Title |
β-Thalassemia intermedia: a comprehensive overview and novel approaches
|
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Published in |
International Journal of Hematology, January 2018
|
DOI | 10.1007/s12185-018-2411-9 |
Pubmed ID | |
Authors |
Chingiz Asadov, Zohra Alimirzoeva, Tahira Mammadova, Gunay Aliyeva, Shahla Gafarova, Jeyhun Mammadov |
Abstract |
β-Thalassemia intermedia is a clinical condition of intermediate gravity between β-thalassemia minor, the asymptomatic carrier, and β-thalassemia major, the transfusion-dependent severe anemia. It is characterized by a significant clinical polymorphism, which is attributable to its genetic heterogeneity. Ineffective erythropoiesis, chronic anemia, and iron overload contribute to the clinical complications of thalassemia intermedia through stepwise pathophysiological mechanisms. These complications, including splenomegaly, extramedullary erythropoiesis, iron accumulation, leg ulcers, thrombophilia, and bone abnormalities can be managed via fetal hemoglobin induction, occasional transfusions, chelation, and in some cases, stem cell transplantation. Given its clinical diversity, thalassemia intermedia patients require tailored approaches to therapy. Here we present an overview and novel approaches to the genetic basis, pathophysiological mechanisms, clinical complications, and optimal management of thalassemia intermedia. |
X Demographics
Geographical breakdown
Country | Count | As % |
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Unknown | 1 | 100% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 1 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
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Unknown | 150 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
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Student > Master | 16 | 11% |
Student > Bachelor | 15 | 10% |
Student > Ph. D. Student | 9 | 6% |
Other | 8 | 5% |
Student > Doctoral Student | 7 | 5% |
Other | 19 | 13% |
Unknown | 76 | 51% |
Readers by discipline | Count | As % |
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Medicine and Dentistry | 38 | 25% |
Nursing and Health Professions | 8 | 5% |
Agricultural and Biological Sciences | 7 | 5% |
Biochemistry, Genetics and Molecular Biology | 5 | 3% |
Unspecified | 5 | 3% |
Other | 13 | 9% |
Unknown | 74 | 49% |