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- Title
The utility of ADAMTS13 in differentiating TTP from other acute thrombotic microangiopathies: results from the UK TTP Registry.
- Authors
Hassan, Sevda; Westwood, John‐Paul; Ellis, Debra; Laing, Chris; Mc Guckin, Siobhan; Benjamin, Sylvia; Scully, Marie
- Abstract
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 x 109/l; range 0-96) than aHUS (57 x 109/l; range 13-145, P < 0.0001) or STEC-HUS (35 x 109/l; range 14-106, P < 0.0001); they also had lower creatinine levels (92 lmol/l; range 43-374) than aHUS (255 lmol/l; range 23-941, P < 0.0001) and STEC-HUS (324 lmol/l; range 117-639, P < 0.0001). However, 12/34 (35.3%) aHUS patients had a platelet count <30 x 109/l and 26/150 (17.3%) of TTP patients had a platelet count >30 x 109/l; 23/150 (15.3%) of TTP patients had a creatinine level >150 lmol/l. This study highlights the wide variety of TMA presentations, and confirms the utility of ADAMTS13 testing in TTP diagnosis.
- Subjects
THROMBOTIC microangiopathies; THROMBOTIC thrombocytopenic purpura treatment; VEROCYTOTOXINS; TREATMENT effectiveness; FLUORESCENCE resonance energy transfer; MEDICAL registries; DIAGNOSIS
- Publication
British Journal of Haematology, 2015, Vol 171, Issue 5, p830
- ISSN
0007-1048
- Publication type
Article
- DOI
10.1111/bjh.13654