The utility of ADAMTS13 in differentiating TTP from other acute thrombotic microangiopathies: results from the UK TTP Registry
| Autor: | Sevda Hassan, Sylvia Benjamin, John-Paul Westwood, Marie Scully, Chris Laing, Debra Ellis, Siobhan Mc Guckin |
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| Rok vydání: | 2015 |
| Předmět: |
Adult
Male medicine.medical_specialty Adolescent Thrombotic thrombocytopenic purpura ADAMTS13 Protein urologic and male genital diseases Gastroenterology Diagnosis Differential Young Adult chemistry.chemical_compound hemic and lymphatic diseases Internal medicine medicine Humans Thrombotic Microangiopathies heterocyclic compounds Platelet Registries Child neoplasms Aged Retrospective Studies Aged 80 and over Creatinine Purpura Thrombotic Thrombocytopenic Platelet Count business.industry Infant Newborn Infant Retrospective cohort study Hematology Middle Aged respiratory system medicine.disease ADAMTS13 ADAM Proteins chemistry Child Preschool Immunology Female Differential diagnosis Haemolytic-uraemic syndrome business Biomarkers |
| Zdroj: | British Journal of Haematology. 171:830-835 |
| ISSN: | 0007-1048 |
| DOI: | 10.1111/bjh.13654 |
| Popis: | Thrombotic microangiopathies (TMAs) are frequently difficult to differentiate clinically, and measurement of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) remains vital in thrombotic thrombocytopenic purpura (TTP) diagnosis. We retrospectively reviewed cases referred for ADAMTS13 testing, using UK TTP Registry screening data. Of a total 810 cases, 350 were confirmed as TTP. The 460 non-TTP cases comprised secondary TMAs (24·57%) and haemolytic uraemic syndrome (HUS) (27·17% aHUS, 2·83% Shiga-like toxin-producing E. coli [STEC]-HUS); the remainder were TMAs with no clear association, not TMAs, or had no confirmed diagnosis. ADAMTS13 levels were significantly lower in TTP than STEC-HUS, aHUS and other TMAs. TTP patients had significantly lower platelet count (15 × 10(9) /l; range 0-96) than aHUS (57 × 10(9) /l; range 13-145, P0·0001) or STEC-HUS (35 × 10(9) /l; range 14-106, P0·0001); they also had lower creatinine levels (92 μmol/l; range 43-374) than aHUS (255 μmol/l; range 23-941, P0·0001) and STEC-HUS (324 μmol/l; range 117-639, P0·0001). However, 12/34 (35·3%) aHUS patients had a platelet count30 × 10(9) /l and 26/150 (17·3%) of TTP patients had a platelet count30 × 10(9) /l; 23/150 (15·3%) of TTP patients had a creatinine level150 μmol/l. This study highlights the wide variety of TMA presentations, and confirms the utility of ADAMTS13 testing in TTP diagnosis. |
| Databáze: | OpenAIRE |
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