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Research ArticleOriginal Article
Open Access

Evaluation of haemoglobin constant spring phenotypes and their haematological characteristics among high school students in Terengganu, Malaysia

A single - centred study

Sharifatul F. Embong, Sumaiyah Adzahar, Adibah Daud, Mohammad H. Nordin, Sarah A. Halim, Wan Zuhairah W. Embong, Nabilah Rameli, Razan H. Zulkeflee and Rosnah Bahar
Saudi Medical Journal March 2025, 46 (3) 292-298; DOI: https://doi.org/10.15537/smj.2025.46.3.20240771
Sharifatul F. Embong
From the Department of Pathology and Medical Laboratory (F. Embong, Adzahar, Daud), Faculty of Medicine, Sultan Zainal Abidin University, from the Department of Pathology (W. Embong), Sultanah Nur Zahirah Hospital, from the Department of Pathology (Rameli), Dungun Hospital, Terengganu, from the Department of Pathology and Laboratory Medicine (Halim), College of Medicine, International Islamic University Malaysia, Pahang, and from the Department of Haematology (Zulkeflee, Bahar), School of Medical Sciences, University of Science Malaysia, Kelantan, Malaysia.
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Sumaiyah Adzahar
From the Department of Pathology and Medical Laboratory (F. Embong, Adzahar, Daud), Faculty of Medicine, Sultan Zainal Abidin University, from the Department of Pathology (W. Embong), Sultanah Nur Zahirah Hospital, from the Department of Pathology (Rameli), Dungun Hospital, Terengganu, from the Department of Pathology and Laboratory Medicine (Halim), College of Medicine, International Islamic University Malaysia, Pahang, and from the Department of Haematology (Zulkeflee, Bahar), School of Medical Sciences, University of Science Malaysia, Kelantan, Malaysia.
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  • ORCID record for Sumaiyah Adzahar
  • For correspondence: [email protected]
Adibah Daud
From the Department of Pathology and Medical Laboratory (F. Embong, Adzahar, Daud), Faculty of Medicine, Sultan Zainal Abidin University, from the Department of Pathology (W. Embong), Sultanah Nur Zahirah Hospital, from the Department of Pathology (Rameli), Dungun Hospital, Terengganu, from the Department of Pathology and Laboratory Medicine (Halim), College of Medicine, International Islamic University Malaysia, Pahang, and from the Department of Haematology (Zulkeflee, Bahar), School of Medical Sciences, University of Science Malaysia, Kelantan, Malaysia.
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Mohammad H. Nordin
From the Department of Pathology and Medical Laboratory (F. Embong, Adzahar, Daud), Faculty of Medicine, Sultan Zainal Abidin University, from the Department of Pathology (W. Embong), Sultanah Nur Zahirah Hospital, from the Department of Pathology (Rameli), Dungun Hospital, Terengganu, from the Department of Pathology and Laboratory Medicine (Halim), College of Medicine, International Islamic University Malaysia, Pahang, and from the Department of Haematology (Zulkeflee, Bahar), School of Medical Sciences, University of Science Malaysia, Kelantan, Malaysia.
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Sarah A. Halim
From the Department of Pathology and Medical Laboratory (F. Embong, Adzahar, Daud), Faculty of Medicine, Sultan Zainal Abidin University, from the Department of Pathology (W. Embong), Sultanah Nur Zahirah Hospital, from the Department of Pathology (Rameli), Dungun Hospital, Terengganu, from the Department of Pathology and Laboratory Medicine (Halim), College of Medicine, International Islamic University Malaysia, Pahang, and from the Department of Haematology (Zulkeflee, Bahar), School of Medical Sciences, University of Science Malaysia, Kelantan, Malaysia.
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Wan Zuhairah W. Embong
From the Department of Pathology and Medical Laboratory (F. Embong, Adzahar, Daud), Faculty of Medicine, Sultan Zainal Abidin University, from the Department of Pathology (W. Embong), Sultanah Nur Zahirah Hospital, from the Department of Pathology (Rameli), Dungun Hospital, Terengganu, from the Department of Pathology and Laboratory Medicine (Halim), College of Medicine, International Islamic University Malaysia, Pahang, and from the Department of Haematology (Zulkeflee, Bahar), School of Medical Sciences, University of Science Malaysia, Kelantan, Malaysia.
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Nabilah Rameli
From the Department of Pathology and Medical Laboratory (F. Embong, Adzahar, Daud), Faculty of Medicine, Sultan Zainal Abidin University, from the Department of Pathology (W. Embong), Sultanah Nur Zahirah Hospital, from the Department of Pathology (Rameli), Dungun Hospital, Terengganu, from the Department of Pathology and Laboratory Medicine (Halim), College of Medicine, International Islamic University Malaysia, Pahang, and from the Department of Haematology (Zulkeflee, Bahar), School of Medical Sciences, University of Science Malaysia, Kelantan, Malaysia.
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Razan H. Zulkeflee
From the Department of Pathology and Medical Laboratory (F. Embong, Adzahar, Daud), Faculty of Medicine, Sultan Zainal Abidin University, from the Department of Pathology (W. Embong), Sultanah Nur Zahirah Hospital, from the Department of Pathology (Rameli), Dungun Hospital, Terengganu, from the Department of Pathology and Laboratory Medicine (Halim), College of Medicine, International Islamic University Malaysia, Pahang, and from the Department of Haematology (Zulkeflee, Bahar), School of Medical Sciences, University of Science Malaysia, Kelantan, Malaysia.
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Rosnah Bahar
From the Department of Pathology and Medical Laboratory (F. Embong, Adzahar, Daud), Faculty of Medicine, Sultan Zainal Abidin University, from the Department of Pathology (W. Embong), Sultanah Nur Zahirah Hospital, from the Department of Pathology (Rameli), Dungun Hospital, Terengganu, from the Department of Pathology and Laboratory Medicine (Halim), College of Medicine, International Islamic University Malaysia, Pahang, and from the Department of Haematology (Zulkeflee, Bahar), School of Medical Sciences, University of Science Malaysia, Kelantan, Malaysia.
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Article Figures & Data

Tables

    • View popup
    Table 1

    - Genotypes of hemoglobin constant spring (N=389).

