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Thalassemia intermedia

MedGen UID:
82892
•Concept ID:
C0271979
•
Disease or Syndrome
Synonyms: Intermedia, Thalassemia; Intermedias, Thalassemia; Thalassemia Intermedia; Thalassemia Intermedias
SNOMED CT: Thalassemia intermedia (934007)
 
OMIM®: 141900

Professional guidelines

PubMed

Clinical Classification, Screening and Diagnosis for Thalassemia.
Viprakasit V, Ekwattanakit S
Hematol Oncol Clin North Am 2018 Apr;32(2):193-211. doi: 10.1016/j.hoc.2017.11.006. PMID: 29458726
Non-transfusion-dependent thalassemia and thalassemia intermedia: epidemiology, complications, and management.
Vichinsky E
Curr Med Res Opin 2016;32(1):191-204. Epub 2015 Nov 25 doi: 10.1185/03007995.2015.1110128. PMID: 26479125
Guidelines for diagnosis and management of Beta-thalassemia intermedia.
Karimi M, Cohan N, De Sanctis V, Mallat NS, Taher A
Pediatr Hematol Oncol 2014 Oct;31(7):583-96. doi: 10.3109/08880018.2014.937884. PMID: 25247665

Recent clinical studies

Etiology

The hemoglobinopathies, molecular disease mechanisms and diagnostics.
Harteveld CL, Achour A, Arkesteijn SJG, Ter Huurne J, Verschuren M, Bhagwandien-Bisoen S, Schaap R, Vijfhuizen L, El Idrissi H, Koopmann TT
Int J Lab Hematol 2022 Sep;44 Suppl 1(Suppl 1):28-36. doi: 10.1111/ijlh.13885. PMID: 36074711Free PMC Article
Alpha- and Beta-thalassemia: Rapid Evidence Review.
Baird DC, Batten SH, Sparks SK
Am Fam Physician 2022 Mar 1;105(3):272-280. PMID: 35289581
Beta-thalassemia.
Galanello R, Origa R
Orphanet J Rare Dis 2010 May 21;5:11. doi: 10.1186/1750-1172-5-11. PMID: 20492708Free PMC Article
Beta-thalassemia.
Cao A, Galanello R
Genet Med 2010 Feb;12(2):61-76. doi: 10.1097/GIM.0b013e3181cd68ed. PMID: 20098328
Alpha and beta thalassemia.
Muncie HL Jr, Campbell J
Am Fam Physician 2009 Aug 15;80(4):339-44. PMID: 19678601

Diagnosis

The hemoglobinopathies, molecular disease mechanisms and diagnostics.
Harteveld CL, Achour A, Arkesteijn SJG, Ter Huurne J, Verschuren M, Bhagwandien-Bisoen S, Schaap R, Vijfhuizen L, El Idrissi H, Koopmann TT
Int J Lab Hematol 2022 Sep;44 Suppl 1(Suppl 1):28-36. doi: 10.1111/ijlh.13885. PMID: 36074711Free PMC Article
Clinical Classification, Screening and Diagnosis for Thalassemia.
Viprakasit V, Ekwattanakit S
Hematol Oncol Clin North Am 2018 Apr;32(2):193-211. doi: 10.1016/j.hoc.2017.11.006. PMID: 29458726
β-Thalassemia.
Origa R
Genet Med 2017 Jun;19(6):609-619. Epub 2016 Nov 3 doi: 10.1038/gim.2016.173. PMID: 27811859
Beta-thalassemia.
Galanello R, Origa R
Orphanet J Rare Dis 2010 May 21;5:11. doi: 10.1186/1750-1172-5-11. PMID: 20492708Free PMC Article
Alpha and beta thalassemia.
Muncie HL Jr, Campbell J
Am Fam Physician 2009 Aug 15;80(4):339-44. PMID: 19678601

Therapy

Non-Transfusion-Dependent Thalassemia: A Panoramic Review.
Shash H
Medicina (Kaunas) 2022 Oct 21;58(10) doi: 10.3390/medicina58101496. PMID: 36295656Free PMC Article
Thalassemia Intermedia: Chelator or Not?
Lee YC, Yen CT, Lee YL, Chen RJ
Int J Mol Sci 2022 Sep 5;23(17) doi: 10.3390/ijms231710189. PMID: 36077584Free PMC Article
Non-transfusion-dependent thalassemia and thalassemia intermedia: epidemiology, complications, and management.
Vichinsky E
Curr Med Res Opin 2016;32(1):191-204. Epub 2015 Nov 25 doi: 10.1185/03007995.2015.1110128. PMID: 26479125
Transfusion therapy and alloimmunization in Thalassemia Intermedia: a 10 year experience at a tertiary care university hospital.
Al-Riyami AZ, Al-Mahrooqi S, Al-Hinai S, Al-Hosni S, Al-Madhani A, Daar S
Transfus Apher Sci 2014 Aug;51(1):42-6. Epub 2014 Apr 21 doi: 10.1016/j.transci.2014.04.009. PMID: 24816438
Butyrate trials.
Cappellini MD, Graziadei G, Ciceri L, Comino A, Bianchi P, Pomati M, Fiorelli G
Ann N Y Acad Sci 1998 Jun 30;850:110-9. doi: 10.1111/j.1749-6632.1998.tb10468.x. PMID: 9668533

