Haem

Question 1

Blood film post splenectomy

Answer 1

Howell- Jolly bodies
Pappenheimer bodies
Target cells
Irregular contracted erythrocytes

Question 2

Fever, chills during blood transfusion

Answer 2

Non-haemolytic febrile reaction

Question 3

Non-haemolytic febrile reaction management

Answer 3

Slow or stop the transfusion
Paracetamol
Monitor

Question 4

Pruritus, urticaria during blood transfusion

Answer 4

Minor allergic reaction

Question 5

Minor allergic reaction management

Answer 5

Temporarily stop the transfusion
Antihistamine

Question 6

Non-haemolytic febrile reaction

Answer 6

Thought to be caused by antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage

Question 7

Minor allergic reaction

Answer 7

Thought to be caused by foreign plasma proteins

Question 8

Hypotension, dyspnoea, wheezing, angioedema during blood transfusion

Answer 8

Anaphylaxis

Question 9

blood transfusion anaphylaxis management

Answer 9

Stop the transfusion
IM adrenaline
ABC support
oxygen
fluids

Question 10

blood transfusion anaphylaxis management

Answer 10

Stop the transfusion
IM adrenaline
ABC support
oxygen
fluids

Question 11

Fever, abdominal pain, hypotension during blood transfusion

Answer 11

Acute haemolytic reaction

Question 12

Acute haemolytic reaction management

Answer 12

Stop transfusion
Confirm diagnosis
check the identity of patient/name on blood product
send blood for direct Coombs test, repeat typing and cross-matching
Supportive care
fluid resuscitation

Question 13

Acute haemolytic reaction

Answer 13

ABO-incompatible blood e.g. secondary to human error

Question 14

Pulmonary oedema, hypertension during blood transfusion

Answer 14

Transfusion-associated circulatory overload (TACO)

Question 15

TACO management

Answer 15

Slow or stop transfusion
Consider intravenous loop diuretic (e.g. furosemide) and oxygen

Question 16

TACO

Answer 16

Excessive rate of transfusion, pre-existing heart failure

Question 17

Hypoxia, pulmonary infiltrates on chest x-ray, fever, hypotension during blood transfusion

Answer 17

Transfusion-associated acute lung injury (TRALI)

Question 18

TRALI management?

Answer 18

Stop the transfusion

Oxygen and supportive care

Question 19

TRALI

Answer 19

Non-cardiogenic pulmonary oedema thought to be secondary to increased vascular permeability caused by host neutrophils that become activated by substances in donated blood

Question 20

Warfarin

Answer 20

reduction of vitamin K to its active hydroquinone form
* Prolong PT (extrinsic)
* Monitor using INR (as it involve PT and more efficient)
* 2,7,9,10
* War with your gf, she will become ur ex (extrinsic)

Question 21

Unfractionated heparin

Answer 21

• 9,10,11,12
• High risk of osteoporosis and HIT
• Useful in renal failure and where high risk of bleeding (anticoagulation can be terminated rapidly)
• Protamine sulphate can reverse heparin effect completely
• Monitor APTT

Question 22

LMWH (dalteparin, enoxaparin)

Answer 22

• 10
• Low risk of osteoporosis and HIT
• Not in renal failure
• Protamine sulphate reverse LMWH partially
• Monitor factor Xa (as the name RivaroXaban) however frequent monitoring not needed
• 1st line in VTE prophylaxis and treatment and ACS

Question 23

Platelet transfusion for thrombocytopenia before surgery or an invasive procedure. Aim for plt levels of:

Answer 23

> 50×109/L for most patients
50-75×109/L if high risk of bleeding
100×109/L if surgery at critical site

Question 24

Normoblastic causes of macrocytic anaemia

Answer 24

alcohol
liver disease
hypothyroidism
pregnancy
reticulocytosis
myelodysplasia
drugs: cytotoxics

Question 25

Megaloblastic causes of macrocytic anaemia

Answer 25

Vit B12 deficiency Folate deficiency (e.g. secondary to methotrexate)

Question 26

Acute intermittent porphyria presentation

Answer 26

abdominal: abdominal pain, vomiting neurological: motor neuropathy psychiatric: e.g. depression hypertension and tachycardia common

Question 27

Acute intermittent porphyria

Answer 27

rare autosomal dominant condition caused by a defect an enzyme involved in the biosynthesis of haem

Question 28

Tranexamic acid administration in major haemorrhage

Answer 28

IV bolus followed by slow infusion effective if within 3 hours

Question 29

What medication is contraindicated in T2DM and G6PD deficiency

Answer 29

sulphonyureas

Question 30

Extrinsic pathway

Answer 30

Activation of TF (tissue factor) and factor VII

Question 31

Intrinsic pathway

Answer 31

Factors VIII, IX, XI and XII activation

Question 32

Common pathway

Answer 32

Factors V and X activation (converts prothrombin into thrombin) Where thrombin then converts fibrinogen into fibrin

Question 33

PT what pathway

Answer 33

extrinsic

Question 34

APTT what pathway

Answer 34

intrinsic

Question 35

Haemophilia A

Answer 35

Factor VIII deficiency

Question 36

Haemophilia B

Answer 36

Factor IX deficiency

Question 37

Vit K factors

Answer 37

II, VII, IX, X

Question 38

APTT and PT in vw

Answer 38

normal PT, abnormal APTT, prolonged bleeding time

Question 39

Haemophilia A and B inheritance

Answer 39

X-linked recessive

Question 40

Von willebrand disease features

Answer 40

Excess or prolonged bleeding from minor wounds Excess or prolonged bleeding post-operatively Easy bruising Menorrhagia Epistaxis GI bleeding

Question 41

TTP pentad

Answer 41

Fever Microangiopathic haemolytic anaemia (MAHA) Thrombocytopaenic purpura CNS involvement: headache, confusion, seizures AKI

Question 42

TTP treatment

Answer 42

Plasma exchange ASAP Caplacizumab

Question 43

Factor V leiden cause

Answer 43

mutation in clotting factor V, which becomes resistant to inactivation by protein C meaning it won't turn off clotting

Question 44

Protein C deficiency cause

Answer 44

Protein C (and its co-factor, protein S) inactivates clotting factors V and VIII. Inactivating mutations in protein C or S increase the risk of thrombosis

Question 45

Antithrombin III function

Answer 45

inhibits factor IIa, Xa, IXa, and XI

Question 46

Alpha thalassaemia presentation

Answer 46

Jaundice Fatigue Facial bone deformities

Question 47

Alpha thalassaemia management

Answer 47

blood transfusions stem cell transplant splenectomy

Question 48

Beta thalassaemia minor presentation

Answer 48

isolated microcytosis and mild anaemia typically asymptomatic

Question 49

Beta thalassaemia major presentation

Answer 49

Severe symptomatic anaemia at 3-9 months of age when levels of foetal haemoglobin fall Frontal bossing Maxillary overgrowth Extramedullary hematopoiesis (hepatosplenomegaly).

Question 50

Beta thalassaemia management

Answer 50

regular blood transfusions consider iron chelating agent