Hemophilia and Coagulation Disorders PDF
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This document provides information on Hemophilia and Coagulation Disorders. It covers various aspects of the disease, including the types and inheritance patterns. Details on the severity of the disorder and associated complications are also detailed.
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Hemophilia and Coagulation Disorders HEMOPHILIA Often called ‘the disease of kings’ because it was carried by many members of Europe’s royal family. Queen Victoria of England was a carrier of Hemophilia 2 TYPES Disease...
Hemophilia and Coagulation Disorders HEMOPHILIA Often called ‘the disease of kings’ because it was carried by many members of Europe’s royal family. Queen Victoria of England was a carrier of Hemophilia 2 TYPES Disease Factor deficiency Inheritance Hemophilia A VIII X linked recessive Hemophilia B IX X linked recessive Hemophilia C XI Autosomal recessive Parahemophilia V Autosomal recessive 4 X-linked recessive Hemophilia A (FVIII def): 80-85% Hemophilia B (FIX def) Mutations of the clotting factor gene Family H/O bleeding common, - generally affects males on the maternal side - 1/3 no family history – due to new mutations Severity of Hemophilia is defined by measured level of clotting factor activity Distribution Clotting factor activity Severe hemophilia 50%
