Homozygosity for a new type of gγaγdelta;beta;deg;thalassemia in a malaysian Male

K. Faridah, E. George, R. J. Trent, B. J. Padanilam, H. J.J. Huang, T. H.J. Huisman

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

Hematological and clinical data are presented for a young Malay patient with a homozygous (δbeta;deg;thalas- semic condition. His red blood cells contained 1007% fetal hemoglobin with α and Gγ chains only. Detailed gene mapping defined a large deletion with a 5' end between the Aha III and Apa I sites, some 200-400 bp 5' to the Aγ globin gene and a 3' end beyond sequences 17-18 kb 3' to the β globin gene. This GγAγdelta;beta;deg;type of thalassemia is different from all the other six types described before. Comparison of the hematological data of this patient with those of homozygotes for either the Sicilian or Spanish types of GγAγdelta;beta;deg;thalassemia showed no differences; all homozygotes have a moderate anemia which is accentuated by the relatively high oxygen affinity of the Hb F containing erythrocytes.

Original languageEnglish (US)
Pages (from-to)353-363
Number of pages11
JournalHemoglobin
Volume10
Issue number4
DOIs
StatePublished - Jan 1 1986

Fingerprint

Thalassemia
Globins
Homozygote
Genes
Erythrocytes
Fetal Hemoglobin
Chromosome Mapping
Anemia
Oxygen
Blood
Cells

ASJC Scopus subject areas

  • Hematology
  • Clinical Biochemistry
  • Genetics(clinical)
  • Biochemistry, medical

Cite this

Faridah, K., George, E., Trent, R. J., Padanilam, B. J., Huang, H. J. J., & Huisman, T. H. J. (1986). Homozygosity for a new type of gγaγdelta;beta;deg;thalassemia in a malaysian Male. Hemoglobin, 10(4), 353-363. https://doi.org/10.3109/03630268608996867

Homozygosity for a new type of gγaγdelta;beta;deg;thalassemia in a malaysian Male. / Faridah, K.; George, E.; Trent, R. J.; Padanilam, B. J.; Huang, H. J.J.; Huisman, T. H.J.

In: Hemoglobin, Vol. 10, No. 4, 01.01.1986, p. 353-363.

Research output: Contribution to journalArticle

Faridah, K, George, E, Trent, RJ, Padanilam, BJ, Huang, HJJ & Huisman, THJ 1986, 'Homozygosity for a new type of gγaγdelta;beta;deg;thalassemia in a malaysian Male', Hemoglobin, vol. 10, no. 4, pp. 353-363. https://doi.org/10.3109/03630268608996867
Faridah, K. ; George, E. ; Trent, R. J. ; Padanilam, B. J. ; Huang, H. J.J. ; Huisman, T. H.J. / Homozygosity for a new type of gγaγdelta;beta;deg;thalassemia in a malaysian Male. In: Hemoglobin. 1986 ; Vol. 10, No. 4. pp. 353-363.
@article{c9f1b4e8552744018313a6efb400e57a,
title = "Homozygosity for a new type of gγaγdelta;beta;deg;thalassemia in a malaysian Male",
abstract = "Hematological and clinical data are presented for a young Malay patient with a homozygous (δbeta;deg;thalas- semic condition. His red blood cells contained 1007{\%} fetal hemoglobin with α and Gγ chains only. Detailed gene mapping defined a large deletion with a 5' end between the Aha III and Apa I sites, some 200-400 bp 5' to the Aγ globin gene and a 3' end beyond sequences 17-18 kb 3' to the β globin gene. This GγAγdelta;beta;deg;type of thalassemia is different from all the other six types described before. Comparison of the hematological data of this patient with those of homozygotes for either the Sicilian or Spanish types of GγAγdelta;beta;deg;thalassemia showed no differences; all homozygotes have a moderate anemia which is accentuated by the relatively high oxygen affinity of the Hb F containing erythrocytes.",
author = "K. Faridah and E. George and Trent, {R. J.} and Padanilam, {B. J.} and Huang, {H. J.J.} and Huisman, {T. H.J.}",
year = "1986",
month = "1",
day = "1",
doi = "10.3109/03630268608996867",
language = "English (US)",
volume = "10",
pages = "353--363",
journal = "Hemoglobin",
issn = "0363-0269",
publisher = "Informa Healthcare",
number = "4",

}

TY - JOUR

T1 - Homozygosity for a new type of gγaγdelta;beta;deg;thalassemia in a malaysian Male

AU - Faridah, K.

AU - George, E.

AU - Trent, R. J.

AU - Padanilam, B. J.

AU - Huang, H. J.J.

AU - Huisman, T. H.J.

PY - 1986/1/1

Y1 - 1986/1/1

N2 - Hematological and clinical data are presented for a young Malay patient with a homozygous (δbeta;deg;thalas- semic condition. His red blood cells contained 1007% fetal hemoglobin with α and Gγ chains only. Detailed gene mapping defined a large deletion with a 5' end between the Aha III and Apa I sites, some 200-400 bp 5' to the Aγ globin gene and a 3' end beyond sequences 17-18 kb 3' to the β globin gene. This GγAγdelta;beta;deg;type of thalassemia is different from all the other six types described before. Comparison of the hematological data of this patient with those of homozygotes for either the Sicilian or Spanish types of GγAγdelta;beta;deg;thalassemia showed no differences; all homozygotes have a moderate anemia which is accentuated by the relatively high oxygen affinity of the Hb F containing erythrocytes.

AB - Hematological and clinical data are presented for a young Malay patient with a homozygous (δbeta;deg;thalas- semic condition. His red blood cells contained 1007% fetal hemoglobin with α and Gγ chains only. Detailed gene mapping defined a large deletion with a 5' end between the Aha III and Apa I sites, some 200-400 bp 5' to the Aγ globin gene and a 3' end beyond sequences 17-18 kb 3' to the β globin gene. This GγAγdelta;beta;deg;type of thalassemia is different from all the other six types described before. Comparison of the hematological data of this patient with those of homozygotes for either the Sicilian or Spanish types of GγAγdelta;beta;deg;thalassemia showed no differences; all homozygotes have a moderate anemia which is accentuated by the relatively high oxygen affinity of the Hb F containing erythrocytes.

UR - http://www.scopus.com/inward/record.url?scp=0022621068&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0022621068&partnerID=8YFLogxK

U2 - 10.3109/03630268608996867

DO - 10.3109/03630268608996867

M3 - Article

C2 - 2427478

AN - SCOPUS:0022621068

VL - 10

SP - 353

EP - 363

JO - Hemoglobin

JF - Hemoglobin

SN - 0363-0269

IS - 4

ER -