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Long-term neuropsychological sequelae in TTP
Table 4. Descriptive statistics of neuropsychological tests in acquired thrombotic thrombocytopenic purpura patients with and without ADAMTS13 deficiency next to the psychological evaluation.
Test
Memory, mean (SD)
Digit span (direct)
Digit span (backward) Rey word list (direct)* Rey word list (deferred)*
Attention, mean (SD)
Trail making A, seconds
Trail making B, seconds
ADAMTS13 activity during remission
Mean difference (95% CI)
0.56 (-0.20, 1.31) 0.11 (-0.98, 1.21) 1.13 (-4.33, 6.59) 0.72 (-0.59, 2.02)
-4.88 (-15.62, 5.87)
-11.05 (-48.09, 26.00)
<45% (n=18)
6.06 (1.00) 4.61 (1.79) 27.22 (7.72) 4.44 (1.67)
32.06 (13.39)
209.89 (58.93)
≥45% (n=16) 5.50 (1.16)
4.50 (1.27) 26.09 (7.65) 3.73 (2.02)
36.94 (17.32)
220.94 (45.19)
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,5 of 18 (28%) and 5 of 16 (31%) patients with and without ADAMTS13 deficiency next to the visit had suffered from recurrent TTP bouts, respectively. *: Available in 34 TTP patients; CI: confidence interval; SD: standard deviation.
Table 5. Descriptive statistics of health-related quality of life components in acquired thrombotic thrombocytopenic purpura patients and in Italian reference individuals.
Test
Physical domain Physical activity Limitation physical role Pain
General health
PCS-36
Mental domain
Vitality
Social activity
Limitation emotional role Mental health
MCS-36
TTP patients (n=35)
N with score<50 (%)
2 (6) 11 (31) 5 (14) 5 (14) 4 (11)*
13 (37) 7 (20) 22 (63) 4 (11) 15 (43)*
General population (n=2031) mean (SD)
84.46 (23.18) 78.21 (35.93) 73.67 (27.65) 65.22 (22.18) NA
61.89 (20.69) 77.43 (23.34) 76.16 (37.25) 66.59 (20.89) NA
Mean difference (95%CI)
-3.06 (-8.43 to 2.31) -21.44 (-26.17 to -16.72) -11.47 (-16.39 to -6.55) -5.31 (-9.38 to -1.24) NA
-9.43 (-13.93 to -4.93) -23.17 (-26.28 to -20.06) -33.47 (-36.86 to -30.08) -14.13 (-16.20 to -12.06) NA
mean (SD)
81.40 (15.37) 56.77 (13.04) 62.20 (13.92) 59.91 (11.54) 260.29 (44.63)
52.46 (12.85) 54.26 (8.60) 42.69 (8.76) 52.46 (5.45)
201.86 (23.84)
At the time of neuropsychological evaluation, 10 (29%) of 35 patients had suffered from recurrent TTP bouts. *Being PCS-36 and MCS-36 the weighted sum of the original scales of the SF-36,this number indicates patients with a score below 200.CI:confidence interval;NA:not available;SD:standard deviation;MCS-36:mental component summary (MCS) of the Short Form (36) Health Status Questionnaire (SF36);PCS-36:physical component summary (PCS) of the Short Form (36) Health Status Questionnaire (SF36);TTP:throm- botic thrombocytopenic purpura.
vascular and neurovascular diseases. High percentage of cognitive impairment were also found in patients after acute coronary syndrome (16% of patients),20 stroke (one- third of the sample examined),21 and after a transient ischemic attack (TIA) (more than a third of patients).22 After TIA, also depressive symptoms were found as prevalent as 34%.23 However, there are important differ- ences between these studies and ours. First, the evaluation of cognitive decline and depression were generally made during the acute phase and in patients older than ours.23 Second, the literature mainly highlights a major deteriora- tion at the level of functional abilities in daily life activi- ties, especially in stroke patients. Finally, different tests were performed and different cognitive domains (e.g. lan- guage) evaluated, making any comparison difficult. We also found an overall impaired quality of life compared with the general population. In the HrQoL domains, men- tal components were more impaired than physical com- ponents, suggesting that the condition determines a con- siderable emotional burden, probably related to its course of intermittent relapse and remission phases. Indeed, the
HrQoL mental domain was negatively affected by the presence of clinical anxiety and depression in our patients. Our findings are consistent with those of Lewis et al.2 and Cataland et al.24 although they reported a greater impact of acquired TTP on the physical component of the HrQoL,2 and on both the mental and the physical component.24
We believe that our findings have clinical relevance for the management of TTP patients. Clinicians should be aware of the association between neurological manifesta- tions during the acute TTP episodes and long-term impaired neuropsychological abilities. They should put attention on signs and symptoms of neurological impair- ment during the remission phase and consider early signs of anxiety and depression in order to improve the quality of life of TTP patients.
Our study has limitations. First, the neurocognitive and emotional status of the patients and their quality of life before their first episode of TTP was not objectively eval- uated. However, patients did perceive and referred at the time of the psychological consultation a worsening of their conditions after TTP diagnosis. Second, the evalua-
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