title: Regional consensus opinion for the management of Beta thalassemia major in the Arabian Gulf area.
abstract: Thalassemia syndrome has diverse clinical presentations and a global spread that has far exceeded the classical Mediterranean basin where the mutations arose. The mutations that give rise to either alpha or beta thalassemia are numerous, resulting in a wide spectrum of clinical severity ranging from carrier state to life-threatening, inherited hemolytic anemia that requires regular blood transfusion. Beta thalassemia major constitutes a remarkable challenge to health care providers. The complications arising due to the anemia, transfusional iron overload, as well as other therapy-related complications add to the complexity of this condition. To produce this consensus opinion manuscript, a PubMed search was performed to gather evidence-based original articles, review articles, as well as published work reflecting the experience of physicians and scientists in the Arabian Gulf region in an effort to standardize the management protocol.
authors.affiliation: The Pharmaceutical Research Institute at Albany College of Pharmacy and Health Sciences, Rensselaer, New York, USA. shaker.mousa@acphs.edu.
authors.forename: Mohamad H
authors.initials: MH
authors.lastname: Qari
authors.forename: Yasser
authors.initials: Y
authors.lastname: Wali
authors.forename: Muneer H
authors.initials: MH
authors.lastname: Albagshi
authors.forename: Mohammad
authors.initials: M
authors.lastname: Alshahrani
authors.forename: Azzah
authors.initials: A
authors.lastname: Alzahrani
authors.forename: Ibrahim A
authors.initials: IA
authors.lastname: Alhijji
authors.forename: Abdulkareem
authors.initials: A
authors.lastname: Almomen
authors.forename: Abdullah
authors.initials: A
authors.lastname: Aljefri
authors.forename: Hussain H
authors.initials: HH
authors.lastname: Al Saeed
authors.forename: Shaker
authors.initials: S
authors.lastname: Abdullah
authors.forename: Ahmad
authors.initials: A
authors.lastname: Al Rustumani
authors.forename: Khoutir
authors.initials: K
authors.lastname: Mahour
authors.forename: Shaker A
authors.initials: SA
authors.lastname: Mousa
mesh_terms: D001083:Arabia / Q000453:epidemiology; D006801:Humans; D017086:beta-Thalassemia / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology*
references.citation: N Engl J Med. 1993 Sep 16;329(12):840-4
references.pmid: 8355742
references.citation: Eur Heart J. 2001 Dec;22(23):2171-9
references.pmid: 11913479
references.citation: Blood. 2002 Jan 1;99(1):36-43
references.pmid: 11756150
references.citation: Pediatr Endocrinol Rev. 2011 Mar;8 Suppl 2:290-4
references.pmid: 21705980
references.citation: J Pediatr Endocrinol Metab. 1997 Mar-Apr;10(2):175-84
references.pmid: 9364350
references.citation: Br J Haematol. 2010 Aug;150(4):486-9
references.pmid: 20456362
references.citation: Lancet Infect Dis. 2006 Apr;6(4):226-33
references.pmid: 16554247
references.citation: Genet Med. 2007 Jun;9(6):372-7
references.pmid: 17575503
references.citation: Semin Hematol. 1990 Jul;27(3):229-38
references.pmid: 2197726
references.citation: Am J Hum Genet. 1980 Jan;32(1):26-41
references.pmid: 6153864
references.citation: Blood. 2012 Jul 12;120(2):473-6
references.pmid: 22645178
references.citation: Pediatr Pulmonol. 2012 Aug;47(8):801-7
references.pmid: 22328228
references.citation: Br J Haematol. 1995 Mar;89(3):473-8
references.pmid: 7734344
references.citation: Haematologia (Budap). 1995;26(4):205-9
references.pmid: 7590515
references.citation: Lancet. 2012 Jan 28;379(9813):373-83
references.pmid: 21908035
