Beta-thalassemia natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maryam Hadipour, M.D.[2]
Overview
Without regular blood transfusions, affected individuals by major beta-thalassemia typically develop severe anemia and related complications such as pulmonary hypertension, right heart failure, iron overload early in life, while beta-thalassemia intermedia patients would have a variable clinical course, complications, and prognosis. Beta-thalassemia minor subjects would not have significant symptoms, while in some cases might have an increased risk for iron deficiency anemia. Iron overload complications happen in transfusion-dependent thalassemia.
Natural history
- Untreated beta-thalassemia can result in significant morbidity, mortality, and diminished quality of life. Patients with non-transfusion-dependent beta-thalassemia (NTD) can develop a wide range of complications in critical organ systems due to ineffective erythropoiesis, hemolysis, and primary iron overload. NTD patients can progress to transfusion-dependent beta-thalassemia (TD) later in life, which further exacerbates the risk of complications and iron overload[1].
- Untreated thalassemia major patients are at a high risk of developing pulmonary hypertension and right heart failure. The prevalence of these conditions in beta-thalassemia patients depends on the severity of the disease and the adequacy of treatment. Thalassemia major patients who do not receive treatment almost universally develop pulmonary hypertension. On the other hand, thalassemia intermedia patients are more likely to develop pulmonary hypertension compared to treated thalassemia major patients[2].
- Untreated beta-thalassemia major patients may also experience irreversible bone and organ damage due to iron overload[3].
Complications
Prognosis
References
- ↑ Tang CH, Furnback W, Wang B, Tang J, Tang D, Lu MY, Huang VW, Musallam KM (October 2021). "Relationship between transfusion burden, healthcare resource utilization, and complications in patients with beta-thalassemia in Taiwan: A real-world analysis". Transfusion. 61 (10): 2906–2917. doi:10.1111/trf.16636. PMC 9291481 Check
|pmc=value (help). PMID 34505291 Check|pmid=value (help). Vancouver style error: initials (help) - ↑ Bloomfield GS, Lagat DK, Akwanalo OC, Carter EJ, Lugogo N, Vedanthan R, Velazquez EJ, Kimaiyo S, Sherman CB (September 2012). "Waiting to inhale: An exploratory review of conditions that may predispose to pulmonary hypertension and right heart failure in persons exposed to household air pollution in low- and middle-income countries". Glob Heart. 7 (3): 249–259. doi:10.1016/j.gheart.2012.06.015. PMC 3653331. PMID 23687634.
- ↑ Bender MA, Hulihan M, Dorley MC, Aguinaga M, Ojodu J, Yusuf C (December 2021). "Newborn Screening Practices for Beta-Thalassemia in the United States". Int J Neonatal Screen. 7 (4). doi:10.3390/ijns7040083. PMC 8703506 Check
|pmc=value (help). PMID 34940053 Check|pmid=value (help). Vancouver style error: initials (help)