| Secondary erythrocytosis caused by hemoglobin Tak/(δβ)0-thalassemia syndrome. | |
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MedLine Citation:
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PMID: 20798489 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Secondary erythrocytosis may arise from several causes, but an association with oxygen transport is rare. We describe for the first time a form of secondary erythrocytosis caused by compound heterozygosity for hemoglobin (Hb) Tak and (δβ)(0)-thalassemia found in an adult Thai individual. The patient had marked erythrocytosis and microcytosis with increased Hb and hematocrit values. Hb analyses using the Hb Gold Analyzer showed Hb A₂ (72.5%) and Hb F (30.0%) without Hb A while the capillary electrophoresis revealed 2.3% Hb A₂ and a major peak of Hb F (91.2%). Further molecular investigation identified that he was in fact a compound heterozygote for Hb Tak and deletional (δβ)(0)-thalassemia. Hematological parameters of the patient were compared with those observed for a Thai boy who demonstrated features of erythrocytosis and microcytosis caused by homozygous Hb Tak with α(+)-thalassemia and with those of pure carriers of Hb Tak and (δβ)(0)-thalassemia in our series. This report confirms the importance of both Hb and molecular investigations for the assessment of genotype/phenotype correlation and the appropriate management of the patients. |
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Authors:
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Nattaphol Prakobkaew; Sanita Singsanan; Goonnapa Fucharoen; Satja Surapot; Supan Fucharoen |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't Date: 2010-08-26 |
Journal Detail:
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Title: Acta haematologica Volume: 124 ISSN: 1421-9662 ISO Abbreviation: Acta Haematol. Publication Date: 2010 |
Date Detail:
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Created Date: 2010-10-07 Completed Date: 2010-10-25 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0141053 Medline TA: Acta Haematol Country: Switzerland |
Other Details:
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Languages: eng Pagination: 115-9 Citation Subset: IM |
Copyright Information:
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Copyright © 2010 S. Karger AG, Basel. |
Affiliation:
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Biomedical Science Program, Graduate School, Khon Kaen University, Khon Kaen, Thailand. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Base Sequence Child Genotype Hemoglobins, Abnormal / genetics* Humans Male Phenotype Polycythemia / etiology*, genetics* alpha-Thalassemia / etiology, genetics beta-Thalassemia / complications*, genetics* |
| Chemical | |
Reg. No./Substance:
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0/Hemoglobins, Abnormal; 0/hemoglobin Tak |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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