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INTRODUCTION - Hemoglobinopathies

Developed by-Dr.Abdulrazzaq Othman Alagbare


M.D M.S.c C.P - Lecturer of Hematology and Immunohematology
What is hemolytic anemia
anemia's that result from an increase in the rate of red cell destruction
Hemolytic anemias can be classified by the

1-Mode of Hereditary (or) Acquired


transmission

2-Type of defect Intrinsic red cell Extrinsic


(or) environmental’
defects,
change
Hemolytic anemias can be classified as

Inherited hemolytic anemia

Characterized with
• They are genetic
• They has no therapy
• They are permanent
• They are chronic
• They starting early in the life some starting after 7 month of the birth
• Some needs other factors to starting
2-Acquired hemolytic anemia
Characterized with
Destruction of red blood cells (RBCs) not due to genetic or congenital disorder
• They has therapy
• They are not permanent
• They are acute
• They starting late in the life
Caused by factors during the life such as
• Malaria
• Antibodies against RBC
• Toxins
• Chemicals, Drugs
Types of Inherited hemolytic anemia
Hemoglobinopathies:  related to Hb disorders - structural alteration of
the globin - HbSS, HbEE, HbCC
Thalassemias:  related to Hb chains disorders

Enzymopathies:  related to enzymes deficiency of RBC (G6PD


and PK)
Membrane disorders:  related to Membrane structure defect
 e.g., hereditary spherocytosis
About 5% of the population in the world are carriers of hemoglobinopathies
What is the evidence of active bone marrow in cases of hemolytic anemia?

Presence of
‫الحمد هلل رب‬
‫العالمين‬
‫هذاعمل ابتغي به‬
‫وجه هللا الكريم‬

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