references.citation: J Med Assoc Thai. 2012 Jan;95(1):16-21
references.pmid: 22379736
references.citation: Pediatr Endocrinol Rev. 2008 Oct;6 Suppl 1:208-13
references.pmid: 19337180
references.citation: Blood. 2003 Mar 15;101(6):2137-43
references.pmid: 12424197
references.citation: Acta Haematol. 1998;99(2):76-9
references.pmid: 9554453
references.citation: Gastroenterology. 2011 Oct;141(4):1202-11, 1211.e1-3
references.pmid: 21741344
references.citation: Pediatr Hematol Oncol. 2012 Sep;29(6):579-84
references.pmid: 22839111
references.citation: Blood. 2007 Jun 1;109(11):5027-35
references.pmid: 17299088
references.citation: Br J Haematol. 2007 Feb;136(3):353-65
references.pmid: 17129232
references.citation: Blood. 2001 Jun 1;97(11):3411-6
references.pmid: 11369631
references.citation: Ann Intern Med. 2003 Apr 15;138(8):627-33
references.pmid: 12693884
references.citation: Haematologica. 2004 Oct;89(10):1187-93
references.pmid: 15477202
references.citation: N Engl J Med. 2000 Aug 3;343(5):327-31
references.pmid: 10922422
references.citation: Osteoporos Int. 2001;12(7):570-5
references.pmid: 11527055
references.citation: Eur J Haematol. 2008 Jan;80(1):67-70
references.pmid: 18173741
references.citation: Transfus Med. 1999 Sep;9(3):227-38
references.pmid: 10555817
references.citation: Intern Med J. 2010 Oct;40(10):689-96
references.pmid: 20492011
references.citation: Int J Surg. 2009 Oct;7(5):476-81
references.pmid: 19695352
references.citation: Blood. 2006 May 1;107(9):3455-62
references.pmid: 16352812
references.citation: Pediatr Clin North Am. 2010 Feb;57(1):181-205
references.pmid: 20307718
references.citation: Orphanet J Rare Dis. 2010 May 21;5:11
references.pmid: 20492708
references.citation: Cardiovasc Hematol Agents Med Chem. 2012 Mar 1;10(1):25-30
references.pmid: 22239492
references.citation: Ann N Y Acad Sci. 2010 Aug;1202:205-13
references.pmid: 20712794
references.citation: Eur J Radiol. 2009 Jan;69(1):120-2
references.pmid: 18029131
references.citation: Bone Marrow Transplant. 2008 Sep;42(6):397-404
references.pmid: 18574445
references.citation: Br J Haematol. 2008 Jun;141(6):891-4
references.pmid: 18371108
references.citation: Sultan Qaboos Univ Med J. 2011 Nov;11(4):455-61
references.pmid: 22087393
references.citation: Hemoglobin. 2013;37(1):65-73
references.pmid: 23297837
references.citation: Saudi Med J. 2000 Dec;21(12):1168-72
references.pmid: 11360093
references.citation: Hematology. 2012 Apr;17 Suppl 1:S28-31
references.pmid: 22507773
references.citation: Horm Res. 2002;58 Suppl 1:72-9
references.pmid: 12373018
references.citation: J Cardiovasc Med (Hagerstown). 2008 May;9(5):515-25
references.pmid: 18404006
references.citation: Hemoglobin. 2011;35(5-6):636-42
references.pmid: 22035015
references.citation: Br J Haematol. 1998 Dec;103(4):897-901
references.pmid: 9886298
references.citation: N Engl J Med. 1999 Dec 23;341(26):1986-95
references.pmid: 10607817
references.citation: J Med Genet. 1986 Jun;23(3):245-51
references.pmid: 3723553
references.citation: Lancet. 2000 Jun 10;355(9220):2051-2
references.pmid: 10885361
references.citation: Ann N Y Acad Sci. 2005;1054:457-61
references.pmid: 16339697
references.citation: Blood. 2010 Oct 7;116(14):2582-9
references.pmid: 20554970
references.citation: Hemoglobin. 1995;19(6):353-60
references.pmid: 8718694
references.citation: Hematology. 2013 Jan;18(1):50-5
references.pmid: 23321010
references.citation: Transfus Med. 1994 Sep;4(3):177-8
references.pmid: 7827643
references.citation: Hemoglobin. 2007;31(4):439-52
references.pmid: 17994378
references.citation: N Engl J Med. 1999 Jul 8;341(2):99-109
