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Long-term neuropsychological sequelae in TTP
2.02--0.96) memory, respectively (Table 2). Similarly, lower scores in TTP patients were observed in the Rey List tests for both direct (mean difference -5.87; 95% CI: 8.57- -3.17) and deferred memory (mean difference -1.67; 95% CI: -2.32--1.02).
With regards to the attention domain, TTP patients were slower in performing the trail making B test (sus- tained and divided attention) in comparison with the gen- eral population (mean difference 65.09 seconds; 95% CI: 47.23-82.94). Conversely, patients were slightly faster in performing the trail making A test, which measures focused attention (mean difference -10.63 seconds; 95% CI: 15.81--5.44).
When we analyzed scores of neurocognitive assess- ments in patients with and without neurological signs and symptoms at presentation of the first acute TTP episode, we observed a higher degree of impairment in the memory domains of the first group of patients in 3 of 4 memory tests (digit span [direct]: mean difference -0.78; 95% CI: -1.54--0.02; digit span [backward]: mean differ- ence 0.90; 95% CI: -1.96--0.17]; Rey word list [deferred]: mean difference -1.39; 95% CI: -2.65--0.13) (Table 3).
No differences in neuropsychological assessments were found between patients with ADAMTS13 activity levels during remission below and above 45% (Table 4).
Results of emotional assessment
TTP patients presented a mean level of anxiety with the HAM-A of 9.6 (SD=8.1) and a mean level of depres- sion with the HAM-D of 7.4 (SD=5.7). The presence of clinical anxiety (HAM-A score >13) was detected in seven (20%) of interviewed patients, while the presence of clin- ical depression (HAM-D score >7) was present in 15 (43%) of them. All seven patients with clinical anxiety presented concomitant clinical depression. Five (14%) patients showed a severe anxiety (HAM-A score >24) and five (14%) a medium level of depression (HAM-D score >18). No patients presented severe levels of depression (HAM-D score >24).
Among the type of disturbances, we found that the most impaired domains in HAM-D were “work activi- ties” (n=10, 77%), “depressed mood” (n=8, 60%) and “early insomnia” (n=4, 27%) while in HAM-A there were “intellectual symptoms” (i.e. difficulty in concentration and poor memory) (n=6 82%) and “tension” (n=4, 54%).
At correlation analysis, better wellbeing was associated with better memory function (the sign of the correlation coefficient is negative because of the opposite interpreta- tion scale of the two measurements): HAM-A test versus: digit span direct Spearman rho -0.472, P=0.004; digit span backward Spearman rho -0.597, P<0.001; Rey list direct Spearman rho 0.310, P=0.075; Rey list recall: Spearman rho -0.432, P=0.011; HAM-D test versus: digit span direct Spearman rho -0.474, P=0.004; digit span backward Spearman rho -0.594, P<0.001; Rey list direct Spearman rho 0.357, P=0.038; Rey list recall: Spearman rho -0.499, P=0.003.
Results of HrQoL assessment
Table 5 displays the mean scores of the SF-36 assess- ments for each of the eight domains by physical and men- tal components: physical activity (PA), role physically (RP), bodily pain (BP), general health (GH), vitality (VI), social functioning (SF), role emotional (ER) and mental health (MH). Acquired TTP patients showed lower nor-
Table 1. Demographic and clinical characteristics of 35 thrombotic thrombocytopenic purpura patients included in the study. Clinical and laboratory data pertain the first acute thrombotic thrombocytopenic purpura episode.
Characteristics
Demographic data
Male, n (%)
Caucasian, n (%)
Age at TTP onset, years, median (IQR) Age at neuropsychological evaluation,
years, median (IQR)* Mean school level, years Job status – workers, n (%)
Clinical characteristics at the first acute TTP episode Neurological involvement, n (%)
Platelet count, x 109/L, median (IQR)†
Hemoglobin, g/dL, median (IQR)†
Number of PEX to attain remission, median (IQR)†
Laboratory parameters close to the
neuropsychological evaluation†
Platelet count, x 109/L, median (IQR)
Hemoglobin, g/dL, median (IQR)
ADAMTS13 activity close to neuropsychological evaluation†
Normal (45-138%), n (%)
Moderately reduced (10-45%), n (%)
Severely reduced (<10%), n (%)
TTP patients (n=35)
8 (23) 34 (97) 41 (35-48)
45 (39-55) 13
30 (77)
22 (63) 13 (8-27) 7.8 (6.8-10.0) 11 (6-20)
251 (212-297)
13.4 (12.8-14.3)
16 (47)
12 (35)
6 (18)
At the time of neuropsychological evaluation, 10 (29%) of 35 patients had suffered from recurrent TTP bouts.*Neuropsychological evaluation was performed at a medi- an time of 36 months (IQR: 17-54) from the last acute TTP event.†Available in 33 (platelet count, hemoglobin and number of PEX to remission at first acute TTP episode),34 (ADAMTS13 activity close to neuropsychological evaluation) and 31 sub- jects (platelet count and hemoglobin close to the neuropsychological evaluation). IQR: interquartile range; PEX: plasma exchange; TTP: thrombotic thrombocytopenic purpura
malized scores than the Italian reference sample14 in all scales but physical activity. With regard to the physical components, the most impacted area was the physical role, with a mean score of 57 (median 55; range: 40-85) and 11 patients (31%) with scores below 50. With regard to the mental components, emotional role was the most compromised, with a mean score of 43 (median 43; range: 30-56) and 22 patients (63%) with scores below 50. Overall, the mental dimension was more affected than the physical dimension, with the mental component score MCS-36 (equivalent to the sum of MH, ER, SF and VI scores) lower than the physical component score PCS- 36 (equivalent to the sum of PA, RP, BP and GH scores) by almost 60 points (mean difference -58.43; 95% CI: -71.49- -45.37) and 15 (43% of patients pertaining to the MCS- 36) versus four (11% of patients pertaining to the PCS-36) patients with scores below 50, the commonly accepted cut-off for an acceptable quality of life.15,16
Finally, at correlation analysis, the better the mental component score of the HrQoL survey was, the better were the results of emotional wellbeing assessments, especially the anxiety evaluation (MCS-36 vs. HAM-A test: Spearman rho -0.358, P=0.035; MCS-36 vs. HAM-D test: Spearman rho -0.316, P=0.064).
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