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    1 - Sickle-Cell Diseases (SCD)

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    Ahmed Ali
    Hdh

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    1 - Sickle-Cell Diseases (SCD)

    Uploaded by

    Ahmed Ali
    6 views14 pages
    Hdh

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    1- Sickle-cell diseases (SCD)

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    Hdh

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    © All Rights Reserved
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    Download as pdf or txt
    6 views14 pages

    1 - Sickle-Cell Diseases (SCD)

    Uploaded by

    Ahmed Ali
    Hdh

    Copyright:

    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
    of 14

    Sickle-cell diseases (SCD)

    Developed by-Dr.Abdulrazzaq Othman Alagbare


    M.D M.S.c C.P - Lecturer of Hematology and Immunohematology
    Sickle-cell syndromes
    • Sickle-cell disorders are those conditions in which the red cells become
    sickle-shaped when they are subjected to low oxygen tension
    • Abnormalities occur in the beta chain ,
    • the Beta chain is affected by a point mutation.
    • in the 6th position on the  chain the normal glutamic acid has been
    changed with valine
    Sickle cell types

    1-Sickle cell disease -HbSS

    2- Sickle cell trait –HbAS


    in the 6th position on the 
    chain the normal glutamic acid
    has been changed with valine
    The Hb S is insoluble and forms
    crystals when exposed to low
    oxygen tension

    The RBC live only 20-30 days


    Cont. Sickle-cell syndromes

    The normal Hb is soluble

    The Hb S is insoluble and forms crystals when exposed to low oxygen tension.

    The red cells sickle may block the different areas of the microcirculation or large
    vessels causing infarcts of various organs.
    Cont. Sickle-cell syndromes
    The sickle cell in capillaries when exposed to low oxygen tension
    The following may be caused

    • Hb  crystalized
    • Hb  is insoluble
    • The RBC Loss  deformability
    • Sickled cells  block the small vessels
    Cont. Sickle-cell syndromes
    Clinical syndrome types Type Presentation
    Sickle-cell Disease Homozygous  Onset at 6–9 months;
    – HbSS βs/βs  severe anaemia (Hb 3-5g/dl), with
     painful crises,
     infarction,
     Haemolytic crises
     symptoms are linked to anemia
    and pain
    Cont. Sickle-cell syndromes
    Clinical syndrome types Type Presentation

    Sickle-cell trait - Heterozygous  Usually asymptomatic;


    HbAS β/βs  mild microcytic hypochromic anaemia
    (Hb 10–11 g/dL)

    Individuals with sickle cell disease undergo autosplenectomy during


    adolescent years
    Laboratory finding in sickle cell anemias

    Test HbSS HbAS

    Hb 6-8g/dl 10-11 g/dl

    RBCc low Normal

    WBC (WBC shift to the left) 10-20x103 /cumm Normal

    Retic-count Over 20% Normal

    ESR Zero Zero


    Laboratory finding in sickle cell anemias

    Blood film:

    • Normochromic, normocytic anemia,


    • Target cells,
    • NRBC,
    • Sickle cells+++
    • Reticulocytosis

    microcytic hypochromic anemia not observed in HbSS


    Sickle cell screening test Types
    Test Types chemicals Action:
    1.sickling test sodium sodium metabisulfate remove the O2
    metabisulfate around the RBC

    Sickling test
    Sickle cell screening test Types

    Test Types chemicals Action:

    2- Solubility test sodium dithionite The normal solubility test


    Hb is soluble
    The HbS is
    Insoluble
    And cause
    turbidity
    NOTE about the sickling tests
     Not diagnostic
     Not say if the patient
    homozygous or heterozygous
    Sickling test solubility test
     It is positive in each case
    combined with sickle cells
    Clinical syndrome Electrophoresis Result
     it is the diagnostic test
    HbA HbA2 HbF Pathological Hb
    Sickle-cell Disease – Zero Normal 10% HbSS 90%
    HbSS
    Sickle-cell trait - HbAS 50-60% Normal 10% HbS 30-40%

    Notes
    It is very important to take care about the following categories during
    the lab work
     During pregnancy
     Before anesthesia
    ‫الحمد هلل رب‬
    ‫العالمين‬
    ‫هذا الجهد ابتغاء وجه‬
    ‫هللا الكريم‬

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