Haemotology Conditions B

Question 1

Polycythaemia Vera - Description

Answer 1

malignant proliferation of of common myeloid progenitor cells

Question 2

Polycythaemia Vera - Cause (1)

Answer 2

1) JAK2 mutation (acquired) (95%)

Question 3

Polycythaemia Vera - Risk Factors (2)

Answer 3

1) >40 years old

2) Budd-Chiari syndrome

Question 4

Polycythaemia Vera - Pathophysiology (5)

Answer 4

1) gain of function JAK2 mutation
2) increased signalling of haemopoeitic growth factors via JAK2 (e.g. erythropoietin)
3) malignant proliferation of common myeloid progenitor cells
4) erythrocytes, granulocytes, megakaryocytes don’t require erythropoietin to avoid apoptosis
5) excess erythrocytes, granulocytes, megakaryocytes

Question 5

Polycythaemia Vera - Symptoms (8)

Answer 5

1) asymptomatic
2) erythromelalgia (burning sensation of fingers and toes)
3) pruritus (esp. in warm weather)
non-specific hyperviscosity symptoms
4) headaches
5) dizziness
6) fatigue
7) tinnitus
8) visual disturbances

Question 6

Polycythaemia Vera - Signs (3)

Answer 6

1) hepatomegaly
2) splenomegaly
3) plethoric

Question 7

Polycythaemia Vera - Complications (6)

Answer 7

1) thrombosis
2) haemorrhage
3) hypertension
4) angina
5) intermittent claudication
6) gout

Question 8

Polycythaemia Vera - Investigations (2/2)

Answer 8

initial
1) genetic screening (JAK2)
2) FBC (polycythaemia >99th percentile, leucocytosis, thrombocytosis)
consider
1) serum erythropoietin (low)
2) bone marrow biopsy (erythrocyte, granulocyte, megakaryocyte proliferation)

Question 9

Polycythaemia Vera - Management (0/4/1)

Answer 9

medical
1) low dose aspirin
2) allopurinol (gout)
3) chemotherapy (venesection not tolerated)
4) radioactive phosphorous (>70 years old, acute leukaemia risk)
surgery
1) venesection (500ml weekly)

Question 10

Disseminated Intravascular Coagulation - Description

Answer 10

systemic activation of coagulation due to systemic inflammatory response syndrome

Question 11

Disseminated Intravascular Coagulation - Causes (5)

Answer 11

systemic inflammatory response syndrome

1) sepsis
2) trauma
3) obstetric emergency
4) pancreatitis
5) malignancy

Question 12

Disseminated Intravascular Coagulation - Pathophysiology (6)

Answer 12

1) systemic inflammatory response syndrome
2) systemic cytokine release
3) systemic tissue factor release
4) systemic thrombin release
5) systemic excessive positive feedback thrombin release (leading to depletion)
6) systemic excessive fibrin clot formation

Question 13

Disseminated Intravascular Coagulation - Symptoms (1)

Answer 13

1) confusion —> coma

Question 14

Disseminated Intravascular Coagulation - Signs (5)

Answer 14

1) tachycardia
2) hypotension
3) oliguria
4) bruising
5) ecchymoses

Question 15

Disseminated Intravascular Coagulation - Complications (6)

Answer 15

1) haemorrhage
2) cardiac tamponade
3) haemothorax
4) haemorrhagic stroke
5) acute kidney injury
6) gangrene

Question 17

Disseminated Intravascular Coagulation - Investigations (3/0)

Answer 17

initial

1) FBC (thrombocytopenia)
2) coagulation tests (high PTT, low fibrinogen)
3) high D-dimer

Question 18

Immune Thrombocytopenia Purpura - Description

Answer 18

thrombocytopenia due to autoimmune destruction of platelets

Question 19

Immune Thrombocytopenia Purpura - Causes (Primary/Acute) (2)

Answer 19

children

1) viral infection (e.g. varicella zoster, measles)
2) vaccination (e.g. MMR)

Question 20

Immune Thrombocytopenia Purpura - Causes (Secondary/Chronic) (4)

Answer 20

adults (esp. women of childbearing age)

1) HIV
2) hepatitis
3) autoimmune (e.g. SLE)
4) chronic lymphocytic leukaemia

Question 21

Immune Thrombocytopenia Purpura - Pathophysiology (3)

Answer 21

1) IgG autoantibodies form to platelets and megakaryocytes
2) macrophages phagocytose opsonised platelets and megakaryocytes
3) thrombocytopenia

