Bleeding Disorders

Question 1

Important questions to ask in Bleeding Hx

Answer 1

Does the Pt actually have a bleeding disorder
How severe is the DIsorder
Pattern Of bleeding
Is the disorder congenital or acquired: prev episodes, age at first onset
Mode of inheritance

Question 2

Features relevant to a Hx of bleeding

Answer 2

Bruising
Epistaxis
Post Sx bleeding
Menorrhegia
Post-trauma bleeding
Post-partum Bleeding

Question 3

Inheritance of Thrombophilia A and B

Answer 3

X-Linked

Question 4

Clinical Features of haemophilia

Answer 4

Haemarthrosis 
Muslce haematoma
Retroperitoneal Bleed
Post-Surgical Bleeding
CNS Bleed

Question 5

Further Complications may include

Answer 5

Synovitis
Chronic haemophillic arthropathy
Neurovascular compression and compartment syndrome
Other sequelae of bleeding (stroke)

Question 6

Dx of Haemophilia

Answer 6

Often Clinical
Increased APTT and normal PT (intrinsic pathway is affected)
Low Factor 8 = Type A
Low Factor 9 = Type B
Genetic analysis may be helpful

Question 7

Management of Haemophilia

Answer 7

Replace Missing Factors - Recombinant 8 or 9
Avoid anticoags
Desmopressin - increased VwB
Tranexamic Acid

Question 8

Von Willibrand Disease

Answer 8

Abnormality in V Wb factor with attached F8
Type 1 = Quantitative defect
Type 2 = Qualitative Defect
Type 3 = Severe (complete) defect

Question 9

Mx of V Wb deficiency

Answer 9

Desmopressin or V wb concentrate

Tranexamic acid

Question 10

What is Thrombocytopaenia

Answer 10

Decrease in platelet production: BM failure
Increase in consumption or destruction: IP, DIC, Hypersplenism
Congestion: HSM

Question 11

Symptoms of thrombocytopaenia

Answer 11

Mucosal Bleeding

Bruising and petechiae

Question 12

What is ITP

Answer 12

Immune thrombocytopaenia purpura
Anti-platelet antibodies reduce platelet numbers rapidly

Common in Children 2 weeks post infection (URTI)
Chronic in adults (F>M)

Question 13

ITP is assoc. with

Answer 13

Infection - esp. HIV/EBV
Lymphoma
Drug use

Question 14

Investigation of ITP

Answer 14

peripheral blood smear - Anti-platelet antibodies

Coombs test for platelets

Question 15

Management of ITP

Answer 15

Steroids - Prednisolone
IvIG - may halt platelet drop

In severe bleeds - platelet transfusion may be helpful but Abs will just destroy them again
in condition of chronic standing - splenectomy

Question 16

Disseminated Intravascular Coagulopathy (DIC)

Answer 16

widespread activation of coagulation – consumption of CLotting factors (mainly V and VIII) –> overactive bleeding

Question 17

Featured of DIC

Answer 17

Bruising
Bleeding
Renal Failure

Question 18

Ix of DIC

Answer 18

Decreased Fibrinogen
Decreased Platelets
Increased PT and APTT
D-Dimer (fibrogin degredation products)
Schistocytes of Peripheral FIlm

Question 19

Treatment of DIC

Answer 19

Replace Fibrinogen (cryoprecipitate)
FFP to replace Clotting Factors
Replace platelets if <50
All-transretinoic acid will reduce mortality