Depression and cognitive impairment following recovery from thrombotic thrombocytopenic purpura

Abstract

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.

Thrombotic thrombocytopenic purpura: diagnostic criteria, clinical features, and long-term outcomes from 1995 through 2015

“…In previous reports, we described the demographics, presenting clinical features, and long-term outcomes of these patients. [5][6][7][8][9] This article focuses on new observations that have not been previously reported. We describe the limitations of ADAMTS13 activity measurements for the diagnosis of TTP.…”

Clinical importance of ADAMTS13 activity during remission in patients with acquired thrombotic thrombocytopenic purpura

“…For comparisons at other remission ADAMTS13 activity levels, the hazard ratio for relapse was not significant. Other morbidities that may occur after recovery from TTP (hypertension, chronic kidney disease, depression, minor cognitive impairment, and death) 10,11 did not appear to be related to remission ADAMTS13 activity (supplemental Table 2). …”

“…[6][7][8] The use of PEGf in SCN has been reported only in single patients and in retrospective cohorts with limited pharmacokinetic analysis. [9][10][11][12] One limitation might derive from severe skin and lung toxicities, which were mainly observed in patients with cyclic neutropenia or glycogen storage disease type Ib but not in classical SCN. 9 In this study, we describe the long-term outcome of PEGf treatment in children with SCN who were poorly compliant to classical G-CSF (filgrastim).…”

Caplacizumab for Acquired Thrombotic Thrombocytopenic Purpura

“…3 These microthrombi cause tissue ischemia and organ dysfunction (commonly involving the brain, heart, and kidneys), resulting in early death 4,5 or in long-term complications, such as cognitive deficits, depression, and arterial hypertension, and a shortened life expectancy. [6][7][8][9][10] Treatment of acquired TTP consists of rapid initiation of plasma exchange to remove autoantibodies and ultralarge von Willebrand factor multimers and to replenish ADAMTS13. Immunosuppressive therapy (e.g., glucocorticoids and rituximab) 1,11 inhibits autoantibody formation.…”