    Types and coinheritancen (%)
    Heterozygous Hb CS359 (92.2)
    Homozygous Hb CS2 (0.5)
    Compound heterozygous Hb CS
    Heterozygous alpha 3.717 (4.3)
    Heterozygous alpha 4.23 (0.7)
    Haemoglobin adana2 (0.5)
    Heterozygous IVS-11 (0.3)
    Heterozygous alpha SEA1 (0.3)
    Haemoglobin Quang Sze1 (0.3)
    Haemoglobin Malay1 (0.3)
    Beta thalassemia trait1 (0.3)
    Haemoglobin D Punjab1 (0.3)

    Values are presented as numbers and percentages (%).

    Hb CS: hemoglobin constant spring, IVS-1: intervening sequence-1, SEA: Southeast Asian

      • View popup
      Table 2

      - The mean peak values in Zone 2 capillary electrophoresis findings and hematological parameters for heterozygous, homozygous, and compound heterozygous hemoglobin constant spring (N=389).

      Haematological parametersTypes of Hb CSP-values
      Heterozygous Hb CS (n=359)Homozygous Hb CS (n=2)Compound heterozygous Hb CS (n=28)
      Zone 2 peak on CE0.7±0.44.5±1.11.2±0.8<0.001
      Hb (g/dL)12.8±20.110.9±29.011.1±26.8<0.001
      RBC (1012/L)5.2±0.64.7±2.24.9±1.20.2
      MCV (fl)77.2±3.981.5±16.873.2±8.2<0.001
      MCH (pg)25.0±1.524.4±5.322.7±2.0<0.001

      Values are presented as means ± standard abbreviations (SDs). All pairs of mean scores are significantly different by post hoc test (Tukey test; p<0.001). Hb CS: hemoglobin constant spring, CE: capillary electrophoresis, Hb: hemoglobin, RBC: red blood cell, MCV: mean corpuscular volume, MCH: mean corpuscular hemoglobin

        • View popup
        Table 3

        - Haematological parameters of compound heterozygous hemoglobin constant spring (n=28).

        Coinheritancen (%)Haematological parameters
        Hb (g/dL)RBC (1012/L)MCV (fl)MCH (pg)Hb CS level (%)
        Het alpha 3.717 (60.7)11.9±18.25.2±0.972.8±3.622.9±1.41.1 (0.2)
        Het alpha 4.23 (10.6)11.4±19.45.4±0.767.3±3.121.1±0.90.9 (0.2)
        Hb Adana2 (7.1)4.1±25.51.8±1.389.2±28.424.1±3.73.2 (2.3)
        Beta thal trait*1 (3.6)-----
        Hb Quang Sze*1 (3.6)-----
        Het IVS-1*1 (3.6)-----
        Hb Malay*1 (3.6)-----
        Het alpha SEA*1 (3.6)-----
        Hb D punjab*1 (3.6)-----

        Values are presented as numbers and percentages (%) and means ± standard deviations (SDs).

        Hb: hemoglobin, RBC: red blood cell, MCV: mean corpuscular volume, MCH: mean corpuscular hemoglobin, Hb CS: hemoglobin constant spring, Het: heterozygous, IVS-1: Intervening Sequence-1, SEA: Southeast Asian

        • ↵* No mean and SD value due to only one sample involved.

        • View popup
        Table 4

        - Detection of hemoglobin constant spring by capillary electrophoresis and high-performance liquid chromatography samples (N=389).

        Genotypes of Hb CSCEHPLC
        Zone 2No peakSmall peak
        Heterozygous Hb CS359 (92.3)186 (51.8)173 (48.2)
        Homozygous Hb CS2 (0.5)0 (0.0)2 (100)
        Compound heterozygous Hb CS28 (7.2)3 (10.7)25 (89.3)

        Values are presented as numbers and percentages (%). Hb CS: hemoglobin constant spring,

        CE: capillary electrophoresis, HPLC: high-performance liquid chromatography

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        Evaluation of haemoglobin constant spring phenotypes and their haematological characteristics among high school students in Terengganu, Malaysia
        Sharifatul F. Embong, Sumaiyah Adzahar, Adibah Daud, Mohammad H. Nordin, Sarah A. Halim, Wan Zuhairah W. Embong, Nabilah Rameli, Razan H. Zulkeflee, Rosnah Bahar
        Saudi Medical Journal Mar 2025, 46 (3) 292-298; DOI: 10.15537/smj.2025.46.3.20240771

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        Evaluation of haemoglobin constant spring phenotypes and their haematological characteristics among high school students in Terengganu, Malaysia
        Sharifatul F. Embong, Sumaiyah Adzahar, Adibah Daud, Mohammad H. Nordin, Sarah A. Halim, Wan Zuhairah W. Embong, Nabilah Rameli, Razan H. Zulkeflee, Rosnah Bahar
        Saudi Medical Journal Mar 2025, 46 (3) 292-298; DOI: 10.15537/smj.2025.46.3.20240771
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        Keywords

        • alpha thalassemia
        • haemoglobin constant spring
        • high performance liquid chromatography
        • capillary electrophoresis

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