references.pmid: 10395635
references.citation: Br J Haematol. 2006 Jun;133(6):667-74
references.pmid: 16704445
references.citation: Blood. 2005 Jan 15;105(2):855-61
references.pmid: 15256427
references.citation: Genet Med. 2010 Feb;12(2):61-76
references.pmid: 20098328
references.citation: Hemoglobin. 2011;35(5-6):659-64
references.pmid: 22074126
references.citation: Cold Spring Harb Perspect Med. 2012 Dec 01;2(12):a011726
references.pmid: 23209183
references.citation: Am Fam Physician. 2009 Aug 15;80(4):339-44
references.pmid: 19678601
references.citation: Blood. 2012 Nov 8;120(19):3875-81
references.pmid: 22968457
references.citation: Int J Hematol. 2009 Nov;90(4):435-445
references.pmid: 19862602
references.citation: Blood Cells Mol Dis. 2011 Oct 15;47(3):166-75
references.pmid: 21843958
references.citation: Br J Haematol. 2003 Nov;123(4):730-7
references.pmid: 14616979
references.citation: Hemoglobin. 2010 Jun;34(3):305-21
references.pmid: 20524820
references.citation: Blood Rev. 2008 Mar;22(2):53-63
references.pmid: 18039551
references.citation: J Biomed Biotechnol. 2001;1(3):129-132
references.pmid: 12488606
references.citation: N Engl J Med. 1990 Feb 15;322(7):417-21
references.pmid: 2300104
references.citation: Hemoglobin. 2011;35(2):125-34
references.pmid: 21417569
references.citation: Hemoglobin. 2009;33 Suppl 1:S136-8
references.pmid: 20001617
references.citation: J Cardiovasc Magn Reson. 2008 Sep 25;10:42
references.pmid: 18817553
references.citation: JAMA. 2008 Jan 23;299(3):324-31
references.pmid: 18212317
references.citation: Ann Saudi Med. 2006 Jan-Feb;26(1):14-6
references.pmid: 16521869
references.citation: Hemoglobin. 2011;35(5-6):469-75
references.pmid: 21967642
references.citation: Int J Hematol. 2008 Jul;88(1):7-15
references.pmid: 18594779
references.citation: J Pediatr Endocrinol Metab. 2003 Mar;16 Suppl 2:259-66
references.pmid: 12729401
references.citation: Pediatr Int. 2005 Feb;47(1):84-9
references.pmid: 15693873
references.citation: Eur J Pediatr. 2008 Apr;167(4):377-81
references.pmid: 17899187
references.citation: Blood. 1997 Feb 1;89(3):739-61
references.pmid: 9028304
references.citation: Indian J Pediatr. 2011 Jun;78(6):679-83
references.pmid: 21234716
references.citation: J Trop Pediatr. 2003 Jul;49 Suppl 1:i1-20
references.pmid: 12934793
references.citation: Hemoglobin. 2009;33 Suppl 1:S145-59
references.pmid: 20001620
references.citation: Lung. 2006 May-Jun;184(3):177-85
references.pmid: 16902843
references.citation: Haematologica. 2010 Mar;95(3):376-81
references.pmid: 19903676
references.citation: Baillieres Clin Haematol. 1998 Mar;11(1):1-51
references.pmid: 10872472
references.citation: Obstet Gynecol. 2005 Jul;106(1):203-10
references.pmid: 15994657
references.citation: Blood. 2004 Aug 15;104(4):1201-3
references.pmid: 15039283
references.citation: Ann N Y Acad Sci. 1998 Jun 30;850:344-8
references.pmid: 9668556
references.citation: AJR Am J Roentgenol. 2009 May;192(5):1425-9
references.pmid: 19380572
references.citation: Acta Paediatr Jpn. 1997 Jun;39(3):354-7
references.pmid: 9241900
references.citation: J Assist Reprod Genet. 1998 May;15(5):219-25
references.pmid: 9604751
references.citation: Hemoglobin. 2009;33 Suppl 1:S183-7
references.pmid: 20001624
references.citation: J Clin Endocrinol Metab. 2003 Aug;88(8):3966-72
references.pmid: 12915694
references.citation: Community Genet. 2005;8(1):35-9
references.pmid: 15767753
references.citation: N Engl J Med. 1999 Feb 11;340(6):438-47
references.pmid: 9971869
references.citation: Br J Haematol. 2006 Aug;134(4):438-44
references.pmid: 16822284