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Multicenter Study
. 2019 Aug;186(4):592-607.
doi: 10.1111/bjh.15959. Epub 2019 May 20.

Brain functional impairment in beta-thalassaemia: the cognitive profile in Italian neurologically asymptomatic adult patients in comparison to the reported literature

Immacolata Tartaglione  1 , Renzo Manara  2 , Martina Caiazza  1 , Pasquale Alessandro Carafa  3 , Violetta Caserta  4 , Teresa Ferrantino  4 , Ilaria Granato  5 , Noemi Ippolito  5 , Caterina Maietta  4 , Tiziana Oliveto  5 , Maddalena Casale  1 , Rosanna Di Concilio  6 , Angela Ciancio  7 , Elisa De Michele  8 , Camilla Russo  9 , Andrea Elefante  9 , Sara Ponticorvo  2 , Andrea Gerardo Russo  2 , Gianluca Femina  2 , Antonietta Canna  2 , Mario Ermani  10 , Mario Cirillo  11 , Fabrizio Esposito  2 , Antonella Centanni  5 , Paolo Gritti  4 , Silverio Perrotta  1
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Multicenter Study

Brain functional impairment in beta-thalassaemia: the cognitive profile in Italian neurologically asymptomatic adult patients in comparison to the reported literature

Immacolata Tartaglione et al. Br J Haematol. 2019 Aug.

Abstract

Cognitive involvement in beta-thalassaemia is strikingly controversial and poorly studied in adulthood. This multicentre prospective study investigated 74 adult neurologically-asymptomatic beta-thalassaemia patients (mean-age 34·5 ± 10·3 years; 53 transfusion-dependent [TDT], 21 non-transfusion dependent [NTDT]) and 45 healthy volunteers (mean-age 33·9 ± 10·7 years). Participants underwent testing with Wechsler Adult Intelligence Scale-Fourth Edition (WAIS-IV), Brief Psychiatric Rating Scale (BPRS) and multiparametric brain 3T-magnetic resonance imaging (MRI) for parenchymal, vascular and iron content evaluation. Patients had lower Full-Scale Intelligence Quotient (FSIQ) than controls (75·5 ± 17·9 vs. 97·4 ± 18·1, P < 0·0001) even after correction for education level. Compared to TDT, NTDT showed a trend of higher FSIQ (P = 0·08) but a similar cognitive profile at WAIS-subtests. FSIQ correlated with total and indirect bilirubin (P < 0·0001 and P = 0·002, respectively); no correlation was found with splenectomy, intracranial MRI/magnetic resonance-angiography findings, brain tissue iron content or other disease-related clinical/laboratory/treatment data. FSIQ did not correlate with BPRS scores, although the latter were higher among patients (28·74 ± 3·1 vs. 27·29 ± 4·8, P = 0·01) mainly because of increased depression and anxiety levels. Occupation rate was higher among controls (84·4% vs. 64·9%, P = 0·004) and correlated with higher FSIQ (P = 0·001) and education level (P = 0·001). In conclusion, Italian adult beta-thalassaemia patients seem to present a characteristic cognitive profile impairment and an increased rate of psychological disorders with possible profound long-term socio-economic consequences.

Keywords: Wechsler Adult Intelligence Scale; beta-thalassaemia; blood transfusion; brain magnetic resonance imaging; intelligence quotient.

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