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.

BACKGROUND: Plasma exchange (PEX) treatment for patients with thrombotic thrombocytopenic purpura‐hemolytic uremic syndrome (TTP‐HUS) has risk for major complications. STUDY DESIGN AND METHODS: Data for PEX‐related complications have been prospectively collected on all patients enrolled in the Oklahoma TTP‐HUS Registry, 1996 to 2011. PEX‐related complications have been defined as major or minor and as central venous catheter related or plasma related. RESULTS: During 15 years, 1996 to 2011, 72 (24%) of 302 consecutive patients had major PEX‐related complications. Analysis of five consecutive 3‐year cohorts demonstrated that there has been a significant trend for decreasing frequency of all PEX‐related major complications (p = 0.014) and central venous catheter–related major complications (p = 0.021) but not for the less common plasma‐related major complications (p = 0.380). ADAMTS13 activity was measured in 288 (95%) of the 302 patients. Analysis of the 66 patients with ADAMTS13 activity of less than 10% demonstrated a significant trend for decreasing frequency of PEX‐related major complications (p = 0.036); the trend for the 222 patients with ADAMTS13 activity of at least 10% was not significant (p = 0.118). The decreased frequency of PEX‐related major complications among patients with ADAMTS13 activity of less than 10% may be related to a significant trend for decreasing duration of PEX treatment (p = 0.040) and decreasing frequency of requirement for more than one central venous catheter (p = 0.044). The decreased duration of PEX treatment may be related to increased use of adjunctive treatments: corticosteroids (p < 0.001) and rituximab (p < 0.001). CONCLUSIONS: The frequency of PEX‐related major complications has decreased from 1996 to 2011, possibly related to increased use of corticosteroids and rituximab and the decreased duration of PEX required to achieve remission.