HEMOGLOBIN ABNORMALITIES

HEMOGLOBIN ABNORMALITIES

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Normal HB made up of haem and two  polypeptide globin chains, Alpha and Beta chain.

Classifications of hemoglobin abnormalities:

I - Abnormal chain production.

II – Abnormal chain structure.
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THALASSEMIA:

Normal hemoglobin has the balance 1:1 in production of Alpha and Beta chain.

Talassaemias have one or multiple gene defects that lead to reduce the production of one or more globin chain.

The production of imbalanced globin chain leads to precipitation of globin chain inside red cells. All that results in ineffective erythropoiesis and hemolysis.
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TYPES OF THALASSEMIA:

I – a-Talassaemia with reduced a-chain synthesis.

II – b-Talassaemia with reduced a-chain synthesis.
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B-THALASSEMIA:

I – Trait B-Talassaemia:

Asymptomatic stat.

Here stores of iron are in normal situation.

II – Intermia B-Talassaemia:

Moderate anemia and does not require regular blood transfusion.

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Signs and symptoms:

Splenomegaly – leg ulcers – bone deformation – gallstones.

   III – major b-Talassaemia:

Present in the first year of the life with:

Recurrent  infections and severe anemia – hepatosplenomegaly.

Thalassaemic face:

Enlarged maxilla- prominent frontal and parietal bones.
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LAB. INVESTIGATION OF THALASSEMIA:

FILM AND BLOOD COUNT:

SHOW HUPOCHROMIC/MICROCYTIC ANEMIA.

Reticulocytes count and nucleated red cells in the peripheral  circulation are raised.

DIAGNOSIS :

Is made by the hemoglobin electrophoresis showing an increase of HB-F And reducing of HB-A.

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TREATMENT AND MANGENENT:

I - Blood transfusion. That leads to

Iron overload and damaging the endocrine glands liver, heart => cardiac failure and pancreases.

II - Iron chelating agent.

III – Bone marrow transplantation in some cases.

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Internal medicine.

Dr.Zafer Maklad

zafernm@yahoo.com