Bleeding Disorder

Question 1

Haemorrhagic Diathesis

Answer 1

Any quantitative or qualitative abnormality due to inhibition of function of Platelets, vWF, coagulation factors.

Question 2

Best questions to ask in terms of History of Bleeding

Answer 2

Bruising 
Epistaxis
Post-surgical bleeding (dental surgery, circumcision, tonsillectomy, appendicectomy, adenoidectomy). 
Menorrhagia
Post-partum haemorrhage
Post-trauma 
Family history of bleeding disorder

Question 3

How to determine the severity of bleeding

Answer 3

What makes you bleed. Is the severity of the bleeding out of context with the severity of the insult.

Question 4

Platelet type bleeding disorder

Answer 4

Thrombocytopenia and Mucosal bleeding

• Epistaxis
• Purpura
• Menorrhagia
• GI

Question 5

Coagulation bleeding disorder

Answer 5

Articular
Muscle Haematoma
CNS bleeding

Question 6

Congenital or Acquired

Answer 6

Previous Episodes ?
Age at first event
Previous surgical challenges
Associated History

Question 7

Age of presentation of haemophilia

Answer 7

Between 6 months and 2 years

Question 8

Hereditary Disorder

Answer 8

Family members with a similar history and the sex of the people afflicted with haemophilia.

Question 9

Haemophilia A (more common) and B

Answer 9

Both X-lined with identical phenotypes.
The severity of bleeding depends on the residual coagulation factor activity

<1% severe
1-5% moderate
5-30% mild

Question 10

Prevalence of Haemophilia A

Answer 10

1:10,000

Question 11

Prevalence of Haemophilia B

Answer 11

1:60,000

Question 12

Clinical features of haemophilia

Answer 12

Haemarthrosis (especially in the hinge joint) 
Muscle Haematoma
CNS bleeding 
Retroperitoneal bleeding 
Post surgical bleeding
Muscle wasting in the calves and thighs

Question 13

Knee damage in haemophilia

Answer 13

Acute inflammatory response, the macrophages produce pro-inflammatory cytokines which results in synovitis

End stage haemophilic arthropathy

Question 14

Clinical Complications of Haemophilia

Answer 14

Synovitis in the knee
Chronic Haemophilic Arthropathy
Neurovascular Compression (compartment syndrome)
Stroke

Question 15

Diagnosis of Haemophilia

Answer 15

Clinical Presentation - child fails to walk on limbs due to pain in joints

Prolonged APTT

Normal prothrombin time

Normal BT

Reduced FVIII or FIX

Genetic analysis

Question 16

Haemophilia Treatment

Answer 16

Coagulation factor replacement FVIII/IX which are now mostly recombinant products.

Desmopressin (releases stored vWF into the circulation, especially useful in procedures)

Tranexamic Acid

Emphasis on using concentrates to reduce chance of bleeding

Synovectomy

Question 17

Development in Inhibitors

Answer 17

AntiFVIII antibody development (30% of boys with severe haemophilia A)

Question 18

von Willebrand Disease

Answer 18

Common (1in200)
Autosomal inheritance
Platelet type bleeding
Quantative (type 1) and qualitative (type 2 mutation in binding sites resulting in futile vWF) abnormalities of vWF

Question 19

Type 3 vWD

Answer 19

Severe complete deficiency

Question 20

Treatment of vWD

Answer 20

vWF concentrate or DDAVP
Tranexamic Acid
Topical applications
OCP

Question 21

Cause of thrombocytopenia

Answer 21

Decreased production of platelets (marrow failure, aplasia, infiltration)

Increased consumption (immune ITP, non-immune DIC, hypersplenism)

Question 22

Idiopathic thrombocytopenic purpura associated with

Answer 22

Infection esp EBV,HIV
Collagenosis
Lymphoma

Drug induced

Question 23

Blood count Idiopathic thrombocytopenic purpura

Answer 23

Thrombocytopenia is the only feature in the blood

Increased platelet consumption despite normal bone marrow

Question 24

Treatment of Idiopathic thrombocytopenic purpura

Answer 24

Steroids, IV IgG, splectomy, thrombopoietin analogues (eltrombopag, romiplostim stimulate platelet pre-cursor cells)

Question 25

Liver Failure

Answer 25

Haemorrhagic and pro-thrombotic disorder. Reduced procoagulatns and anticoagulants in the liver results in either disorder. Renal failure, infection etc trigger

Question 26

Treatment of Liver Failure

Answer 26

Replacement FFP, vitamin K, Factor II, VII, IX, X

Question 27

Haemorrhagic Disease of the newborn

Answer 27

Immature coagulation systems. Vitamin K deficient diet. Resulting in fatal and incapacitating haemorrhage. Completely preventable by administration of vitamin K at birth.