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beta-Thalassemia (Cooley's Anemia)

A disorder characterized by reduced synthesis of the beta chains of hemoglobin. There is retardation of hemoglobin A synthesis in the heterozygous form (thalassemia minor), which is asymptomatic, while in the homozygous form (thalassemia major, Cooley's anemia, Mediterranean anemia, erythroblastic anemia), which can result in severe complications and even death, hemoglobin A synthesis is absent.
Also Known As:
Cooley's Anemia; Thalassemia Major; Erythroblastic Anemia; Mediterranean Anemia; Thalassemia Minor; Microcytemia, beta Type; Thalassemia Intermedia; Thalassemia Major (beta-Thalassemia Major); Thalassemia Minor (beta-Thalassemia Minor); Thalassemia, beta Type; beta Thalassemia; Anemia, Cooley; Anemia, Cooleys; Anemias, Erythroblastic; Anemias, Mediterranean; Disease, Hemoglobin F; Intermedia, Thalassemia; Intermedias, Thalassemia; Major, Thalassemia (beta-Thalassemia Major); Majors, Thalassemia (beta-Thalassemia Major); Mediterranean Anemias; Microcytemias, beta Type; Minor, Thalassemia (beta-Thalassemia Minor); Minors, Thalassemia (beta-Thalassemia Minor); Thalassemia Intermedias; Thalassemia Major (beta Thalassemia Major); Thalassemia Majors (beta-Thalassemia Major); Thalassemia Minor (beta Thalassemia Minor); Thalassemia Minors (beta-Thalassemia Minor); Thalassemia, beta; Thalassemias, beta; Thalassemias, beta Type; Type Microcytemia, beta; Type Microcytemias, beta; Type Thalassemia, beta; Type Thalassemias, beta; beta Thalassemias; beta Type Microcytemia; beta Type Microcytemias; beta Type Thalassemia; beta Type Thalassemias; Anemia, Cooley's; Anemia, Erythroblastic; Anemia, Mediterranean; Hemoglobin F Disease
Networked: 5503 relevant articles (235 outcomes, 817 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Iron Overload
2. Sickle Cell Anemia (Hemoglobin S Disease)
3. Anemia
4. Infections
5. Thalassemia

Experts

1. Karimi, Mehran: 57 articles (03/2022 - 01/2002)
2. Fucharoen, Suthat: 48 articles (01/2021 - 06/2002)
3. Galanello, Renzo: 45 articles (03/2017 - 03/2002)
4. De Sanctis, Vincenzo: 35 articles (01/2022 - 09/2003)
5. Cappellini, Maria Domenica: 35 articles (10/2021 - 10/2004)
6. Haghpanah, Sezaneh: 29 articles (01/2021 - 09/2009)
7. Wood, John C: 28 articles (04/2016 - 03/2004)
8. Piga, Antonio: 27 articles (12/2021 - 09/2002)
9. Rivella, Stefano: 27 articles (01/2021 - 03/2002)
10. Maggio, Aurelio: 27 articles (02/2018 - 03/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to beta-Thalassemia:
1. IronIBA
2. DeferasiroxFDA Link
3. Deferoxamine (Desferal)FDA LinkGeneric
4. Hydroxyurea (Hydrea)FDA LinkGeneric
5. Hemoglobins (Hemoglobin)IBA
6. DeferiproneIBA
7. Fetal Hemoglobin (Hemoglobin F)IBA
8. Ferritins (Ferritin)IBA
11/01/1992 - "Flow cytometric analysis of lymphocyte subsets and lymphocyte surface ferritin shows no significant difference in the number of total T-cells, B cells, NK cells, helper T-cells (CD 4), suppressor T-cells (CD 8) and CD 4/CD 8 ratio among normal subjects (n = 11) and Hb E trait (n = 6), beta-thalassemia (beta-thal) trait (n = 5), neither in normal and nonsplenectomized patients with beta-thal/Hb E (n = 10) except B cells and CD 4. There is a significant reduction in lymphocytes surface spleen-type and heart-type ferritin in patients with beta-thal/Hb E when compared to normal subjects. "
09/29/2023 - "Erythrocytapheresis in paediatric patients with β-thalassemia major was as effective in decreasing ferritin levels as in previously reported studies with SCD. "
01/01/2023 - "The objectives of this study were to compare ocular biometry and anterior segment parameters in children with thalassemia major and healthy controls and to examine the relationship between ferritin levels, anthropometric measurements, and ocular parameters. "
04/01/2022 - "Compared to controls, the level of ferritin had a very significant increase (P≤0.01) in all the study patients with beta-thalassemia, male and female. "
03/01/2022 - "The aim of this study was to evaluate electrocardiography, echocardiography according cardiac T2* and ferritin findings of patients followed-up for β-thalassemia major, and to investigate the importance of these findings for early detection of cardiac complications. "
9. GlobinsIBA
01/01/2022 - "Erythrocytes differentiated from SCD HSCs transduced with the DS demonstrated significantly reduced in vitro sickling phenotype compared to the SS. Erythrocytes differentiated from transduced HSCs from β-thalassemia major patients demonstrated improved globin chain balance by increased γ-globin with reduced microcytosis. "
01/01/2011 - "In order to evaluate the efficacy of a non-invasive prenatal diagnosis (NIPD) test to exclude α-thalassemia major that uses SNPs linked to the normal paternal α-globin allele, we established a novel protocol to reliably detect paternal SNPs within the (--(SEA)) breakpoints and performed evaluation of the diagnostic potential of the protocol in a total of 67 pregnancies, in whom plasma samples were collected prior to invasive obstetrics procedures in southern China. "
09/21/2018 - "In addition, BCL11A enhancer modification in mobilized CD34+ cells from patients with β-thalassemia major resulted in a readily detectable γ-globin increase with a preferential increase in G-gamma, leading to an improved phenotype and, likely, a survival advantage for maturing erythroid cells after editing. "
01/01/2022 - "Genetic Background Studies of Eight Common Beta Thalassemia Mutations in Thailand Using β-Globin Gene Haplotype and Phylogenetic Analysis."
04/01/2019 - "Materials and Methods: In this study, we investigated three frequent mutations (c.315+1G>A, c.93-21G>A and c.92+5G>C in β-globin gene, the frequency of RFLP haplotypes, and LD between markers at β-globin gene cluster) in 150 beta-thalassemia patients and 50 healthy individuals. "
10. Chelating AgentsIBA

Therapies and Procedures

1. Therapeutics
2. Chelation Therapy (Therapy, Chelation)
3. Hematopoietic Stem Cell Transplantation
4. Splenectomy
5. Transplantation