Bleeding Disorders Flashcards
3 Types of Bleeding disorders
Vascular defects (easy bruising) Platelet disorders (low or abnormal function) Coagulation disorders (Factor deficiency) Mixed (DIC)
Cause of superficial bleeding?
Vascular defects (easy bruising) Platelet disorders (low or abnormal function)
Cause of Bleeding immediately after injury
Vascular defects (easy bruising) Platelet disorders (low or abnormal function)
Cause of bleeding into deep tissues?
Coagulation disorders (Factor deficiency)
Cause of delayed but severe bleeding after injury
Coagulation disorders (Factor deficiency)
Cause of prolonged bleeding?
Coagulation disorders (Factor deficiency)
Congenital vascular defects?
Osler-Weber-Rendu sydrome
Ehlers-Danlos sydrome (Connective tissue disorders)
Acquired vascular defects?
Senile purpura
Infection (meningococcal, measles, dengue)
Steroids
Scurvy (Perifollicular haemorrhages)
4 underlying mechanisms of platelet disorders?
Acquired abnormal platelet function
Congenital abnormal platelet function
Reduced production of platelets
Increased destruction of platelets
Normal platelet count?
150-400x10^9 g/L
Acquired abnormal platelet function conditions?
Aspirin
Cardiopulmonary bypass
Uraemia
Congenital abnormal platelet function conditions?
Platelet storage pool disease
Thrombasthenia (Glycoprotein deficiency)
Thrombocytopenic conditions
Bone marrow failure
Auto-immune Thrombocytopenic Purpura
Drugs eg. heparin, DIC, HUS, TTP
Age of onset of acute and chronic ITP
Acute - 2-6 yrs
Chronic - Adults
Treatment of chronic ITP
IV Ig, Steroids, Splenectomy
Factor VIII Deficiency
Haemophilia A
Inheritance pattern of Haemophilia A
X-linked recessive
1/10000 males
Diagnosis of Haemophilia A
Increased APTT
Normal PT
Reduced Factor VIII assay
Management of Haemophilia A
Avoid NSAIDs and IM injections
Desmopressin - increases vWF release (factor VIII carrier)
Factor VIII concentrates for life
Factor IX Deficiency
Haemophilia B
Inheritance pattern of Haemophilia B
X-linked recessive
1/50,000 males
Management of Haemophilia B
Factor IX concentrates
Reduced platelet function and factor VIII
Von Willebrand’s disease
Inheritance pattern of vW DIsease
Autosomal dominant
1/10,000 males
Diagnosis of vW Disease
Increased APTT Increased Bleeding time Decreased Factor VIII Decreased vWF Ag. Normal INR Normal Platelets
Management of vW Disease
Desmopressin
vWF
Factor VIII Concentrates
Widespread activation of coagulation
DIC
Clotting factors and platelets are consumed
Cuases of DIC
Malignancy Trauma Sepsis Obstetric complications Toxins
Diagnosis of DIC
Low plts Low Fibrinogen High FDP/D Dimer Long PT Long INR
Management of DIC
Treat the cause Tranfusions FFP Platelets Cryo
Liver Disease
Reduced synthesis of factor II, V, VII, IX, X, XI and fibrinogen
Reduced Vit K absorption
Abnormalities of platelet function
Factors reduced in Vit K deficiency
II, VII, IX, X
Protein C, S
Cause of Vit K Deficiency
Warfarin
Vit K malabsorption/malnutrition
Abx Therapy
Biliary Obstruction
Treatment of Vit K Deficiency
IV Vitamin K
Fresh frozen plasma for acute haemorrhage