Bleeding Disorders Flashcards
What is the normal haemostatic response to bleeding?
Primary - platelet plug, vWF
Secondary - fibrin plug
Haemorrhagic diathesis is caused by abnormalities in what?
Platelets
vWF
Coaglation factors
What are common in a history of bleeding?
Bruising Epistaxis Post-surgical bleed Menorrhagia Post-partum haemorrhage Post-trauma haemorrhage
What is an abnormal platelet pattern of bleeding?
Mucosal Epistaxis Purpura Menorrhagia GI bleeds Patechiae (don't blanche) Bruising
What is a coagulation factor pattern of bleeding?
Articular (knee or ankle)
Muscle haematoma
CNS bleeds
Hot, tender, swollen joint
How can you differentiate congenital and acquired bleeding disorders?
Previous episodes
Age of first event
Previous surgical issues
Associated history
What is the cause of Haemophilia A and B?
X-linked gene causing lack of factor VIII (A) or IX (B)
Degree of bleeding/severity in Haemophilia A/B depends on what?
Residual coagulation factor activity
Clinical features of Haemophilia?
Haemarthrosis (hinge joints) Muscle hematoma CNS bleed Retroperitoneal bleed Post-surgical bleed
What complications are associated with Haemophilia?
Synovitis
Chronic Haemophilic Arthropathy
Compartment syndrome
Stroke
How is Haemophilia diagnosed?
Prolonged APTT Normal PTT Reduced Factor VIII or IX Gene analysis CVS/Amniocentesis in carrier mother
How is Haemophilia bleeding managed?
Missing factor replaced
Tranexamic acid
Desmopressin
Prophylaxis
How is Haemophilic Arthritis treated?
Splints Physiotherapy Analgesia Synovectomy Joint replacement
How does von Willebrand disease present?
Platelet type bleeding (mucosal)
Autosomal (1 in 200)
Outline von Willebrand disease types
Type 1: minor
Type 2: (qualitative) determined by site of mutation
Type 3: complete deficiency
How is von Willebrand disease managed?
vWF concentrate
Desmopressin
Tranexamic acid
Oral contraceptive (to control period)
Name 3 acquired bleeding disorders?
Thrombocytopenia Liver failure Renal failure DIC Antiplatelet/anticoagulant drugs
What are the causes of decreased platelet production in thrombocytopenia?
Marrow failure
Aplasia
Infiltration
What are the causes of increased platelet destruction in thrombocytopenia?
Immune thrombocytopenic purpura
Non-immune DIC
Hypersplenism
How does Thrombocytopenia present?
Petechiae
Ecchymosis - bruising
Mucosal bleeding
CNS bleeding
How is Immune thrombocytopenic purpura managed?
Steroids
IV IgG
Splenectomy
Thrombopoeitin analogues
How does Liver failure associated bleeding disorder present?
Prolonged PT, APTT
Reduced Fibrinogen
Cholestasis
Variceal bleeding
How is Liver failure associated bleeding disorder managed?
Vitamin K
Replacement FFP
How is Haemorrhagic Disease of the Newborn managed?
Vitamin K at birth
Spontaneous bleeding only occurs in what?
SEVERE haemophilia
DDAVP is associated with what complications?
Risk in Hyperlipidemic/MI patients
Hyponatremia in babies
What is the difference between qualitative and quantitative vW disease?
Quantititive - “amount of factor missing”
Qualitative - “variation depends on location of mutation”
What are the causes of DIC (Disseminated Intravascular Coagulation)?
Massive trauma, infection, surgery, pregnancy complications etc