A second Australian family with hemoglobin north shore (β134 val→glu)

Howard Gurney, Ibrahim Baig, Susan Gordon, Kiran Phadke, Heather Kearsley, Pat Fleming, Ken Wyatt, Wilbur Hughes

Research output: Contribution to journalArticleResearchpeer-review

Abstract

A second Australian family is reported with Hemoglobin North Shore (β134 Val→Glu), an unstable hemoglobin, causing no clinical symptoms. All affected family members showed only mild reticulocytosis and microcytosis on the blood film, despite the strongly positive isopropanol test for Hb (hemoglobin) stability and numerous red cell inclusions. Hb North Shore constituted 31-38% of the total hemoglobin and migrated on the anodal side of Hb A at pH 8.9. The association of a mildly raised Hb A2 level and thalassemic phenotype with Hb North Shore previously reported, is confirmed in this study.

LanguageEnglish
Pages62-63
Number of pages2
JournalPathology
Volume19
Issue number1
Publication statusPublished - 1987
Externally publishedYes

Fingerprint

Hemoglobins
Hemoglobin A2
Reticulocytosis
Hemoglobin A
2-Propanol
Phenotype
hemoglobin North Shore

Keywords

  • Raised Hb A2
  • Red cell inclusions
  • Unstable hemoglobin

Cite this

Gurney, H., Baig, I., Gordon, S., Phadke, K., Kearsley, H., Fleming, P., ... Hughes, W. (1987). A second Australian family with hemoglobin north shore (β134 val→glu). Pathology, 19(1), 62-63.
Gurney, Howard ; Baig, Ibrahim ; Gordon, Susan ; Phadke, Kiran ; Kearsley, Heather ; Fleming, Pat ; Wyatt, Ken ; Hughes, Wilbur. / A second Australian family with hemoglobin north shore (β134 val→glu). In: Pathology. 1987 ; Vol. 19, No. 1. pp. 62-63.
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Gurney, H, Baig, I, Gordon, S, Phadke, K, Kearsley, H, Fleming, P, Wyatt, K & Hughes, W 1987, 'A second Australian family with hemoglobin north shore (β134 val→glu)', Pathology, vol. 19, no. 1, pp. 62-63.

A second Australian family with hemoglobin north shore (β134 val→glu). / Gurney, Howard; Baig, Ibrahim; Gordon, Susan; Phadke, Kiran; Kearsley, Heather; Fleming, Pat; Wyatt, Ken; Hughes, Wilbur.

In: Pathology, Vol. 19, No. 1, 1987, p. 62-63.

Research output: Contribution to journalArticleResearchpeer-review

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T1 - A second Australian family with hemoglobin north shore (β134 val→glu)

AU - Gurney, Howard

AU - Baig, Ibrahim

AU - Gordon, Susan

AU - Phadke, Kiran

AU - Kearsley, Heather

AU - Fleming, Pat

AU - Wyatt, Ken

AU - Hughes, Wilbur

PY - 1987

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N2 - A second Australian family is reported with Hemoglobin North Shore (β134 Val→Glu), an unstable hemoglobin, causing no clinical symptoms. All affected family members showed only mild reticulocytosis and microcytosis on the blood film, despite the strongly positive isopropanol test for Hb (hemoglobin) stability and numerous red cell inclusions. Hb North Shore constituted 31-38% of the total hemoglobin and migrated on the anodal side of Hb A at pH 8.9. The association of a mildly raised Hb A2 level and thalassemic phenotype with Hb North Shore previously reported, is confirmed in this study.

AB - A second Australian family is reported with Hemoglobin North Shore (β134 Val→Glu), an unstable hemoglobin, causing no clinical symptoms. All affected family members showed only mild reticulocytosis and microcytosis on the blood film, despite the strongly positive isopropanol test for Hb (hemoglobin) stability and numerous red cell inclusions. Hb North Shore constituted 31-38% of the total hemoglobin and migrated on the anodal side of Hb A at pH 8.9. The association of a mildly raised Hb A2 level and thalassemic phenotype with Hb North Shore previously reported, is confirmed in this study.

KW - Raised Hb A2

KW - Red cell inclusions

KW - Unstable hemoglobin

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Gurney H, Baig I, Gordon S, Phadke K, Kearsley H, Fleming P et al. A second Australian family with hemoglobin north shore (β134 val→glu). Pathology. 1987;19(1):62-63.