Key Points• After recovering from TTP, the prevalence of hypertension, depression, and systemic lupus erythematosus and risk of death are increased.• TTP may be a more chronic disorder rather than a disorder of acute episodes and complete recovery.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 5 .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 5 .013) and major depression (19% vs 6%; P 5 .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. (Blood. 2013;122(12):2023-2029 Continuing Medical Education online This activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education through the joint sponsorship of Medscape, LLC and the American Society of Hematology. Medscape, LLC is accredited by the ACCME to provide continuing medical education for physicians. Medscape, LLC designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity. All other clinicians completing this activity will be issued a certificate of participation. To participate in this journal CME activity: (1) review the learning objectives and author disclosures; (2) study the education content; (3) take the post-test with a 70% minimum passing score and complete the evaluation at http://www.medscape.org/journal/blood; and (4) view/print certificate. For CME questions, see page 2142. Disclosures Johanna A. Kremer Hovinga, Bernhard Lämmle, and James N. George are consultants for Baxter, Inc. for the development of rADAMTS13 as a potential treatment of thrombotic thrombocytopenic purpura (TTP). James N. George is a consultant for Alexion, Inc. for eculizumab as treatment of aHUS. The Associate Editor Leslie V. Parise has served as an advisor or consultant for Bayer, BD Technologies, Biogen, and the Blood Research Institute of Wisconsin; has received gran...

Purpose Following recovery from an acute episode of acquired thrombotic thrombocytopenic purpura (TTP), patients often describe problems with memory, concentration, and endurance. We have previously reported the occurrence of depression and cognitive impairment in these patients. In this study we describe the frequency, severity and clinical course of depression and cognitive impairment. Findings Fifty-two (85%) of 61 eligible Oklahoma Registry patients who had recovered from TTP, documented by ADAMTS13 activity <10%, have had at least one (median, four) evaluation for depression over 11 years using the Beck Depression Inventory-II; 31 (59%) patients screened positive for depression at least once; in 15 (29%), the results suggested severe depression at least once. Nine of these 15 patients had a psychiatric interview, the definitive diagnostic evaluation; the diagnosis of major depressive disorder was established in eight (89%) patients. In 2014, cognitive ability was evaluated in 33 patients by the Montreal Cognitive Assessment (MoCA) and the Repeatable Battery for Assessment of Neuropsychological Status (RBANS). Both tests detected significant cognitive impairment in the patients as a group. Fifteen of the 33 patients had been evaluated by extensive cognitive tests in 2006. The 2014 RBANS results were significantly worse than the 2006 results for the overall score and two of five RBANS domains (immediate and delayed memory). Neither depression nor cognitive impairment was significantly associated with the occurrence of relapses or ADAMTS13 activity <10% during remission. Conclusion These observations emphasize the importance of screening evaluations for depression and cognitive impairment following recovery from acquired TTP.

366 Introduction Recovery from an acute episode of TTP is typically assumed to be complete. However patients from the Oklahoma TTP-HUS Registry have often described persistent problems with memory, concentration, and endurance. Our previous studies have documented long-term deficits in quality-of-life and cognitive functioning. We have also observed an unexpectedly high frequency of severe depression. Therefore we documented the relative frequency of severe depression during long-term follow-up and compared the relative frequency of severe depression in our patients to US and Oklahoma population data. Methods We included all Oklahoma TTP-HUS Registry patients whose initial episode was associated with severe ADAMTS13 deficiency (<10%), 1995–2010, and who were alive in 2004 when our psychiatric evaluations began. Patients completed the Beck Depression Inventory-II (BDI-II) 1–5 times from 2004–2011. The BDI-II is a self-report measure consisting of 21 items. Scores are interpreted as suggesting no/minimal, mild, moderate or severe depression. In 2011, patients who had BDI-II scores indicating moderate or severe depression on at least 1 evaluation had a structured psychiatric interview to support the diagnosis of depression. In 2012, all patients were asked to complete an 8-item measure, the Patient Health Questionnaire-8 (PHQ-8). The relative frequency of patients whose PHQ-8 scores indicated major depression were compared to the relative frequency of major depression determined by the PHQ-8 in the Oklahoma and US populations in the most recent Behavioral Risk Factor Surveillance System (BRFSS) data, 2006 and 2008. Results Of 68 patients who had severe ADAMTS13 deficiency at the time of their initial episode of TTP, 52 were alive in 2004; 47 (90%) were evaluated by the BDI-II. Fifteen (32%) of the 47 patients had scores suggesting severe depression on at least 1 evaluation; 12 were alive in 2011 and 10 (83%) of these 12 patients underwent a structured psychiatric interview; 9 met criteria for a major depressive disorder based on this diagnostic interview. Seven (15%) of the 47 patients had scores indicating only moderate depression; 4 (57%) of these 7 patients underwent a structured psychiatric interview; 1 (25%) met criteria for a major depressive disorder. Thirty-seven (88%) of 42 surviving patients in 2012 were evaluated by the PHQ-8 6.3 years (median) after their initial episode; 7 (18.9%, 95% CI, 8.0–35.2) had scores suggesting major depression, which is significantly greater than the prevalence of major depression in the US (3.4%) and Oklahoma (3.5%). The greater relative frequency of major depression was consistent across demographic subgroups. Conclusion The relative frequency of severe depression is increased in patients during long-term follow-up after recovery from TTP. Recognition and appropriate management of this clinically important health problem are critical components of the care of patients who have survived acute episodes of TTP. Disclosures: Kremer Hovinga: Baxter Healthcare: Consultancy, Research Funding. Terrell:Amgen, Inc.: Consultancy; Baxter, Inc.: Consultancy. George:Alexion, Inc.: Consultancy; Amgen, Inc.: Consultancy, PI for clinical trial involving romiplostim, PI for clinical trial involving romiplostim Other, Research Funding; Baxter, Inc.: Consultancy.