Long-term deficits in health-related quality of life after recovery from thrombotic thrombocytopenic purpura

Abstract

BACKGROUND: After recovery from thrombotic thrombocytopenic purpura (TTP), patients commonly describe persistent problems with memory, concentration, and endurance. STUDY DESIGN AND METHODS: The Oklahoma TTP-HUS Registry, an inception cohort of 364 patients, January 1, 1989, through June 30, 2007, has annually evaluated health-related quality-of-life since 1998 with the Short Form (SF)-36, a widely used questionnaire that assesses eight domains of physical and mental health. RESULTS: A total of 128 patients were eligible for this study (age ≥ 18 years, alive in January 1998 without prior relapse, survival and no relapse ≥1 year after recovery, no overt disability or additional disorders); 118 (92%) completed the SF-36. At their initial assessment, these patients had significantly worse functioning and well-being than the US population for all domains (p < 0.05). Forty-nine patients who had their initial assessment more than 2 years after recovery had better scores for three domains than 69 patients who had their initial assessment 2 years or less after recovery (p < 0.05). Among these 69 patients, there was no difference between subgroups: 1) idiopathic versus other categories of TTP, 2) the presence or absence of severe ADAMTS13 deficiency, 3) the presence or absence of severe neurologic abnormalities during the episode of TTP, and 4) less than 10 or 10 or more plasma exchange treatments required to achieve remission (p > 0.05). Analysis of serial assessments in 72 (61%) of the 118 patients who had two or more assessments within 5 years of recovery and no intervening relapses demonstrated no significant improvement in any of the domains. CONCLUSION: After recovery from TTP, patients may have persistent cognitive and physical difficulties. © 2008 American Association of Blood Banks.