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S. Riva et al.
tion of neurological and cognitive functions abnormalities observed in TTP patients in the remission phase and far from the clinical manifestations of acute TTP were not supported by instrumental neuro-functional analysis such as functional magnetic resonance imaging, which might be needed to assess the aforementioned alterations at the organic level. Third, we included patients with a history of a single acute TTP event but also patients with relapsing TTP, rendering our study population heterogeneous (in order to exclude potential comorbidities only primary TTP patients should be evaluated). However, the occur- rence 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 con- firmed 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 CI. This is a problem of any study in the field of rare diseases, and stimulate more efforts to promote col- laboration between centers. Finally, the design of our study is cross-sectional and, thus cannot provide risk esti- mates and data about predictive factors. On the other hand, as far as we know, our study is the most compre- hensive investigation on TTP patients, including clinical, cognitive, emotional and HrQoL assessment.
In conclusion, we demonstrated that, despite successful treatment with plasma exchange and immunosuppressive therapy in the acute stage of the disease, patients with TTP suffer from long term neurological sequelae even years after the acute phase. TTP patients with neurologi- cal involvement at the first acute episode seem to be at higher risk of developing memory dysfunction. Furthermore, TTP patients have increased levels of anxi- ety and depression, which negatively affect their quality of life. A faster improvement of the acute state of the dis- ease by novel drugs might have a role in this process and further studies are required to solve this question.
References
1. Deford CC, Reese JA, Schwartz LH, et al. Multiple major morbidities and increased mortality during long-term follow-up after recovery from thrombotic thrombocy- topenic purpura. Blood. 2013;122(12):2023-2029.
2. Lewis QF, Lanneau MS, Mathias SD, et al. Long-term deficits in health-related quality of life following recovery from thrombotic thrombocytopenic purpura. Transfusion. 2009;49(1):118-124.
3. Kennedy KA, Lewis QF, Scott JG, et al. Cognitive deficits after recovery from thrombotic thrombo- cytopenic purpura. Transfusion. 2009;49(6):1092-1101.
4. Ware Jr, JE, Sherbourne CD. The MOS 36- item short-form health survey (SF-36): I. Conceptual framework and item selection. Med Care. 1992;30(6):473-483.
5. Rabin R, de Charro F. EQ-SD: a measure of health status from the EuroQol Group. Ann Med. 2001;35(5):337-343.
6. Wechsler S. The Semantic Basis of Argument Structure. New York, NY: CSLI Publications (Center for the Study of Language and Information). 1995, Stanford, Cambridge University Press.
7. Rey A. L’Examen clinique en psychologie. (The Clinical Exam in Psychology), 1964, Paris: Presses Universitaires de France.
8. Tombaugh TN. Trail Making Test A and B: normative data stratified by age and educa- tion. Arch Clin Neuropsychol. 2004; 19(2):203-214.
9. Hamilton M. A rating scale for depression. J Neurol Neurosurg Psychiatry. 1960;23:56- 65.
10. Hamilton M. The assessment of anxiety states by rating. Br J Med Psychol. 1959;32:50-55.
11. Mondini S, Mapelli D, Vestri A, et al. Esame neuropsicologico breve. Milano: Raffaello Cortina Editore. 2003;160.
12. Carlesimo GA, Caltagirone C, Gainotti GU, et al. The mental deterioration bat- tery: normative data, diagnostic reliability and qualitative analyses of cognitive impairment. Eur Neurol. 1996;36(6):378- 384.
13. Giovagnoli AR, Del Pesce M, Mascheroni S, et al. Trail making test: normative values from 287 normal adult controls. Ital J Neurol Sci. 1996;17(4):305-309.
14. Apolone G, Mosconi P. The Italian SF-36 Health Survey: translation, validation and norming. J Clin Epidemiol. 1998; 51(11):1025-1036.
15. Ware JE, Kosinski M, Gandek B, et al. The factor structure of the SF-36 Health Survey in 10 countries: Results from the IQOLA Project. J Clinic epidemiol. 1998; 51(11):1159-1165.
90(8):709-714.
18. Falter T, Schmitt V, Herold S, et al.
Depression and cognitive deficits as long- term consequences of thrombotic throm- bocytopenic purpura. Transfusion. 2017; 57(5):1152-1162.
19. Chaturvedi S, Oluwole O, Cataland S, et al. Post-traumatic stress disorder and depression in survivors of thrombotic thrombocytopenic purpura. Thromb Res. 2017;151:51-56.
20. Saczynski JS, McManus DD, Waring ME, et al. Change in cognitive function in the month after hospitalization for acute coro- nary syndromes: findings From TRACE- CORE (transition, risks, and actions in coronary events–center for outcomes research and education). Circ Cardiovasc Qual Outcomes. 2017;10(12).
21. Lisabeth LD, Sánchez BN, Baek J, et al. Neurological, functional, and cognitive stroke outcomes in Mexican Americans. Stroke. 2014;45(4):1096-1101.
22. van Rooij FG, Kessels RP, Richard E, et al. Cognitive impairment in transient ischemic attack patients: a systematic review. Cerebrovasc Dis. 2016;42(1-2):1-9.
23. Mitchell AJ, Sheth B, Gill J, et al. Prevalence and predictors of post-stroke mood disor- ders: A meta-analysis and meta-regression of depression, anxiety and adjustment dis- order. Gen Hosp Psychiatry. 2017;47:48-
16. Obidoa CA, Reisine SL, Cherniack M.
How Does the SF-36 Perform in Healthy Populations? A Structured Review of Longitudinal Studies. J Soc Behav Health. 2010;4(1):30-48. 60.
17. Han B, Page EE, Stewart LM, et al. Depression and cognitive impairment fol- lowing recovery from thrombotic throm- bocytopenic purpura. Am J Hematol. 2015;
24. Cataland SR, Scully MA, Paskavitz J, et al. Evidence of persistent neurologic injury following thrombotic thrombocytopenic purpura. Am J Hematol. 2011;86(1):87-89.
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