title: Regional UK TTP registry: correlation with laboratory ADAMTS 13 analysis and clinical features.
abstract: 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 <10% in 74% and 95% of these cases had positive IgG antibodies to ADAMTS 13. Renal impairment and delayed normalisation of platelet count were the main differences between idiopathic and secondary TTP.
authors.affiliation: Department of Haematology, Haemostasis Research Unit, University College London, London, UK.
authors.forename: Marie
authors.initials: M
authors.lastname: Scully
authors.forename: Helen
authors.initials: H
authors.lastname: Yarranton
authors.forename: Ri
authors.initials: R
authors.lastname: Liesner
authors.forename: Jamie
authors.initials: J
authors.lastname: Cavenagh
authors.forename: Beverley
authors.initials: B
authors.lastname: Hunt
authors.forename: Sylvia
authors.initials: S
authors.lastname: Benjamin
authors.forename: David
authors.initials: D
authors.lastname: Bevan
authors.forename: Ian
authors.initials: I
authors.lastname: Mackie
authors.forename: Samuel
authors.initials: S
authors.lastname: Machin
mesh_terms: D051722:ADAM Proteins / Q000037:antagonists & inhibitors / Q000097:blood*; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D004739:England / Q000453:epidemiology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008666:Metalloendopeptidases / Q000037:antagonists & inhibitors / Q000097:blood*; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000453:epidemiology* / Q000209:etiology / Q000628:therapy; D012042:Registries
chemical_list: D007074:Immunoglobulin G; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human