Pathology - Hemophilia A

Anusha Uddandam
Mind Map by Anusha Uddandam, updated more than 1 year ago
Anusha Uddandam
Created by Anusha Uddandam over 3 years ago
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Pathology - Hemophilia A

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Pathology - Hemophilia A
1 Intro
1.1 Sex - linked Recessive Disorder
1.2 Reduced amount or activity of factor VIII associated with life-threatening bleeding
1.3 Bleeding is due to inadequate coagulation and inappropriate clot removal
2 Mode Of Inheritance
2.1 Sex - linked Recessive Disorders
2.2 Causative mutations: deletions, insertions, inversions, point mutations
3 Clinical Features
3.1 Hemarthrosis
3.1.1 Frequent and spontaneous hemorrhage into joints
3.2 Hemorrhage to soft tissues
3.3 Prolonged bleeding followinging trauma
4 Lab Findings
4.1 Reduced: Factor VIII Assay
4.2 Increased: Clotting Time, Activated Partial Thromboplastin Time
4.3 Normal: Bleeding Time, Platelet Count, Prothrombin Time, Fibrinogen Assay, FDP
5 Complications
5.1 Due to Hemophilia
5.1.1 Deforming Arthritis and Contractures
5.1.1.1 Due to repeated bleeding into joints
5.1.1.2 Organization and fibrosis of intramuscular hematomas --> contractures of involved muscles
5.2 Due to Therapy
5.2.1 Viral Hepatitis
5.2.1.1 Hepatitis B,C & D in patients who received multiple transfusions of FFP/Cryoprecipitate
5.2.2 AIDS
5.2.2.1 In people who received FFP before screening tests for AIDS was available
5.2.3 Factor VIII Inhibitors
5.3 Causes of Death in Hemophilia
5.3.1 Prolonged bleeding
5.3.2 Intracranial Hemorrhage
6 Treatment of Hemophlia
6.1 Factor VIII Concentrate
6.2 Recombinant factor VIII
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