Depression and cognitive deficits as long‐term consequences of thrombotic thrombocytopenic purpura

Abstract

BACKGROUND Thrombotic thrombocytopenic purpura (TTP) is an acute life‐threatening microangiopathy with a tendency of relapse characterized by consumptive thrombocytopenia, microangiopathic hemolytic anemia, and spontaneous von Willebrand factor–induced platelet clumping leading to microthrombi. The brain is frequently affected by microthrombi leading to neurologic abnormalities of varying severity. STUDY DESIGN AND METHODS The aim of this observational cohort study was to investigate the prevalence of depression and cognitive deficits in 104 patients having survived acute TTP. TTP survivors were repeatedly assessed by means of different standardized questionnaires to evaluate depression (IDS‐SR) and mental performance (FLei). We received answers of 104 individual TTP patients and 55 of them participated in both surveys. RESULTS Seventy‐one of the 104 responding TTP patients (68%) suffered from depression and the severity of depression was similar in both surveys performed 1 year apart. Furthermore, TTP patients had considerably lower cognitive performance than controls. There was no correlation between prevalence of depression and cognitive deficits and the number and the severity of acute episodes. Impairment of mental performance correlated with the severity of depression (rs = 0.779). CONCLUSION The prevalence of depression and cognitive deficits was significantly higher in TTP patients. Cognitive impairment seemed to be a consequence of depression, almost independently of number and severity of TTP episodes.

Long-term neuropsychological sequelae, emotional wellbeing and quality of life in patients with acquired thrombotic thrombocytopenic purpura

“…In our study, the results of anxiety and depression tests were negatively correlated with scores of neurocognitive assessments, indicating that patients with symptoms of psychologic distress also had more pronounced cognitive defects. This is consistent with the findings of Falter and colleagues 18 , who reported a strong correlation between an impaired mental performance and the severity of depression in 84 TTP patients.…”

“…Our results are in line with previous findings by Kennedy et al in 24 acquired TTP patients from the Oklahoma Registry, who performed significantly worse than the US reference population in both attention and memory functions. 3,17 Conversely, at variance with our results, previous studies did not report an association of neurocognitive impairment with the occurrence of neurological manifestations at the time of the acute TTP event, 3 18 It is worth-noticing that the prevalence of relapsing TTP cases were higher in patients with than without neurological symptoms during the first acute event (41% versus 8%). Unfortunately, the low sample size did not allow us to discriminate the effects of these two factors.…”

“…Beside cognitive problems, we detected clinical anxiety and depression in 20% and 43% of interviewed patients. An even higher prevalence of depression symptoms in acquired TTP patients was reported in two US and one German cohorts (59%, 17 81%, 19 and 73%, 18 respectively), which included also cases of major depression (29%, 18 37%, 19 and 14% 18 ). However, a pre-existing diagnosis of depressive disorder was not an exclusion criterion in these studies, which may partly explain the differences in the observed prevalence.…”

“…However, the occurrence of multiple TTP episodes was never found to be associated with neurocognitive, emotional or HrQoL assessments in any previous study. 3,17,18,24 Fourth, the tests performed to evaluate clinical anxiety and depression, despite being well-validated tools, can only be suggestive of a psychiatric diagnosis, which indeed can only be confirmed by a proper psychiatric interview. Fifth, despite the relatively large sample size for a rare disease, numbers in some analyses are small, leading to statistical uncertainty and wide confidence intervals.…”

Recent advances in understanding and management of acquired thrombocytopenia

“…Patients could commonly have elevated cardiac troponin without any clinical cardiac abnormalities 53 . Similarly, long-term neurocognitive defects are recognized in patients with TTP 55 , 56 .…”