Bleeding Disorders

Question 1

3 processes stopping bleeding

Answer 1

Vasoconstriction
Platelets plugging gaps
Coagulation cascade

Question 2

Bleeding vasc defects

Answer 2

Osler-Weber-Rendu (hereditary haemorrhagic telangectasia)
Connective tissue disorders (e.g. Ehlers-Danlos)
Infection
Steroids
Scurvy
HSP

Question 3

Bleeding platelet disorders

Answer 3

Aplastic/megaloblastic anaemia
Marrow infiltration/supression

Immune thrombocytopaenia and other autoimmune e.g. SLE, drug-induced, viruses
DIC, TTP, sequestration

Myeloproliferative disease, NSAIDs, increased urea all hinder platelet function

Question 4

Bleeding coagulation disorders

Answer 4

Haemophilia, von Willebrand's
Anticoagulants
Liver disease
DIC
Vit K deficiency

Question 5

What is Haemophilia A

Answer 5

X-linked recessive factor VIII gene inversion with high rate of new mutations (30% no family hx)

Question 6

Haemophilia A presentation

Answer 6

After surgery/trauma, often early in life with bleeds into joints/muscles causing arthropathy + haematomas

Question 7

Haemophilia A diagnosis

Answer 7

Increased APTT and decreased factor VIII assay

Question 8

Haemophilia A management

Answer 8

Avoid NSAIDs + IM injections
Desmopressin 0.3mcg/kg/12h IVI over 20 mins for minor bleeds
Major bleeds recombinant factor VIII so levels are 50% of normal
Life threatening bleeds need 100% of normal

Question 9

What is Haemophilia B (Christmas disease)

Answer 9

Inherited x-linked recessive factor IX deficiency behaves like haemophilia A
Treat with recombinant factor IX

Question 10

What is acquired haemophilia

Answer 10

AutoAbs interfere with factor VIII, treat with steroids

Question 11

Acquired haemophilia tests

Answer 11

Inc APTT
Factor VIII autoAbs
Factor VIII activity <50%

Question 12

Bleeding disorder liver causes

Answer 12

Decreased synthesis of clotting factors
Decreased vit K absorption
Platelet function abnormalities

Question 13

Treatment bleeding due to malabsorption

Answer 13

IV vitamin K (10mg) if that is deficient

In acute haemorrhage human prothrombin complex or FFP

Question 14

Immune thrombocytopenia pathology

Answer 14

Anti-platelet autoAbs
Can be acute (2 wks self-limiting purpura usually in children) or chronic (fluctuating bleeding/ purpura/ epistaxis/ menorrhagia)

Question 15

ITP tests

Answer 15

inc megakaryocytes in marrow

Anti-platelet Abs

Question 16

ITP treatment

Answer 16

If mild, none
Prednisolone 1mg/kg/d, reduce after remission
No platelet transfusion as Abs destroy
If relapse, splenectomy or B-cell depletion with rituximab, Eltrombopag (oral thrombopoietin-receptor agonist) or Romiplostim (injectable thrombopoietin analogue)

Question 17

Most common inherited thrombophilia

Answer 17

Activated protein C resistance (factor V Leiden)

Question 18

Thrombophilia causes

Answer 18

Inherited:
Activated protein C resistance (factor V Leiden)
Prothrombin gene mutation
Protein C/S deficiency (vit K dependent factors)
Antithrombin deficiency

Acquired:
Anti-phospholipid syndrome
Oestrogens/pregnancy
Thrombocytosis/polycythaemia

Question 19

Thrombophilia presentations

Answer 19

Arterial thrombosis/MI <50yrs
Unprovoked/recurrent VTE (/with pregnancy/contraceptives)
Neonatal thrombosis/ recurrent foetal loss
Unusual site e.g. mesenteric/portal vein

Question 20

Thrombophilia management

Answer 20

Anticoagulate acute thrombus, only preventative anticoags if recurrent with no other RFs
Counsel on VTE RFs
Prophylaxis (heparin) in pregnancy/ pre-surgery

Question 21

Disseminated intracvascular coagulation tests

Answer 21

Low platelets

Prolonged prothrombin time, APTT, and bleeding time

Question 22

What is post-thrombotic syndrome

Answer 22

After DVT painful heavy calves, pruritis, swelling, varicose veins

Question 23

Post-thrombotic syndrome treatment

Answer 23

Graduated compression stockings, only as treatment and not to be used as prophylaxis

Question 24

von Willebrand’s disease treatment

Answer 24

Tranexamic acid for mild bleeding
Desmopressin induces vWF release from endothelial cells
Factor VIII concentrate