Normochromatic Anemias Caused by Hemoglobinopathies Flashcards
Hgb S
Amino acid valine replaces glutamic acid on the 6th position of beta globin chain.
Autosomal Co-dominant
inheritance located on chromosome 11. one located on each chromosome. normal or abnormal beta globin chain inherited.
Sickling may also be induced by:
Stress, Hypoxia, Acidosis, Dehydration, Fever, and Exposure to cold
Severity of symptoms of Sickle cell depends on what?
four haplotypes it Hgb S
Hgb C
Amino acid lysine replaces glutamic acid on the 6th position of beta globin chain
Hemoglobin C trait
No clinical complications
40% target cells
60% Hgb A and 40% Hgb C
Hemoglobin SC disease
target cells, nrbcs, polychromadsia, hjb cells, and few sickled cells will occur
SC crystals “washington monument” “gloved hand”
Sickle Cell Anemia Trait vs Disease
Trait: Heterogeneous inheritance of abnormal Hgb
Disease: Homogeneous inheritance of abnormal Hgb
Hgb S forms long, thin polymers in areas of low oxygenation in the:
Spleen, Liver, Kidneys, Joints, and Extremities
When referring to sickle cell anemia, red blood cells:
have a life span of 10-20 days. chronic hemolysis occurs. hematocrit 20-25%
Hemoglobin C disease
Massive spleen.
normochromic/normocytic with a MCHC
Hgb C 80%
Hemoglobin E
2nd most common hemoglobin variant worldwide.
lysine substituted for glutamic acid at the 26th position on the b chain of hgb