Regional UK TTP registry: correlation with laboratory ADAMTS 13 analysis and clinical features
| Autor: | Beverley J. Hunt, Ri Liesner, Sylvia Benjamin, Samuel J. Machin, Helen Yarranton, Jamie Cavenagh, Marie Scully, Ian J. Mackie, David H. Bevan |
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| Rok vydání: | 2008 |
| Předmět: |
Hemolytic anemia
Adult Male medicine.medical_specialty Adolescent Thrombotic thrombocytopenic purpura ADAMTS13 Protein Internal medicine medicine Coagulopathy Humans Registries Child Aged Aged 80 and over Hematology Plasma Exchange Purpura Thrombotic Thrombocytopenic Vascular disease business.industry ADAMTS Infant Newborn Infant Metalloendopeptidases Middle Aged medicine.disease Surgery ADAM Proteins England Child Preschool Immunoglobulin G Rituximab Female Caplacizumab business medicine.drug |
| Zdroj: | British journal of haematology. 142(5) |
| ISSN: | 1365-2141 |
| Popis: | Thrombotic thrombocytopenic purpura (TTP) is an acute, rare, life-threatening disorder. This report presents the South East (SE) England registry for TTP, from April 2002 to December 2006, which included 176 patients and 236 acute episodes; 75% of patients were female and 25% were male, overall median age at presentation was 42 years. Mortality was 8.5%, most cases died before treatment was instigated. The main ethnic groups were Caucasian (64%) and Afro Caribbean (27%). Seventy-seven percent of cases were idiopathic, 5% were congenital and the remaining cases had a defined precipitant. Neurological features were the most prevalent, but cardiac involvement accounted for 42% of presenting features. The overall median number of plasma exchanges (PEXs) to remission was 15; between April 2002 and December 2003, the median number of PEXs was 19 and it was 12 between January 2004 and December 2006 (P < 0.0001). In the latter period, adjuvant therapies were reduced, but Rituximab was increased. ADAMTS 13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity was |
| Databáze: | OpenAIRE |
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