Bleeding Disorders Flashcards by Annie Caldwell

Responses in vessel wall damage

Primary
- platelet plug formation
- platelets bind to abnormal surface area for adherence, activation and aggregation
- vWF - bind to cells and surfaces to form an adequate platelet plug
- wall
Secondary
- fibrin plug formation

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Mucosal pattern bleeding is caused by what?

Platelets problems

Von Williebrand problems

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Presentation of coagulation factor bleeding

Bleed into muscles/joints
Different patterns of bleedings into e.g. 
- knees, ankles
- shoulders
- elbows
- less so hips

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Causes of haemorrhagic diathesis

Any quantative or qualitative abnormality 
Inhibition of function of
- platelets
- vWF
- coagulation factors

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What is haemorrhagic diathesis?

The tendency to bleed

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History of bleeding - to ask about

Bruises (girls have easy bruising) 
Epistaxis
Post surgical bleeding
- dental
- circumcision 
- tonsillectomy
- appendicectomy 
Menorrhagia 
- VERY relevant 
- girls who dont have menorrhagia from menarche are then very unlikely to have von Willebrand disease 
PPH
Post trauma 
FH

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Platelet type bleeding will cause….

Mucosal bleeding
Epistaxis (both nostrils)
Purpura
Menorrhagia 
GI bleeding

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Coagulation type bleeding will cause….

Articular - knees, ankles, shoulders
Muscle haematoma
CNS
muscle wasting

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What questions to ask to figure out if it is a congenital or acquired bleeding disorder?

Previous episodes
age of first event
previous surgical challenges
associated history

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Which factor is affected in haemophilia type A?

factor 8

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Which factor is affected in haemophilia type B?

Factor 9

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Genetics of haemophilia

X linked

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In haemophilia, what does the severity of bleeding depend on?

The residual coagulation factor activity

<1% = severe
1-5% moderate = do not bleed spontaneously
5-30% = precautions to be put in place for surgery etc so do not come to too much harm

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Presentation of haemophilia

Hemarthrosis 
- hinge and weight bearing joints 
Muscle haematoma 
- calves, biceps particularly
CNS bleeding (less common)
Retroperitoneal bleeding (less common) 
Post surgical bleeding 
- preventable with clinical management

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Complications of haemophilia

Synovitis
- can result in loss of articular cartilage due to macrophages in the blood producing an inflammatory response
Chronic haemophilic arthropathy
Neurovascular compression (compartment syndromes)
Other sequelae of bleeding (stroke)

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Diagnosis of haemophilia

Clinical 
- present between 6 months - 2 y/o 
- baby walking then stops walking (due to bleeding into joints)
Screening tests for coagulation 
- prolonged APTT 
- normal PT 
Reduced FVIII or FIX 
Genetic analysis

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Treatment of bleeding diathesis

Coagulation factor replacement FVIII/IX (aim to keep trop > 2%)
DDAVP
tranexamic acid
Emphasis on prophylaxis in severe haemophilia
- 1 every 2 weeks or twice a week for Haemophilia A
Gene therapy

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Haemophilia treatment

Splints
physio 
analgesia
synovectomy 
joint replacement

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Haemophilia complications of treatment

Viral infection (HIV, HBV etc)
Development of inhibitors
- Anti FVIII Ab (if severe and have no FVIII antigen, will form a response to the coagulation factor replacement)
- rare in FIX as fewer null mutations
DDAVP
- desmopressin (factor 8 and 9 go up for a day which allows you to have surgery etc)

What does desmopressin/DDAVP do?

Promotes the release of von Willebrand factor

What is DDAVP/Desmopressin contraindicated in?

Elderly 
High risk of vascular disease
Children < 3 y/o 
due to 
- MI 
- hyponatraemia (babies)

How common is von Willebrand disease?

Common - 1 in 200

Presentation of von Willebrand disease

Platelet type bleeding (mucosal)

Pathology of Von Willebrand disease, either ….

Quantative (dont make enough)

Qualitive (doesn’t function right)

Types of Von Willebrand disease

``` Type 1 - quantative deficiency Type 2 (A, B, M, N) - qualitative deficiency determined by the site of the mutation in relation to vWF function Type 3 - severe (complete deficiency) ```

Treatment of von Willebrand disease

vWF concentrate / factor VIII concentrate or DDAVP Tranexamic acid OCP

Causes of acquired bleeding disorders

``` Thrombocytopenia Liver failure Renal failure DIC Drugs (the big group of disorders, most common causing bleeding) - warfarin - heparin - aspirin - clopidogrel - rivaroxaban - apixaban - dabigatran ```

What is thrombocytopenia?

Abnormally low levels of thrombocytes/platelets in blood

Causes of thrombocytopenia

Decreased production - marrow failure (anaemia and thrombocytopenia symptoms) - aplasia - infiltration Increased consumption - Immune - most common - ITP - non immune DIC - inappropriate activation of cascade (using up the platelets and coagulation factors) - burns, sepsis, obstetric emergencies Hypersplenism - massive spleen = platelets get stuck in it - very rarely have a low platelet count (<40)

Presentation of thrombocytopenia

``` Petechiae - mucosal more dangerous ecchymosis mucosal bleeding rare CNS bleeding ```

What level of platelet counts are rarely associated with bleeding disorders?

>40

What does ITP stand for?

Idiopathic thrombocytopenic purpura

What is ITP?

A disorder that can lead to easy or excessive bleeding / bruising - the bleeding resulting from abnormally low levels of platelets

Associations of ITP

Infection (EBC, HIV, glandular fever) Collagenosis lymphoma drug induced (quinine most common)

Treatment of ITP

Steroids IV IgG Splenectomy Thrombopoietin analogues (eltrombopag and romiplostim)

Pathology of liver failure in respect to bleeding

Factor I, II, V, VII, VIII, IX, X, XI Prolonged PT APTT reduced fibrinogen

Treatment of liver failure affecting bleeding

Cholestasis Vit K dept factor deficiency Factor II, VII, IX, X Replacement FFP Vitamin K

In liver failure, where does most bleeding occur?

From structural lesions - varices

Pathology of haemorrhagic disease of the newborn

Immature coagulation systems Vitamin K deficient diet (esp breast) Fatal and incapacitating haemorrhage

How is haemorrhagic disease of the newborn completely prevented?

1mg IM administration of Vit K at birth

How does tranexamic acid work?

Reversibly binds to lysine receptor sites on plasminogen or plasmin, preventing plasmin from binding to and degrading fibrin

What is the most common inherited bleeding disorder?

Von Willebrand disease

Inheritance of VWD

Autosomal dominant

Type 3 VWD is what type of inheritance?

Autosomal recessive

What % of patients have type I VWD?

80%

What is the most common cause of isolated thrombocytopenia?

ITP

What would make you think of haemophilia A/B?

Hemarthrosis without trauma