Mind maps Bleeding disorders Flashcards
Examples
Bernard-soulier syndrome Haemophilia Vitamin K insufficiency Glanzmann's thrombasthenia Von Willebrand Disease
What is Bernard-soulier syndrome
Autosomal recessive bleeding disorder
Deficiency of glycoprotein Ib
Cause of Bernard-soulier syndrome
Hereditary condition that leads to deficiency of glycoprotein Ib
Investigations of Bernard-soulier syndrome
Increased bleeding time
Normal or low platelet count
Conservative treatment of Bernard-soulier syndrome
Patient education
Medical treatment of Bernard-soulier syndrome
Desmospressin may decrease bleeding time
Recombinant activated factor VII
**What are the 4 steps of clot formation
Vessel constriction
Platelet adhesion and aggregation
Blood coagulation
Fibrinolysis
What is Haemophilia
Inherited condition that impairs the body’s ability to coagulate the blood
Types of Haemophilia
Hereditary condition with 2 forms:
Type A = lack of factor VIII
Type B = lack of factor IX
Investigations of Haemophilia
Normal prothrombin time
Increased partial thromboplastin time
Conservative treatment of Haemophilia
Patient education
Avoid aspirin, NSAIDs, heparin and warfarin
Medical treatment of Haemophilia
Replace deficient clotting factor with regular infusions
Diseases that affect the Platelet adhesion and aggregation step of clot formation
Glanzmann’s thrombasthenia
von Willebrand disease
Bernard-Soulier syndrome
Diseases that affect the blood coagulation step of clot formation
Haemophilia
Diseases that affect the fibrinolysis step of clot formation
Antiplasmin deficiency
What is vitamin K insufficiency
Avitaminosis occurring when there is decreased vitamin K1 or K2 or both. This results in:
- decreased synthesis of factors 2, 7, 9, 10
- decreased synthesis of proteins C and S
Causes of vitamin K insufficiency
Drugs e.g. warfarin Malnutrition Malabsorption Alcoholism Cystic fibrosis CKD Cholestatic disease
Investigations of vitamin K insufficiency
Increased prothombin time
Normal or increased partial thromboplastin time
Conservative treatment of vitamin K insufficiecny
Patient education
Dietary advice about food rich in vitamin K
Medical treatment of vitamin K insufficiency
Treat cause
Vitamin K supplements
What is Glanzmann’s thrombasthenia
This is a rare autosomal recessive or acquired autoimmune condition in which platelets are deficient of glycoprotein IIb/IIIa
GpIIb/IIIa binds fibrinogen
What is function of glycoprotein IIb/IIIa
binds fibrinogen
Causes of Glanzmann’s thrombasthenia
Disease of hereditary or acquired autoimmune cause
Investigations of Glanzmann’s thrombasthenia
Increased bleeding time
Conservative treatment of Glanzmann’s thrombasthenia
Patient education
Avoid aspirin and NSAIDs
Medical treatment of Glanzmann’s thrombasthenia
Desmopressin
Recombinant activated factor VII
What is Von Willebrand disease
Hereditary coagulation disorder, which involves a defect in von Willebrand factor (VWF)
What is the most common hereditary coagulation disorder
Von Willebrand disease
What is the function of Von Willebrand factor (VWF)
Bind glycoprotein Ib receptor on platelets to subendothelial collagen
Causes/types of Von Willebrand disease
Hereditary condition
Many types, but most common:
Type 1, Type 2, Type 3 and Type Normandy
Investigations of Von Willebrand disease
Increased activated partial thromboplastin time Increased bleeding time Normal prothrombin time Decreased VWF antigen Decreased factor VIIIc
Conservative treatment of Von Willebrand disease
Patient education
Avoid aspirin and NSAIDs
Medical treatment of Von Willebrand disease
Desmospressin may be useful but is not helpful in type 3 Von Willebrand disease
