed by mixing test. All patients with the first established diagnosis of TTP should be examined for mutations of the ADAMTS13 gene. |
| Databáze: | OpenAIRE |
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