Bleeding disorders Flashcards
Do patients with problems in primary haemostasis present in the same way as those with problems in those with secondary haemostasis?
No
What is haemorrhagic diathesis?
Any quantitative or qualitative abnormality
Inhibition of what can be associated with abnormal bleeding?
Platelets
vWF
Coagulation factors
How is severity of disorder determined?
Depends what it takes to make you bleed
What is important to find out about bleeding history?
Has the patient actually got a disorder Severity Pattern of bleeding Congenital or acquired Mode of inheritance
What are features of bleeding?
Bruising Epistaxis Post-surgical bleeding Menorrhagia Post-partum haemorrhage Post-trauma
What is the most important question to ask?
Previous surgery - dental as well, circumcision
What may be an indication of mild bleeding disorder?
After minor procedures ie immunisation
What is an indication of severe bleeding disorder?
Spontaneous bleeding
What is platelet type bleeding?
Dysfunction in platelets and vWF
How may platelet type bleeding present?
Epistaxis
Purpura
Menorrhagia
GI
How may coagulation factor type bleeding present?
Articular
Muscle haematoma
CNS
How can you determine if bleeding disorder is congenital or acquired?
Previous episodes?
Age at first event
Previous surgical challenges
Associated history
What should be asked about family history?
Family members with similar history
Sex - determine sex linked disorder
Are haemophilia A and B X linked?
Yes
How is severity of haemophilia A and B determined?
Coagulation factor activity:
5-30% = mild
1-5% = moderate
<1% = severe
Does moderate haemophilia A and B bleed spontaneously?
No
What are clinical features of haemophilia?
Haemarthrosis Muscle haematoma CNS bleeding Retroperitoneal bleeding Post surgical bleeding
What joint is worst effected in haemophilia?
Ankle joint
What drives syonvial hyertrophy in haemophilia?
Blood cells being phagocytosed excites inflamamtory cells to produce cytokines
What are clinical complications of haemophilia?
Synovitis
Chronic haemohilic arthropathy
Neurovascular compression - compartment syndromes
Other sequelae of bleeding
How is haemophilia diagnosed?
Clinical diagnosis
Prolonged APTT, normal PT, reduced factor VIII or factor IX
Genetic analysis
How is haemophilia treated?
Coagulation factor replacement Now almost entirely recombinant products DDAVP Tranexamic acid Emphasis on prophylaxis in severe haemophilia Gene therapy?
What are complications of haemophilia treatment?
Viral infection - HIV, HBV, HCV, etc
Anti factor VIII antibodies produced, rarely factor IX
DDAVP - MI, hyponatraemia in babies
What type of bleeding is vWF disease?
Platelet type
What affects qualitative disorder?
Where the mutation arises on the gene
How can vWF be treated?
vWF concentrate or DDAPV
Tranexamic acid
Topical applications
OCP for menorrhagia
What can cause decreased production in thrombocytopenia?
Marrow failure
Aplasia
Infiltration
What can cause increased consumption in thrombocytopenia?
Immune ITP
Non immune DIC
Hypersplenism
What is ITP associated with?
Infections
Collagenosis
Lymphoma
Drug induced
How is ITP treated?
Steroids
IV IgG
Splenectomy
Thrombopoietin analogues
What is affected in liver failure?
Factor I, II, V, VII, VIII, IX, X, XI
Prolonged prothrombin time, reduced fibrinogen
What causes haemorrhagic disease of the newborn?
Baby has immature coagulation systems
Particularly vit K deficiency
How is haemorrhagic disease of the newborn prevented?
Vit K injection immediately on birth