Prognosis

Epidemiology of clinically significant forms of alpha- and beta-thalassemia: A global map of evidence and gaps.
Musallam KM, Lombard L, Kistler KD, Arregui M, Gilroy KS, Chamberlain C, Zagadailov E, Ruiz K, Taher AT
Am J Hematol 2023 Sep;98(9):1436-1451. Epub 2023 Jun 26 doi: 10.1002/ajh.27006. PMID: 37357829
How I treat thalassemia.
Rachmilewitz EA, Giardina PJ
Blood 2011 Sep 29;118(13):3479-88. Epub 2011 Aug 2 doi: 10.1182/blood-2010-08-300335. PMID: 21813448
Beta-thalassemia.
Galanello R, Origa R
Orphanet J Rare Dis 2010 May 21;5:11. doi: 10.1186/1750-1172-5-11. PMID: 20492708Free PMC Article
Unstable and thalassemic alpha chain hemoglobin variants: a cause of Hb H disease and thalassemia intermedia.
Wajcman H, Traeger-Synodinos J, Papassotiriou I, Giordano PC, Harteveld CL, Baudin-Creuza V, Old J
Hemoglobin 2008;32(4):327-49. doi: 10.1080/03630260802173833. PMID: 18654884
Thalassemia.
Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E
Hematology Am Soc Hematol Educ Program 2004:14-34. doi: 10.1182/asheducation-2004.1.14. PMID: 15561674

Clinical prediction guides

Epidemiology of clinically significant forms of alpha- and beta-thalassemia: A global map of evidence and gaps.
Musallam KM, Lombard L, Kistler KD, Arregui M, Gilroy KS, Chamberlain C, Zagadailov E, Ruiz K, Taher AT
Am J Hematol 2023 Sep;98(9):1436-1451. Epub 2023 Jun 26 doi: 10.1002/ajh.27006. PMID: 37357829
Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia.
Haidar R, Mhaidli H, Taher AT
Eur Spine J 2010 Jun;19(6):871-8. Epub 2010 Mar 5 doi: 10.1007/s00586-010-1357-2. PMID: 20204423Free PMC Article
Unstable and thalassemic alpha chain hemoglobin variants: a cause of Hb H disease and thalassemia intermedia.
Wajcman H, Traeger-Synodinos J, Papassotiriou I, Giordano PC, Harteveld CL, Baudin-Creuza V, Old J
Hemoglobin 2008;32(4):327-49. doi: 10.1080/03630260802173833. PMID: 18654884
Thalassemia intermedia: revisited.
Taher A, Isma'eel H, Cappellini MD
Blood Cells Mol Dis 2006 Jul-Aug;37(1):12-20. Epub 2006 Jun 5 doi: 10.1016/j.bcmd.2006.04.005. PMID: 16737833
Beta-thalassemia in Yugoslavia.
Dimovski A, Efremov DG, Jankovic L, Juricic D, Zisovski N, Stojanovski N, Nikolov N, Petkov GT, Reese AL, Stoming TA
Hemoglobin 1990;14(1):15-24. doi: 10.3109/03630269009002251. PMID: 2200761

Recent systematic reviews

Serum lipid profile abnormalities among beta-thalassemia patients: a systematic review and meta-analysis.
Mohamed SOO, Mohamed AEA, Salih MSK, Salih KSK, Abdelrahman ASEE, Abdelgadir AGA, Ahmedkaroum MGA, Abdalla GA, Fadil HAM, Abdelrahman MAM, Salih NSA
Lipids Health Dis 2024 Nov 25;23(1):388. doi: 10.1186/s12944-024-02377-6. PMID: 39587544Free PMC Article
Pregnancy outcomes and iron status in β-thalassemia major and intermedia: a systematic review and meta-analysis.
Vlachodimitropoulou E, Mogharbel H, Kuo KHM, Hwang M, Ward R, Shehata N, Malinowski AK
Blood Adv 2024 Feb 13;8(3):746-757. doi: 10.1182/bloodadvances.2023011636. PMID: 38181780Free PMC Article
Epidemiology of clinically significant forms of alpha- and beta-thalassemia: A global map of evidence and gaps.
Musallam KM, Lombard L, Kistler KD, Arregui M, Gilroy KS, Chamberlain C, Zagadailov E, Ruiz K, Taher AT
Am J Hematol 2023 Sep;98(9):1436-1451. Epub 2023 Jun 26 doi: 10.1002/ajh.27006. PMID: 37357829
Hydroxyurea for nontransfusion-dependent β-thalassemia: A systematic review and meta-analysis.
Algiraigri AH, Wright NAM, Paolucci EO, Kassam A
Hematol Oncol Stem Cell Ther 2017 Sep;10(3):116-125. Epub 2017 Apr 6 doi: 10.1016/j.hemonc.2017.02.002. PMID: 28408107
Thalassemia trait and arterial thromboembolic events: a systematic review and a meta-analysis of the literature.
Dentali F, Romualdi E, Ageno W, Cappellini MD, Mannucci PM
J Thromb Haemost 2011 May;9(5):917-21. doi: 10.1111/j.1538-7836.2011.04253.x. PMID: 21382170

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