3
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Review of disease-related complications and management in adult patients with thalassemia: A multi-center study in Thailand

      research-article

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Disease-related complications and management are different among patients with thalassemia. This study was aimed to review the prevalence, clinical risk factors for the complications and the management in patients with thalassemia in Thailand. A multicenter cross-sectional study was conducted in patients with thalassemia aged ≥ 18 years old. Thalassemia-related complications and management were reviewed. The clinical parameters significantly associated with the complications were analyzed by logistic regression methods. The prevalence of thalassemia-related complications was 100% in patients with transfusion-dependent thalassemia (TDT) and 58.8% in patients with non-transfusion-dependent thalassemia (NTDT). Advanced age was statistically associated with extramedullary hematopoiesis in both TDT and NTDT patients. Splenectomy was a significant risk factor for pulmonary hypertension in both groups of patients. Severe iron overload started earlier in patients with TDT than NTDT and was associated with diabetes mellitus (adjusted odds ratio (AOR) = 6.2, p-value = 0.02). Disease-related complications are more prevalent in patients with TDT than patients with NTDT. Splenectomy and advanced age were important risk factors for developing major complications in both groups. Early screening and management for specific disease-related complications should be considered in patients with thalassemia according to their clinical risk factors.

          Related collections

          Most cited references23

          • Record: found
          • Abstract: not found
          • Article: not found

          The epidemiology of heart failure: The Framingham Study

            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Phenotype-genotype relationships in monogenic disease: lessons from the thalassaemias.

            The remarkable phenotypic diversity of the beta-thalassaemias reflects the heterogeneity of mutations at the beta-globin locus, the action of many secondary and tertiary modifiers, and a wide range of environmental factors. It is likely that phenotype-genotype relationships will be equally complex in the case of many monogenic diseases. These findings highlight the problems that might be encountered in defining the relationship between the genome and the environment in multifactorial disorders, in which the degree of heritability might be relatively low and several environmental agents are involved. They also emphasize the value of an understanding of phenotype-genotype relationships in designing approaches to gene therapy.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia.

              Ineffective erythropoiesis in patients with thalassemia intermedia drives extramedullary hematopoietic tumor formation in several parts of the body. Paraspinal involvement has received increasing attention due to the associated morbidity secondary to spinal cord compression. Although the history and physical examination may help narrow the differential diagnosis, radiographic imaging remains essential to confirm the existence of hematopoietic tissue. Characteristic appearance has been observed mainly on magnetic resonance imaging. Several treatment options have been described, including transfusion therapy, laminectomy, radiotherapy, and the use of fetal hemoglobin inducing agents that decrease the hematopoietic drive. However, the ideal management scheme remains controversial. Until large prospective trials evaluate the efficacy and safety of the available treatment options, both in single and in combination therapy, an individualized approach should be entertained.
                Bookmark

                Author and article information

                Contributors
                Role: ConceptualizationRole: Data curationRole: Formal analysisRole: Funding acquisitionRole: InvestigationRole: MethodologyRole: Project administrationRole: SupervisionRole: Writing – review & editing
                Role: ConceptualizationRole: Data curationRole: Formal analysisRole: InvestigationRole: MethodologyRole: Project administrationRole: ValidationRole: Writing – original draftRole: Writing – review & editing
                Role: ConceptualizationRole: Data curationRole: InvestigationRole: ResourcesRole: Validation
                Role: ConceptualizationRole: Data curationRole: InvestigationRole: ResourcesRole: Validation
                Role: Editor
                Journal
                PLoS One
                PLoS ONE
                plos
                plosone
                PLoS ONE
                Public Library of Science (San Francisco, CA USA )
                1932-6203
                20 March 2019
                2019
                : 14
                : 3
                : e0214148
                Affiliations
                [1 ] Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
                [2 ] Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Srinagarind Hospital, Khon Kaen University, Khon Kaen, Thailand
                [3 ] Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand
                [4 ] Hematology Unit, Chiang Rai Regional Hospital, Chiang Rai, Thailand
                National Institutes of Health, National institute of Diabetes and Digestive and Kidney Diseases, UNITED STATES
                Author notes

                Competing Interests: No Novartis or any pharmaceutical company products nor honoraria were used to conduct any of the research as part of this publication. Chuncharunee S has received research funding and honoraria from Novartis Pharmaceuticals, Janssen-Cilag, Roche, and Celgene. Teawtrakul N received honoraria from Novartis and Janssen-Cilag. Siritanaratkul N. received research funding and honoraria from Novartis, Janssen-Cilag, Roche, and Pfizer. The remaining author declares no competing financial interests. There are no patents, products in development or marketed products to declare. This does not alter our adherence to the PLOS ONE policies on sharing data and materials.

                Author information
                http://orcid.org/0000-0002-2364-1498
                Article
                PONE-D-18-32738
                10.1371/journal.pone.0214148
                6426207
                30893381
                16133d00-cf34-49a5-8e57-52efe8a91a39
                © 2019 Chuncharunee et al

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 14 November 2018
                : 7 March 2019
                Page count
                Figures: 3, Tables: 3, Pages: 13
                Funding
                This study received grants support from the Novartis Thailand.The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
                Categories
                Research Article
                Medicine and Health Sciences
                Hematology
                Hemoglobinopathies
                Thalassemia
                Medicine and Health Sciences
                Clinical Genetics
                Genetic Diseases
                Autosomal Recessive Diseases
                Thalassemia
                Medicine and Health Sciences
                Surgical and Invasive Medical Procedures
                Blood and Lymphatic System Procedures
                Splenectomy
                Medicine and Health Sciences
                Epidemiology
                Medical Risk Factors
                Biology and Life Sciences
                Biochemistry
                Proteins
                Hemoglobin
                Biology and Life Sciences
                Biochemistry
                Proteins
                Protein Complexes
                Ferritin
                Medicine and Health Sciences
                Pulmonology
                Pulmonary Hypertension
                Medicine and Health Sciences
                Diagnostic Medicine
                Clinical Laboratory Sciences
                Transfusion Medicine
                Blood Transfusion
                Medicine and Health Sciences
                Hematology
                Transfusion Medicine
                Blood Transfusion
                Biology and Life Sciences
                Physiology
                Physiological Processes
                Hematopoiesis
                Medicine and Health Sciences
                Physiology
                Physiological Processes
                Hematopoiesis
                Custom metadata
                All relevant data is within the paper and its Supporting Information files.

                Uncategorized
                Uncategorized

                Comments

                Comment on this article