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Hemoglobin
international journal for hemoglobin research
Volume 32, 2008 - Issue 4
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A Rare Thalassemic Syndrome Caused by Interaction of Hb Adana [α59(E8)Gly→Asp] with an α+-Thalassemia Deletion: Clinical Aspects in Two Cases

Varvara Douna Hematology Laboratory, “P. and A. Kyriakou” Children's Hospital, Athens, Greece
,
Ioannis Papassotiriou Department of Clinical Biochemistry, “Aghia Sophia” Children's Hospital, Athens, Greece
,
Anastasia Garoufi Second Department of Pediatrics, University of Athens, “P. and A. Kyriakou” Children's Hospital, Athens, Greece
,
Eleni Georgouli Second Department of Pediatrics, University of Athens, “P. and A. Kyriakou” Children's Hospital, Athens, Greece
,
Vassilis Ladis Thalassemia Unit, First Department of Pediatrics, University of Athens, “Aghia Sophia” Children's Hospital, Athens, Greece
,
Alexandra Stamoulakatou Department of Hematology, “Aghia Sophia” Children's Hospital, Athens, Greece
,
Anna Metaxotou-Mavrommati Laboratory of Medical Genetics, University of Athens, “Aghia Sophia” Children's Hospital, Athens, Greece
,
Emmanuel Kanavakis Laboratory of Medical Genetics, University of Athens, “Aghia Sophia” Children's Hospital, Athens, Greece
&
Joanne Traeger-Synodinos Laboratory of Medical Genetics, University of Athens, “Aghia Sophia” Children's Hospital, Athens, GreeceCorrespondence[email protected]
 show all
Pages 361-369 | Received 15 Oct 2007, Accepted 21 Dec 2007, Published online: 07 Jul 2009

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Katarina M. Semkiu, Jennifer L. Oliveira, Phuong L. Nguyen, Tavanna R. Porter & David B. Wilson. (2020) Hb F-Wentzville [Gγ24(B6)Gly→Glu; HBG2: c.74G>A, p.Gly25Glu]: An Unstable Gγ-Globin Variant Associated with Neonatal Hemolytic Anemia. Hemoglobin 44:1, pages 67-69.
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Stamatia Theodoridou, Aikaterini Teli, Eleni Yfanti, Timoleon-Achilleas Vyzantiadis, Theodoros Theodoridis & Marina Economou. (2018) Compound Heterozygosity for Hb Adana (HBA2: c.179G>A) and the –α3.7/αα Thalassemia Deletion in Greece: Clinical Phenotype and Genetic Counseling. Hemoglobin 42:2, pages 129-131.
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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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Dewi Megawati, Ita M. Nainggolan, Maria Swastika, Susi Susanah, Johanes C. Mose, Alida R. Harahap & Iswari Setianingsih. (2014) Severe α-Thalassemia Intermedia Due to a Compound Heterozygosity for the Highly Unstable Hb Adana (HBA2: c.179G>A) and a Novel Codon 24 (HBA2: c.75T>A) Mutation. Hemoglobin 38:2, pages 149-151.
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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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Joanne Traeger-Synodinos, Varvara Douna, Ioannis Papassotiriou, Alexandra Stamoulakatou, Vasilis Ladis, Tania Siahanidou, Irine Fylaktou & Emmanuel Kanavakis. (2010) Variable and Often Severe Phenotypic Expression in Patients with the α-Thalassemic Variant Hb Agrinio [α29(B10)Leu→Pro (α2)]. Hemoglobin 34:5, pages 430-438.
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Varvara Douna, Ioannis Papassotiriou, Anna Metaxotou-Mavrommati, Alexandra Stamoulakatou, Dimitra Liapi, Dimitrios Kampourakis, Amalia Tsilimigaki, Emmanuel Kanavakis & Joanne Traeger-Synodinos. (2008) Further Identification of The Hyperunstable α-Globin Chain Variant Hb Heraklion [codons 36/37 (–CCC); Pro→0 (α1)] in Greek Cases With Co-Inherited α+-Thalassemia Mutations. Hemoglobin 32:4, pages 379-385.
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Henri Wajcman, Jan Traeger-Synodinos, Ioannis Papassotiriou, Piero C. Giordano, Cornelis L. Harteveld, Véronique Baudin-Creuza & John Old. (2008) Unstable and Thalassemic α Chain Hemoglobin Variants: A Cause of Hb H Disease and Thalassemia Intermedia. Hemoglobin 32:4, pages 327-349.
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Articles from other publishers (7)

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.
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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.
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Samaneh Farashi & Cornelis L. Harteveld. (2018) Molecular basis of α-thalassemia. Blood Cells, Molecules, and Diseases 70, pages 43-53.
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Jin Ai Mary Anne Tan, Siew Leng Kho, Chin Fang Ngim, Kek Heng Chua, Ai Sim Goh, Seoh Leng Yeoh & Elizabeth George. (2016) DNA studies are necessary for accurate patient diagnosis in compound heterozygosity for Hb Adana (HBA2:c.179>A) with deletional or nondeletional α-thalassaemia. Scientific Reports 6:1.
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Samaneh Farashi & Hossein Najmabadi. (2015) Diagnostic pitfalls of less well recognized HbH disease. Blood Cells, Molecules, and Diseases 55:4, pages 387-395.
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Economou Marina, Papadopoulou Eleni, Tsatra Ioanna & Athanassiou-Metaxa Miranda. (2010) A Case of Mild Thalassemic Syndrome Caused by Interaction of Hb Adana With an α+-thalassemia Deletion. Journal of Pediatric Hematology/Oncology 32:2, pages 167-168.
Crossref
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.
Crossref

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