Yours

7.4: Hemoglobin C (Hb CC) Disease

A Laboratory Guide to Clinical Hematology · LibreTexts

0%

Contents

    Text

    Size
    100%
    Theme
    Typeface
    Line length
    Spacing

    From LibreTexts, under CC BY-NC 4.0, as the source states it; its equations are written in TeX, which the PDF shows typeset. This page at LibreTexts.

    Cause(s):

    β globin chain amino acid substitution in the 6th position from glutamic acid (Glu) to lysine (Lys).1,2

    Inheritance:

    Autosomal dominant1

    Demographics:

    West Africa1

    Clinical Features:1-3

    See Hemoglobin C under RBC inclusions for additional information.

    Less splenic sequestration and milder chronic hemolysis compared to sickle cell disease. Patients are usually asymptomatic.

    Laboratory Features:1,2

    CBC:

    Hb: Decreased

    Hct: Decreased

    PBS:

    Normochromic, normocytic RBCs

    *Hb C crystals

    Target cells

    nRBCs

    BM:

    N/A

    Hemoglobin Electrophoresis:

    Hb S: >90

    Hb A: None

    Hb A2: Normal

    Hb F: <7%

    *Not always seen, more likely in patients who have had a splenectomy.

    Other Tests:

    Solubility Screen: Negative

    Metasulfite Sickling Test: Negative

    HPLC

    Hemoglobin Electrophoresis

    		  

    References:

    1. Laudicina RJ. Hemoglobinopathies: qualitative defects. In: Clinical laboratory hematology. 3rd ed. New Jersey: Pearson; 2015. p.231–50.

    2. Randolph TR. Hemoglobinopathies (structural defects in hemoglobin). In: Rodak’s hematology clinical applications and principles. 5th ed. St. Louis, Missouri: Saunders; 2015. p. 426-453.

    3. Harmening DM, Yang D, Zeringer H. Hemolytic anemias: intracorpuscular defects. 5th ed. Philadelphia: F.A. Davis Company; 2009. p. 207-29).

    This page titled 7.4: Hemoglobin C (Hb CC) Disease was last modified on Fri, 11 Sep 2026 16:40:04 GMT and is shared under a CC BY-NC 4.0 license and was authored, remixed, and/or curated by Valentin Villatoro and Michelle To (Open Education Alberta) via source content that was edited to the style and standards of the LibreTexts platform.