Hemophilia and Coagulation Disorders PDF

Summary

This document provides an overview of hemophilia and coagulation disorders. It covers different types of hemophilia, their inheritance patterns, and clinical presentations. The document also touches on the management and causes of these medical conditions.

Full Transcript

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%

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