title: Multiple major morbidities and increased mortality during long-term follow-up after recovery from thrombotic thrombocytopenic purpura.
abstract: Recovery from acute episodes of thrombotic thrombocytopenic purpura (TTP) appears complete except for minor cognitive abnormalities and risk for relapse. The Oklahoma TTP-HUS (hemolytic uremic syndrome) Registry enrolled 70 consecutive patients from 1995 to 2011 with ADAMTS13 activity <10% at their initial episode; 57 survived, with follow-up through 2012. The prevalence of body mass index (BMI), glomerular filtration rate (GFR), urine albumin/creatinine ratio (ACR), hypertension, major depression, systemic lupus erythematosus (SLE), and risk of death were compared with expected values based on the US reference population. At initial diagnosis, 57 survivors had a median age of 39 years; 45 (79%) were women; 21 (37%) were black; BMI and prevalence of SLE (7%) were greater (P < .001) than expected; prevalence of hypertension (19%; P = .463) was not different. GFR (P = .397) and ACR (P = .793) were not different from expected values. In 2011-2012, prevalence of hypertension (40% vs 23%; P = .013) and major depression (19% vs 6%; P = .005) was greater than expected values. Eleven patients (19%) have died, a proportion greater than expected compared with US and Oklahoma reference populations (P < .05). TTP survivors may have greater risk for poor health and premature death.
authors.affiliation: Department of Biostatistics and Epidemiology, College of Public Health.
authors.forename: Cassandra C
authors.initials: CC
authors.lastname: Deford
authors.forename: Jessica A
authors.initials: JA
authors.lastname: Reese
authors.forename: Lauren H
authors.initials: LH
authors.lastname: Schwartz
authors.forename: Jedidiah J
authors.initials: JJ
authors.lastname: Perdue
authors.forename: Johanna A
authors.initials: JA
authors.lastname: Kremer Hovinga
authors.forename: Bernhard
authors.initials: B
authors.lastname: Lämmle
authors.forename: Deirdra R
authors.initials: DR
authors.lastname: Terrell
authors.forename: Sara K
authors.initials: SK
authors.lastname: Vesely
authors.forename: James N
authors.initials: JN
authors.lastname: George
mesh_terms: D051722:ADAM Proteins / Q000378:metabolism; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D000368:Aged; D002423:Cause of Death; D002648:Child; D004789:Enzyme Activation; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007677:Kidney Function Tests; D008297:Male; D008875:Middle Aged; D009017:Morbidity; D009026:Mortality; D011697:Purpura, Thrombotic Thrombocytopenic / Q000453:epidemiology*; D012008:Recurrence; D012042:Registries; D055815:Young Adult
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human