Page 218 - 2020_07-Haematologica-web

S. Riva et al.
Table 2. Descriptive statistics of neuropsychological tests in acquired thrombotic thrombocytopenic purpura patients and mean values of the Italian general population.
Test
Memory
Digit span (direct)
Digit span (backword) Rey word list (direct)* Rey word list (deferred)*
Attention
Trail making A, seconds
Trail making B, seconds
TTP patients Mean (SD)
5.74 (1.12) 4.51 (1.54) 26.37 (7.74) 4.03 (1.86)
34.37 (15.1)
214.09 (51.99)
General population Mean
7.00 6.00 32.20 5.70
45.00
149.00
Mean difference (95% CI)
-1.26 (-1.64, -0.87) -1.49 (-2.02, -0.96) -5.87 (-8.57, -3.17) -1.67 (-2.32, -1.02)
-10.63 (-15.81, -5.44)
65.09 (47.23, 82.94)
In memory tests a higher score indicates a better performance,in attention tests a lower score indicates a better performance.At the time of neuropsychological evaluation,10 (29%) of 35 patients had suffered from recurrent TTP bouts. *: Available in 34 TTP patients; CI: confidence interval; SD: standard deviation; TTP: thrombotic thrombocytopenic purpura.
Table 3. Descriptive statistics of neuropsychological tests in acquired thrombotic thrombocytopenic purpura (TTP) patients with and without neu- rological manifestations at onset of the first acute TTP event.
Test
Memory, mean (SD)
Digit span (direct)
Digit span (backword) Rey word list (direct)* Rey word list (deferred)*
Attention, mean (SD)
Trail making A, seconds
Trail making B, seconds
Neurological involvement at first acute TTP event
Mean difference (95% CI)
-0.78 (-1.54, -0.02) -0.90 (-1.96, 0.17) -2.54 (-8.11, 3.04) -1.39 (-2.65, -0.13)
7.57 (-3.01, 18.14)
-16.87 (-53.95, 20.20)
Present (n=22)
5.45 (1.14) 4.18 (1.59) 25.36 (7.73) 3.50 (1.92)
37.18 (16.26)
207.82 (49.91)
Absent (n=13)
6.23 (0.93) 5.08 (1.32) 27.89 (7.80) 4.89 (1.44)
29.62 (12.00)
224.69 (55.71)
In memory tests a lower score indicates a worse performance,in attention tests a higher score indicates a better performance.At the time of neuropsychological evaluation,9 of 22 (41%) and 1 of 13 (8%) patients with and without neurological involvement at the first acute TTP event had suffered from recurrent TTP bouts, respectively. *: Available in 34 TTP patients; CI: confidence interval; SD: standard deviation; TTP: thrombotic thrombocytopenic purpura.
Discussion
Neurological signs and symptoms of acute TTP are mainly transient, brief and resolve with remission of the acute phase. Our study demonstrates persisting neurolog- ical, neuropsychological, emotional and HrQoL impair- ments in TTP patients even years after the acute phase.
During the remission phase, TTP patients showed a sig- nificant impairment in memory domains (direct, back- ward and deferred memory) when compared with the general population. This memory impairment was posi- tively associated with the presence of neurological symp- toms during the acute phase of the disease, as shown by the comparison between patients with and without neu- rological involvement during the first acute TTP event. Attention domains were also affected, but they were unrelated to neurological involvement during the acute phase. 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 neuro- logical manifestations at the time of the acute TTP event,3,18 although a trend towards a worse mental per- formance was detected in German patients with neurolog-
ical symptoms compared with patients with no neurolog- ical symptoms (median of FLei mental performance score: 45 [IQR: 15-65] vs. 31 [IQR: 13-40], Mann-Whitney U test P=0.193).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% vs. 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. 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 perform- ance and the severity of depression in 84 TTP patients.
It is interesting to compare our results with other cardio-
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haematologica | 2020; 105(7)