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Hemostasis & its Disorders
Long-term neuropsychological sequelae, emotional wellbeing and quality of life in patients with acquired thrombotic thrombocytopenic purpura
Ferrata Storti Foundation
Haematologica 2020 Volume 105(7):1957-1962
Silvia Riva,1,2* Ilaria Mancini,3* Alberto Maino,1,4 Barbara Ferrari,1 Andrea Artoni,1 Pasquale Agosti3 and Flora Peyvandi1,3
1Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy; 2St Mary's University, Twickenham, London, UK; 3Università degli Studi di Milano, Department of Pathophysiology and Transplantation, and Fondazione Luigi Villa, Milan, Italy and 4Azienda Provinciale per i Servizi Sanitari, Internal Medicine Unit, Trento, Italy
*SR and IM contributed equally as co-first authors.
ABSTRACT
Neurological symptoms related to microthrombosis are the hallmark of acute manifestations of acquired thrombotic thrombocytopenic purpura (TTP). Despite the achievement of hematological remission, patients may report persisting neurological impairment that affects their quality of life. To assess the long-term neuropsychological consequences of acute TTP, we recruited 35 acquired TTP patients (77% females, median age at onset 41 years, interquartile range: 35-48) regularly followed at our out- patient clinic of thrombotic microangiopathies in Milan (Italy) from December 2015 to October 2016. Patients underwent a psychological eval- uation of memory and attentional functions, emotional wellbeing and health-related quality of life at least three months after their last acute TTP event (median 36 months, interquartile range: 17-54). During the psycholog- ical consultation, 17 patients (49%) referred persisting subjective neurologi- cal impairment in the frame of a remission phase, with at least one symptom as disorientation, loss of concentration, dizziness, lack of balance, headache and diplopia. Neuropsychological assessment revealed lower scores than the Italian general population pertaining to direct, indirect and deferred memory. A higher degree of impairment of memory domains was found in patients with neurological involvement at the time of presentation of the first acute TTP episode. Anxiety and depression were detected in seven (20%) and 15 (43%) patients, respectively. Health-related quality of life was lower than the Italian general population, with mental domains more impacted than physical domains (mean difference 58.43, 95% confidence interval: 71.49- 45.37). Our study demonstrates compromised memory and attention func- tions, persisting anxiety/depression symptoms and a generally reduced qual- ity of life in patients recovering from acute acquired TTP. New clinical strate- gies should be considered to improve these symptoms.
Introduction
Thrombotic thrombocytopenic purpura (TTP) is a rare multisystem microan- giopathy with fluctuating signs and symptoms. Its mortality rate can reach 90% when patients are left untreated, but it is reduced to 10% if patients are properly treated in the first 24 hours after diagnosis. Recovery is usually complete, but with a risk of relapse and the uncommon occurrence of persistent neurologic, cardiac and renal abnormalities.1 After recovery, despite normal physical examination and laboratory data, many patients complain of difficulties with memory, headache, loss of concentration and endurance, as expressed at patient support group meet- ings. Some attempts to quantify those neurological deficits in acquired TTP patients have been made in the context of small observational studies, but without
Correspondence:
FLORA PEYVANDI
flora.peyvandi@unimi.it
Received: May 17, 2019.
Accepted: September 19, 2019. Pre-published: September 26, 2019.
doi:10.3324/haematol.2019.226423
Check the online version for the most updated information on this article, online supplements, and information on authorship & disclosures: www.haematologica.org/content/105/7/1957
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haematologica | 2020; 105(7)
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