Question 22

Immune Thrombocytopenia Purpura - Symptoms (3)

Answer 22

1) epistaxis
2) gum bleeding
3) menorrhagia

Question 23

Immune Thrombocytopenia Purpura - Signs (2)

Answer 23

1) purpura

2) bruising

Question 24

Immune Thrombocytopenia Purpura - Complications (1)

Answer 24

1) haemorrhage (rare)

Question 25

Immune Thrombocytopenia Purpura - Investigations (2/1)

Answer 25

initial
1) FBC (thrombocytopenia)
2) bone marrow biopsy* (thrombocytopenia, normal megakaryocytes)
consider
1) platelet autoantibodies (60-70%)

Question 26

Immune Thrombocytopenia Purpura - Management (0/3/1)

Answer 26

medical
1) IV IgG (1st line)
2) prednisolone (1st line)
3) azathioprine (2nd line) 
surgery
1) splenectomy (2nd line)

Question 27

Thrombotic Thrombocytopenia Purpura - Description

Answer 27

thrombocytopenia due to increased platelet consumption

Question 28

Thrombotic Thrombocytopenia Purpura - Causes (5)

Answer 28

1) idiopathic
2) autoimmune (e.g. SLE)
3) iatrogenic (e.g. quinine)
4) malignancy
5) pregnancy

Question 29

Thrombotic Thrombocytopenia Purpura - Pathophysiology (5)

Answer 29

1) autoimmune destruction of ADAMS-13
2) decreased von-Willebrand factor degradation
3) increased von-Willebrand factor multimers
4) increased platelet adhesion and aggregation
5) thrombocytopenia

Question 30

Thrombotic Thrombocytopenia Purpura - Symptoms (8)

Answer 30

1) fever
neurological
2) focal neurology
3) seizures
4) coma
abdominal 
5) abdominal pain
6) nausea
7) vomiting
8) diarrhoea

Question 31

Thrombotic Thrombocytopenia Purpura - Signs (2)

Answer 31

1) purpura

2) fluctuating cerebral function

Question 32

Thrombotic Thrombocytopenia Purpura - Complications (3)

Answer 32

1) thromboembolic events (e.g. DVT, PE, MI, stroke)
2) haemolytic anaemia
3) acute kidney injury

Question 33

Thrombotic Thrombocytopenia Purpura - Investigations (2/0)

Answer 33

initial

1) FBC (thrombocytopenia)
2) high lactate dehydrogenase

Question 34

Thrombotic Thrombocytopenia Purpura - Management (0/4/2)

Answer 34

medical
1) vitamin B9 supplement
2) low dose aspirin
3) IV methylprednisolone
4) IV rituximab (2nd line)
surgery
1) plasma exchange (removed ADAMS-13 autoantibodies, replace ADAMS-13) (1st line)
2) splenectomy (3rd line

Question 35

Heparin Induced Thrombocytopenia - Description

Answer 35

thrombocytopenia due to immune reaction to heparin

Question 36

Heparin Induced Thrombocytopenia - Causes (1)

Answer 36

1) heparin (esp. high dose or unfractionated)

Question 37

Heparin Induced Thrombocytopenia - Pathophysiology (4)

Answer 37

1) IgG antibodies form to heparin/PF4 complex
2) heparin/PF4/IgG complexes activate platelets
3) increased platelet adhesion and aggregation
4) thrombocytopenia

Question 38

Heparin Induced Thrombocytopenia - Signs (2)

Answer 38

1) necrosis at heparin injection site

2) recent surgery/trauma

Question 39

Heparin Induced Thrombocytopenia - Complications (2)

Answer 39

1) thromboembolic events (e.g. DVT, PE, MI, stroke)

2) bleeding —> haemorrhage

Question 40

Heparin Induced Thrombocytopenia - Investigations (1/1)

Answer 40

initial
1) FBC (thrombocytopenia)
consider
1) HIT antigen assay* (positive)

Question 41

Heparin Induced Thrombocytopenia - Management (1/2/0)

Answer 41

conservative
1) stop heparin
medical
1) vitamin K supplement
2) antiacoagulant (e.g. warfarin)

Question 42

Disseminated Intravascular Coagulation - Management (0/2/4)

Answer 42

medical
1) treat underlying cause
2) heparin
surgery
1) red cell transfusion
2) platelet transfusion
3) fresh frozen plasma transfusion (coagulation factors)
4) cryoprecipitate (fibrinogen)