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    2 - HEMOGLOBIN HBCC, HbEE

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    Ahmed Ali
    Gwge

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    2 - HEMOGLOBIN HBCC, HbEE

    Uploaded by

    Ahmed Ali
    4 views10 pages
    Gwge

    Original Title

    2- HEMOGLOBIN HbCC, HbEE

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    © © All Rights Reserved

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    Gwge

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    Download as pdf or txt
    4 views10 pages

    2 - HEMOGLOBIN HBCC, HbEE

    Uploaded by

    Ahmed Ali
    Gwge

    Copyright:

    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
    of 10

    HEMOGLOBIN HbCC-HbEE

    1
    Developed by-Dr.Abdulrazzaq Othman Alagbare
    M.D M.S.c C.P - Lecturer of Hematology and Immunohematology
    HEMOGLOBIN HbCC
    Definition and HbC  caused by a mutation in β chains (β6 Glu→ Lys) which
    causes is associated Insoluble hemoglobin like HbSS.
    Hemolytic anemia varies from moderate to severe, similar With
    crystals forming in or out of RBC and folded cells

    2
    HEMOGLOBIN HbCC

    Diagnostic test Electrophoresis: HbCC Hemoglobin trait HbAC

    HbA Nil HbA: 55-60 %

    HbA2: Normal HbA2: Normal

    HbF: 7% HbF: Normal

    HbC: >90 % HbC: 35-45 %


    3
    HEMOGLOBIN E–β-THALASSEMIA
    Definition HbE  caused by a mutation in β chains (β26 Glu→ Lys)
    and causes which is associated with the phenotype of β thalassaemia.

    Hemolytic anemia varies from moderate to severe, similar to β-


    thalassemias  Very small RBC with MCV lower than 60 fl

    4
    HEMOGLOBIN E–β-THALASSEMIA

    Diagnostic Electrophoresis: HbEE (Homo.) Hemoglobin trait (Hetro.) HbEA

    test HbA Nil HbA: 55-60 %

    HbA2: Normal HbA2: Normal

    HbF: 7% HbF: Normal

    HbE: >90 % HbE: 35-45 %


    5
    β- chain - Hemoglopinopathies
    Hb Defect Hb type RBC changes
    HbSS Valine Replace Glu at Produce insoluble Hb Sickling shape
    6th position of β- chain
    HbCC Lysin Replace Glu at 6th Produce insoluble Hb Crystals inside the RBC
    And folded cells
    position of β- chain
    HbEE Lysin Replace Glu at Produce unstable Hb Microcyte with MCV 55
    26th position of β- fl
    6
    chain
    Hemoglopinopathies combinations

    β/βs β/βc β/βs β/β0

    βs/βc β0/βs

    Sickle-cell / HbC disease  Sickle-cell / β-thalassaemia

    Hemoglopinopathies combinations OCCUR among the heterozygous individuals


    7
    Hemoglopinopathies combinations

    SC disease is a variant of homozygous sickle


    Clinical phenotype of
    cell anemia (SS disease) which is of almost
    HbE/β thalassaemia resembles
    equal severity and has a similar clinical
    either thalassaemia major
    presentation

    8
    SUMMARY ……Sickle-cell syndromes
    Mostly the following clinical syndrome types Type
    are observed

    Sickle-cell Disease – HbSS Homozygous βs/βs

    Sickle-cell trait - HbAS Heterozygous β/βs

    Sickle-cell haemoglobin / C disease βs/βc

     Sickle-cell / β-thalassaemia βs/β0 9


    ‫الحمد هلل رب‬
    ‫العالمين‬
    ‫هذا الجهد ابتغاء وجه‬
    ‫هللا الكريم‬

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