Bleeding Disorders

Question 1

How do you approach a bleeding disorder?

Answer 1

?have they got one eg history of bleeding (often, timing, circumstance)
?how severe is it eg appropriate or not
?what is the pattern eg platelet (mucosal, epistaxis, purpura) or coagulation factor (articular, muscle haematoma, CNS)
?congenital or acquired eg prev episodes, age, surgeries, associated history
?if congenital, mode of inheritance eg affected family members, sex

Question 2

What are the symptoms of and treatment of haemophilia?

Answer 2

Symptoms: haemarthrosis, muscle haematoma, CNS/retroperitoneal bleeding, post surgical bleeding
BLEED Tm: replace coag factor, desmopressin, tranexamic acid, gene therapy, prophylaxis
HAEM Tm: splints, physiotherapy, analgesia, synovectomy, joint replacement

Question 3

What are the symptoms of treatment of Von willebrands disease?

Answer 3

Symptoms: variable in severity, platelet type bleeding eg easy bruising, epistaxis, purpura, menorrhagia
Tm: VWF concentrate, or desmopressin, transexamic acid, topical applications, oral contraceptive

Question 4

What are some other congenital clotting factor and platelet abnormalities?

Answer 4

Haemorrhagic disease of the newborn
Lack of vitamin K at birth leads to fatal and incapacitating haemorrhage
Tm: administration of vitamin K at birth (IM vs PO)

Question 5

What are the symptoms, causes and management of thrombocytopenia?

Answer 5

Causes: decrease in production of platelets eg marrow failure, aplasia infiltration or increase in consumption of platelets eg immune ITP, non immune DIC, hypersplenism
Symptoms: petechiae, ecchymosis, mucosal bleeding
Tm: treat underlying cause, blood or platelet transfusions, medications, surgery, plasma exchange

Question 6

What are the acquired causes of haemostatic failure?

Answer 6

Immune Thrombocytopenic Purpura (associated with infections, collagenosis, lymphoma, drug induced)
Tm: steroids, IV IgG, splenectomy, thrombopoeitin analogues

Liver Failure (coagulation factors produced in the liver, prolonged prothrombin, activated partial thromboplastin time, reduced fibrinogen =  can become vit K deficient)
Tm: replacement fresh frozen plasma, vitamin K