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Heterozygous Hemoglobin Sherwood Forest Causing Polycythemia

Hemoglobin (Hb) Sherwood Forest is a rare high-affinity hemoglobin first described in 1977, arising from an Arg to Thr substitution at codon 104 of the beta chain. This hemoglobin variant has been identified in few individuals and has been associated with a compensatory erythrocytosis in the homozyg...

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Autores principales: Raghunathan, Vikram M., Butera, James N., Treaba, Diana O.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5637849/
https://www.ncbi.nlm.nih.gov/pubmed/29093977
http://dx.doi.org/10.1155/2017/8174207
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author Raghunathan, Vikram M.
Butera, James N.
Treaba, Diana O.
author_facet Raghunathan, Vikram M.
Butera, James N.
Treaba, Diana O.
author_sort Raghunathan, Vikram M.
collection PubMed
description Hemoglobin (Hb) Sherwood Forest is a rare high-affinity hemoglobin first described in 1977, arising from an Arg to Thr substitution at codon 104 of the beta chain. This hemoglobin variant has been identified in few individuals and has been associated with a compensatory erythrocytosis in the homozygous state. Prior scarce case reports have noted that heterozygotes for this variant are phenotypically normal. Here we present a patient who was evaluated in our hematology clinic for chronic erythrocytosis and was found to be heterozygous for Hb Sherwood Forest. No other primary or secondary cause of his polycythemia was identified. This is the first described case of heterozygous Hemoglobin Sherwood Forest causing erythrocytosis.
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spelling pubmed-56378492017-11-01 Heterozygous Hemoglobin Sherwood Forest Causing Polycythemia Raghunathan, Vikram M. Butera, James N. Treaba, Diana O. Case Rep Hematol Case Report Hemoglobin (Hb) Sherwood Forest is a rare high-affinity hemoglobin first described in 1977, arising from an Arg to Thr substitution at codon 104 of the beta chain. This hemoglobin variant has been identified in few individuals and has been associated with a compensatory erythrocytosis in the homozygous state. Prior scarce case reports have noted that heterozygotes for this variant are phenotypically normal. Here we present a patient who was evaluated in our hematology clinic for chronic erythrocytosis and was found to be heterozygous for Hb Sherwood Forest. No other primary or secondary cause of his polycythemia was identified. This is the first described case of heterozygous Hemoglobin Sherwood Forest causing erythrocytosis. Hindawi 2017 2017-09-28 /pmc/articles/PMC5637849/ /pubmed/29093977 http://dx.doi.org/10.1155/2017/8174207 Text en Copyright © 2017 Vikram M. Raghunathan et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Raghunathan, Vikram M.
Butera, James N.
Treaba, Diana O.
Heterozygous Hemoglobin Sherwood Forest Causing Polycythemia
title Heterozygous Hemoglobin Sherwood Forest Causing Polycythemia
title_full Heterozygous Hemoglobin Sherwood Forest Causing Polycythemia
title_fullStr Heterozygous Hemoglobin Sherwood Forest Causing Polycythemia
title_full_unstemmed Heterozygous Hemoglobin Sherwood Forest Causing Polycythemia
title_short Heterozygous Hemoglobin Sherwood Forest Causing Polycythemia
title_sort heterozygous hemoglobin sherwood forest causing polycythemia
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5637849/
https://www.ncbi.nlm.nih.gov/pubmed/29093977
http://dx.doi.org/10.1155/2017/8174207
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