Does absolute excess of alpha chains compromise the benefit of splenectomy in patients with thalassemia intermedia?

Haematologica

Volumn 97, Issue 1, 2012, Pages 151-153

  Graziadei, Giovanna ^a   Refaldi, Chiara ^a   Barcellini, Wilma ^a   Cesaretti, Claudia ^b   Cassinero, Elena ^a   Musallam, Khaled M ^c   Cappellini, Maria Domenica ^a  

^a UNIVERSITY OF MILAN   (Italy)

^b FONDAZIONE IRCCS CA GRANDA OSPEDALE MAGGIORE POLICLINICO   (Italy)

^c AMERICAN UNIVERSITY OF BEIRUT   (Lebanon)

Author keywords

Adverse outcome; Alpha chains; Excess; Splenectomy; Thalassemia intermedia

Indexed keywords

ALPHA GLOBIN; HEMOGLOBIN;

ADOLESCENT; ADULT; ALPHA CHAIN; ALPHA GLOBIN GENE; ANEMIA; BETA GLOBIN GENE; BLOOD TRANSFUSION; CASE REPORT; CHELATION THERAPY; DNA DETERMINATION; FATHER; FEMALE; GENE; HEMOGLOBIN BLOOD LEVEL; HUMAN; ITALY; LETTER; MALE; MOTHER; SIBLING; SPLENECTOMY; THALASSEMIA; THALASSEMIA INTERMEDIA; TREATMENT RESPONSE;

ADOLESCENT; ADULT; ALPHA-GLOBINS; BETA-THALASSEMIA; FEMALE; HUMANS; MALE; MIDDLE AGED; SIBLING RELATIONS; SPLENECTOMY; YOUNG ADULT;

EID: 84855249050     PISSN: 03906078     EISSN: 15928721     Source Type: Journal    
DOI: 10.3324/haematol.2011.046730     Document Type: Letter

References (11)

1. Taher AT, Musallam KM, Cappellini MD, Weatherall DJ. Optimal management of beta thalassaemia intermedia. Br J Haematol. 2011;152(5):512-23.
2. Camaschella C, Kattamis AC, Petroni D, Roetto A, Sivera P, Sbaiz L, et al. Different hematological phenotypes caused by the interaction of triplicated alpha-globin genes and heterozygous beta-tha-lassemia. Am J Hematol. 1997;55(2):83-8.
3. Sampietro M, Cazzola M, Cappellini MD, Fiorelli G. The triplicated alpha-gene locus and heterozygous beta thalassaemia: a case of tha-lassaemia intermedia. Br J Haematol. 1983;55(4):709-10.
4. Harteveld CL, Refaldi C, Cassinerio E, Cappellini MD, Giordano PC. Segmental duplications involving the alpha-globin gene cluster are causing beta-thalassemia intermedia phenotypes in beta-tha-lassemia heterozygous patients. Blood Cells Mol Dis. 2008;40(3): 312-6.
5. Sollaino MC, Paglietti ME, Perseu L, Giagu N, Loi D, Galanello R. Association of alpha globin gene quadruplication and heterozygous beta thalassemia in patients with thalassemia intermedia. Haematologica. 2009;94(10):1445-8.
6. Harteveld CL, Voskamp A, Phylipsen M, Akkermans N, den Dunnen JT, White SJ, et al. Nine unknown rearrangements in 16p13.3 and 11p15.4 causing alpha- and beta-thalassaemia characterised by high resolution multiplex ligation-dependent probe amplification. J Med Genet. 2005;42(12):922-31.
7. Mannu F, Arese P, Cappellini MD, Fiorelli G, Cappadoro M, Giribaldi G, et al. Role of hemichrome binding to erythrocyte membrane in the generation of band-3 alterations in beta-thalassemia intermedia erythrocytes. Blood. 1995;86(5):2014-20.
8. Shinar E, Rachmilewitz EA. Oxidative denaturation of red blood cells in thalassemia. Semin Hematol. 1990;27(1):70-82.
9. Taher AT, Musallam KM, Karimi M, El-Beshlawy A, Belhoul K, Daar S, et al. Splenectomy and thrombosis: the case of thalassemia intermedia. J Thromb Haemost. 2010;8(10):2152-8.
10. Cappellini MD, Robbiolo L, Bottasso BM, Coppola R, Fiorelli G, Mannucci AP. Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia. Br J Haematol. 2000;111(2):467-73.
11. Cappellini MD, Motta I, Musallam KM, Taher AT. Redefining tha-lassemia as a hypercoagulable state. Ann NY Acad Sci. 2010;1202: 231-6.