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Hemoglobin
international journal for hemoglobin research
Volume 23, 1999 - Issue 4
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Original Article

Interaction of an α+-Thalassemia Deletion with Either a Highly Unstable α-Globin Variant (α2, Codon 59, GGC→GAC) or a Nondeletional α-Thalassemia Mutation (AATAA→AATAAG): Comparison of Phenotypes Illustrating “Dominant” α-Thalassemia

J. Traeger-Synodinos First Department of Pediatrics, University of Athens St. Sophia's Children's Hospital, Athens, 11527, Greece
,
A. Metaxotou-Mavrommati First Department of Pediatrics, University of Athens St. Sophia's Children's Hospital, Athens, 11527, Greece
,
M. Karagiorga Thalassemia Unit, St. Sophia's Children's Hospital, Athens, 11527, Greece
,
C. Vrettou First Department of Pediatrics, University of Athens St. Sophia's Children's Hospital, Athens, 11527, Greece
,
I. Papassotiriou Hematology Laboratory, St. Sophia's Children's Hospital, Athens, 11527, Greece; Department of Clinical Biochemistry, St. Sophia's Children's Hospital, Athens, 11527, Greece
,
A. Stamoulakatou Hematology Laboratory, St. Sophia's Children's Hospital, Athens, 11527, Greece
&
E. Kanavakis First Department of Pediatrics, University of Athens St. Sophia's Children's Hospital, Athens, 11527, GreeceCorrespondence[email protected]
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Pages 325-337 | Received 10 May 1999, Accepted 15 Jun 1999, Published online: 07 Jul 2009

Citations (17)

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Hafiza Alauddin, Khairina Kamarudin, Tang Yee Loong, Raja Zahratul Azma, Azlin Ithnin, Norunaluwar Jalil, Noor-Farisah Razak, Danny Koh-Xuan-Rong, Endom Ismail, Loh C-Khai, Zarina Abdul Latiff, Hamidah Alias & Ainoon Othman. (2018) A Unique Interaction of IVS-I-1 (G>A) (HBA2: c.95+1G>A) with Hb Adana (HBA2: c.179G>A) Presenting as Transfusion-Dependent α-Thalassemia. Hemoglobin 42:4, pages 247-251.
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Tekin Aksu, Neşe Yaralı, Cengiz Bayram, Ali Fettah, Zekai Avcı & Bahattin Tunç. (2014) Homozygosity for HBA1: c.179G > A: Hb Adana in an Infant. Hemoglobin 38:6, pages 449-450.
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Hafiza Alauddin, Noor-Adilah Jaapar, Raja Z. Azma, Azlin Ithnin, Noor-Farisah A. Razak, C-Khai Loh, Hamidah Alias, Zarina Abdul-Latiff & Ainoon Othman. (2014) A Case Series of α-Thalassemia Intermedia Due to Compound Heterozygosity for Hb Adana [HBA2: c179G>A (or HBA1); p.Gly60Asp] With Other α-Thalassemias in Malay Families. Hemoglobin 38:4, pages 277-281.
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Ita M. Nainggolan, Alida Harahap, Debby D. Ambarwati, Rosalina V. Liliani, Dewi Megawati, Maria Swastika & Iswari Setianingsih. (2013) Interaction of Hb Adana (HBA2: c.179G>A) with Deletional and Nondeletional α+-Thalassemia Mutations: Diverse Hematological and Clinical Features. Hemoglobin 37:3, pages 297-305.
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Ita M. Nainggolan, Alida Harahap & Iswari Setianingsih. (2010) Hydrops Fetalis Associated with Homozygosity for Hb Adana [α59(E8)Gly→Asp (α2)]. Hemoglobin 34:4, pages 394-401.
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Varvara Douna, Ioannis Papassotiriou, Anastasia Garoufi, Eleni Georgouli, Vassilis Ladis, Alexandra Stamoulakatou, Anna Metaxotou-Mavrommati, Emmanuel Kanavakis & Joanne Traeger-Synodinos. (2008) A Rare Thalassemic Syndrome Caused by Interaction of Hb Adana [α59(E8)Gly→Asp] with an α+-Thalassemia Deletion: Clinical Aspects in Two Cases. Hemoglobin 32:4, pages 361-369.
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John F. Prior, Erna Lim, Neela Lingam, John L. Raven & Jill Finlayson. (2007) A Moderately Severe α-Thalassemia Condition Resulting From a Combination of the α2 Polyadenylation Signal (AATAAA→AATA– –) Mutation and a 3.7 Kb α Gene Deletion in an Australian Family. Hemoglobin 31:2, pages 173-177.
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C. Vrettou, E. Kanavakis, J. Traeger-Synodinos, A. Metaxotou-Mavrommati, I. Basiakos, E. Maragoudaki, A. Stamoulakatou, I. Papassotiriou & C. Kattamis. (2000) Molecular Studies of β-Thalassemia Heterozygotes with Raised Hb F Levels. Hemoglobin 24:3, pages 203-220.
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Articles from other publishers (9)

