Different Hemoglobin combinations-Nr and Abn
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Sickle Cell Trait (6
21 1 glu-val)
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?Sickle cell trait is the heterozygous form of the disease
?Represents a combination of HbA and HbS.
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?Individuals with HbS trait are usually asymptomatic or havemild anemia.
Pathophysiology of Sickle Cell Disease
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HbC (2C2) -? Modification - Glutamic acid to lysine at 6HbD (2d2) -?Modification - Glutamic acid to glutamine at 121
HbE (2e2) -?Modification - Glutamic acid to lysine at 26
Hb Abnormal variants- Quantitative Abnormality
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Decreased or absent synthesis of a particular globin chain (structurally normal)
i.e. Thalassemias
Thalassemia
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? Common in population of African descent, Southeast Asian, Italians,Middle Eastern
? Two types-
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- thalassemia (Reduced production of - globin chains-HbH or Hb Bart's)
- thalassemia (Reduced production of - globin chains-Cooley's Anemia)
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-THALASSEMIA-THALASSEMIA Trait (Minor): - / , - / -
-THALASSEMIA
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-THALASSEMIA Major (4, HbH disease): - - / - ,Hemoglobin Barts (4 , y-globin tetramer, lethal):
With four-gene deletion: - -/- -
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-THALASSEMIA
? One or both -globin genes have mutations that cause partial ( +) or total loss ( O) of
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- chain production.?Decreased synthesis of -globin chains disturbs the balance between the two chains
and -chains precipitate - producing membrane damage and early destruction.
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Normal Hb combinations/compounds
Oxyhemoglobin
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? Hb reacts with four molecules of O2 to form Hb4O8.
Hb4 + O2 Hb4O2 , Hb4O2 + O2 Hb4O4, Hb4O4 + O2 Hb4O6, Hb4O6 + O2 Hb4O8
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https://youtu.be/eor6EK_JP40Carbaminohemoglobin
Carbaminohemoglobin is a compound of hemoglobin with CO2 (carbon dioxide), and is one
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of the forms in which carbon dioxide is transported in the blood at tissue level.10% of carbon dioxide is carried in blood this way, bound to the globin protein.
Abnormal Hb compound-Methemoglobinemia
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?When Fe++ is oxidized to Fe ++ + form in Hemoglobin, this is called as Methemoglobin.
? The affinity of oxygen is impaired in ferric form of iron and binding of oxygen to methemoglobin
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results in an increased affinity for oxygen in the remaining heme sites that are in ferrous state withinthe same tetrameric hemoglobin unit.
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MethemoglobinemiaSign and Symptoms by Methemoglobin formation % :
?Less than 10% metHb - No symptoms
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? 10-20% metHb ?
? >50% metHb -
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TreatmentCarboxy-hemoglobin
Carbon monoxide is a colourless, odourless, and tasteless gas that is lighter than air and can be fatal to life.
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It has a greater affinity for hemoglobin ( 200 times) than oxygen does. It displaces oxygen and quickly bind
to Hb
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SOURCESCarboxy-hemoglobin
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CLINICAL SIGNSThe signs of carbon monoxide poisoning vary with concentration and length of exposure.
TREATMENT
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Glycated hemoglobin
A very minute amount of hemoglobin A is continuously and
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slowly binds with glucose irreversibly and Non enzymatically witheach chain. This is known as `Glycated Hemoglobin' or HbA1c.
A higher glucose concentration results in more HbA1c.
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The normal reference range is approximately 4?5.9%
Self Asessment
Sickle cell trait is the ...............................of the disease, which has mild anemia.
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Decreased or absent synthesis of a particular globin chain that is structurally ...............
Thalassemis major has severe anemia, the type is ...........................................................due to
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enhanced hemolysisHemoglobin Barts has a ....................................and cannot release oxygen to tissues.
-thalassemia major is also called .................. and Microcytic hypochromic anemia
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Thank you