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Background. Thalassemia is a chronic disease that can lead to an impact on psychological functioning and social behavior of patients. However, still little is known about the specific psychological aspects of the disease, such as the degree of tension, life satisfaction and affective control, especially in adult patients. Aim. The purpose of this study is to investigate whether patients with thalassemia have specific psychological pattern relating to the dimensions of tension, satisfaction and quality of life, management of affection. Method. We evaluated 31 patients with thalassemia major and intermedia (19 women and 12 men) aged between 18 and 50 years (M = 34 + 16), belonging to the Complex Unit of Medical Genetics. For the evaluation were used the Profile of Mood States (POMS), the Quality of Life Enjoyment and Satisfaction Questionnaire (Q-LES-Q) and the Rorschach test. Results. The findings show an inverse relationship between the levels of self-reported tension and the affective control indicators at Rorschach. Life satisfaction, instead, seems to vary according to the severity of the disease - major vs. intermediate - and the type of therapy. Conclusions. An understanding of the psychological mechanisms involved in thalassemia, both self-reported and projective, can contribute to a wider patient takeover, by considering the subjective aspects related to the psychological and socioemotional well-being, fundamental in the care compliance.

Background and Objective: Patients with thalassaemia major (TM) and sickle cell disease (SCD) in Spain have been counted since the creation of the Spanish registry of haemoglobinopathies (REHem). The objective of this paper is to update the published data after the increase in cases due to the inclusion of adults and introduction of new-born screening in almost the whole country., Material and Methods: An observational, descriptive, multicentre and ambispective study that included patients with haemoglobinopathies registered in the REHem, started in January 2014 and followed up annually. The data presented correspond until December 31, 2017., Results: Nine hundred and fifty-nine patients were collected. There were 75 cases of thalassaemia (62 TM), 826 of ECF and 58 of other types of haemoglobinopathies. The main diagnostic reason in the TM cohort was anaemia symptoms (70.6%), with a mean age at diagnosis of .7 years; in the SCD cohort it was neonatal screening (33.1%), with a mean age at diagnosis of 2.7 years; 26 patients with TM (41.9%) and 30 with SCD (3.6%) underwent a transplant. There were 2 deaths (3.2%) with TM and 19 (2.3%) with SCD. Overall survival was 96.7% in the TM and 97.5% in the SCD cases at 15 years., Conclusions: Since the previous publication and after the diffusion of new-born screening, the most frequent diagnostic method, to the majority of autonomous regions, and the inclusion of adult patients to the registry, the REHem has increased by more than 240 cases, reaching a total of 959 records., (Copyright © 2019. Published by Elsevier España, S.L.U.)

Background and Objectives: A recently occurring increase of the prevalence of haemoglobinopathies, β-thalassaemia major (TM) and sickle cell disease (SCD) over the last two decades in our country has generated new needs in terms of medical resources for both prevention and treatment of these patients. Allogeneic haematopoietic stem cell transplant (allo-HSCT) is a curative treatment available for patients who have severe haemoglobinopathies. The main objective of this study was to evaluate the results of allo-HSCT in paediatric patients with TM or SCD performed in paediatric hematopoietic transplant units within the Spanish Group of Bone Marrow Transplantation in Children (GETMON)., Material and Methods: Retrospective review of patients undergoing HSCT in the GETMON units until 2015., Results: A total of 65 patients were analysed (43 patients were affected with TM and 22 with SCD), who received allo-HSCT in 6 GETMON units between November 1989 and December 2014. Event-free survival three years post-transplant was 81% and overall survival 92% in patients with TM. Event-free survival three years post-transplant was 79% and overall survival 85% in patients with SCD., Conclusions: The results of this series are comparable to the results of other international series and offer a platform from which to continue trying to improve the evolution of these patients., (Copyright © 2018 Elsevier España, S.L.U. All rights reserved.)