Gisele A. Pedroso, Elza M. Kimura, Magnun N.N. Santos, Dulcinéia M. Albuquerque, Danaê Malimpensa, Susan E. Jorge, Monica P.A. Verissimo, Fernando F. Costa & Maria F. Sonati. (2018) Thalassemia major phenotype caused by HB Zürich-Albisrieden [α2 59(E8) Gly > Arg (HBA2:C.178G > C)] in a Brazilian child. Pediatric Blood & Cancer 65:12, pages e27413.
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Sharon A. Singh, Susmita Sarangi, Abena Appiah-Kubi, Peihong Hsu, W. Byron Smith, Patrick G. Gallagher, Bertil Glader & David H. K. Chui. (2018) Hb Adana (HBA2 or HBA1: c.179G > A) and alpha thalassemia: Genotype-phenotype correlation. Pediatric Blood & Cancer 65:9, pages e27220.
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Rahimah Ahmad, Mohamed Saleem, Nisha Aloysious, Punithawathy Yelumalai, Nurul Mohamed & Syahzuwan Hassan. (2013) Distribution of Alpha Thalassaemia Gene Variants in Diverse Ethnic Populations in Malaysia: Data from the Institute for Medical Research. International Journal of Molecular Sciences 14:9, pages 18599-18614.
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Asude Alpman Durmaz, Haluk Akin, Aslihan Yilmaz Ekmekci, Huseyin Onay, Burak Durmaz, Ozgur Cogulu, Yesim Aydinok & Ferda Ozkinay. (2009) A Severe α Thalassemia Case Compound Heterozygous for Hb Adana in α1 Gene and 20.5 kb Double Gene Deletion. Journal of Pediatric Hematology/Oncology 31:8, pages 592-594.
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Kaimin Chan, Man Sim Wong, T. K. Chan & Vivian Chan. (2004) A thalassaemia array for Southeast Asia. British Journal of Haematology 124:2, pages 232-239.
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Iswari Setianingsih, Alida Harahap & Ita M. Nainggolan. 2003. Tropical Diseases. Tropical Diseases 47 56 .
Emmanuel Kanavakis, Ioannis Papassotiriou, Markissia Karagiorga, Christina Vrettou, Anna Metaxotou‐Mavrommati, Alexandra Stamoulakatou, Christos Kattamis & Joanne Traeger‐Synodinos. (2008) Phenotypic and molecular diversity of haemoglobin H disease: a Greek experience. British Journal of Haematology 111:3, pages 915-923.
Crossref
Emmanuel Kanavakis, Ioannis Papassotiriou, Markissia Karagiorga, Christina Vrettou, Anna Metaxotou-Mavrommati, Alexandra Stamoulakatou, Christos Kattamis & Joanne Traeger-Synodinos. (2000) Phenotypic and molecular diversity of haemoglobin H disease: a Greek experience. British Journal of Haematology 111:3, pages 915-923.
Crossref
Joanne Traeger-Synodinos, Ioannis Papassotiriou, Anna Metaxotou-Mavrommati, Christina Vrettou, Alexandra Stamoulakatou & Emmanuel Kanavakis. (2000) Distinct Phenotypic Expression Associated with a New Hyperunstable Alpha Globin Variant (Hb Heraklion, α1cd37(C2)Pro>0): Comparison to Other α-Thalassemic Hemoglobinopathies. Blood Cells, Molecules, and Diseases 26:4, pages 276-284.
Crossref

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