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UNIT 6 - THALASSAEMIA - Coggle Diagram
UNIT 6 - THALASSAEMIA
PATHOGENESIS
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DEFECTIVE GLOBIN CHAIN SYNTHESIS: reduced synthesis of one or more types of normal hemoglobin polypeptide chains
REDUCED HEMOGLOBIN PRODUCTION: hemoglobin molecule is imcomplete, leading to a reduction in functional hemoglobin
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IMPAIRED ERYTHROPOIESIS AND HEMOLYSIS: inadequate RBC production and ineffective erythropoiesis occur alongside increased RBC destruction
RESULTING ANEMIA: combination of reduced RBC production, ineffective erythropoiesis, and hemolytic leads to chronic anemia
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MANAGEMENT
TRANSFUSION
GOALS OF TRANSFUSION
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AVOIDANCE OF ADVERSE REACTIONS, INCLUDING TRANSMISSION OF INFECTIOUS AGENTS
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DEFINITION
an inherited genetic disorder of the blood, it is a disorder that results from abnormalities in the synthesis of the hemoglobin molecule contained in RBC
causes ineffective erythropoiesis and hemolysis, leading to variable degrees of anemia and extramedullary hematopoiesis
causes bone changes, impaired growth and iron overload
COMPATIBILITY TESTING
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BEFORE EACH TRANSFUSION: it is necessary to perform a full cross match and screen for new antibodies
transfusion from first-degree relatives should be avoided because the risk of developing antibodies that might adversely affect the outcome of later bone marrow transplant
IRON CHELATION
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WITHOUT TREATMENT, THALASSEMIC CHILDREN DEVELOP SERIOUS COMPLICATIONS WHICH CAN BECOME FATAL
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DIETARY ADVICE
a high calorie, high protien diet is advised to maintain normal weight and health
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SPLENECTOMY: anaemia and hypotransfusion result in hypersplenism causing frequent transfusions, as hemoglobin level drops rapidly