Haematology

Question 1

Management of transfusion reactions

Answer 1

• Stop transfusion
• Check patient ID against component label
• Consider anaphylaxis, Circulatory overload (TACO), acute haemolytic transfusion reaction (AHTR), lung injury (TRALI), bacterial infection, etc

Question 2

Rx of transfusion associated circulatory overload (TACO)

Answer 2

Slow rate, IV diuretic + O2

Question 3

Management of immediate haemolytic reactions

Answer 3

• Notify blood services to investigate
• O2
• IV fluids
• Diuretics
• Ionotropes
• Dialysis

Question 4

Management of Transfusion Related Acute Lung Injury (TRALI)

Answer 4

• O2
• Resp. support
• IV fluids
• Notify blood services to investigate/intiate recalls

Question 5

Management of bacterial infection related to transfusions

Answer 5

• IV antibiotics
• O2
• IV fluids
• Notify blood services to investigat/initiate recalls

Question 6

RX of HDN

Answer 6

By careful monitoring

• Antibody titres
• Doppler US
• Intrauterine transfusions

Question 7

Management of severe allergic reaction (transfusion)

Answer 7

• Return unit to blood bank
• O2
• Salbutamol nebuliser
• IV Chlorophenamine (10mg slowly)
• ?ADRENALINE?

Question 8

Rx of hereditary spherocytosis

Answer 8

• Folic acid
• Transfusion
• Splenectomy

Question 9

Commonesnt disease causing enzymopathy in the world

Answer 9

G6PD Deficiency

Question 10

Rx for an aplastic crisis

Answer 10

• Ususally self limitting, <2wks

- MAY need a transfusion

Question 11

Rx of a sequestration crisis

Answer 11

Urgent transfusion required

Question 12

Rx of a “painful” crisis

Answer 12

• Analgesia (severe = opiates)
• Hyrdation
• O2
• Consider antibiotics

Give analgesia within 30mins of presentation + avoid pethidine (demerol)

Question 13

Rx of a chest crisis

Answer 13

• Resp. support
• Antibiotics
• IV fluids
• Analgesia
• Transfusion

Question 14

Disease modifying drug for sickle cell disease

Answer 14

Hydroxycarbamide

Question 15

Rx of thalassaemia major

Answer 15

• Transfusions, 4-6 weekly
• Careful of iron overloading
• Iron chelation therapy
• Bone marrow transplantaion = curative

Question 16

What is used in iron chelation therapy

Answer 16

Oral Deferiprone + Subcutaneous Desferrioxamine

Ascorbic acid increases urinary excretion of iron

Question 17

Management of haemolytic anaemia

Answer 17

-Folic acid (support marrow function)
Correct cause
-Immunosupression if autoimmune (?Prednisolone?)(treat trigger CLL, lymphoma)
-Remove site of destruction (splenectomy)
-Treat sepsis, leaky prosthetic valce, malignancy etc

-Consider transfusion

Question 18

Rx for megaloblastic anaemia

Answer 18

Replace vitamin

• B12 deficiency = B12 IM injection, loading dose then 3 monthly maintenance
• Folate deficiency = Oral folate replacement

Question 19

Rx of acute presentation of arterial thrombosis

Answer 19

• Thrombolysis (TPA, tissue plasminogen activator)

- Antiplatelet/anticoagulant drugs (Clopidogrel/warfarin)(asecondary prevention)

Question 20

Rx for Von Willebrand Disease

Answer 20

• vWF concentrate or Desmopressin (DDAVP)

- Tranexamic acid

Question 21

Rx of ITP

Answer 21

Only if symptomatic

• Prednisolone
• Thrombopoietin analohues (Eltrombopag)
• IV IgG
• Splenectomy (cures =80%)
• Immnosuppression if splenectomy fails (azathioprine)

Question 22

Rx of AML

Answer 22

-Support care
-Anti-leukaemic chemotherapy
-Stem cell transplantation (allogenic)
-Targeted antibodies (midostaurin + Myelotarg anti-CD3 with Calicheomycin)
(Tretinoin in APL (acute promyelocytic leukemia) )

Question 23

Rx of chronic myeloid leukaemia

Answer 23

Direct inhibitors of BCR-ABL first line in all patients nowadays

Tyrosine kinase inhibitors

• Imatinib
• Dasatinib
• Nilotinib
• Busitinib
• Ponatinib

Allogenic transplant (few now) (only in TKI failures)

Question 24

Rx for polycythaemia vera

Answer 24

• Venesection to keep haematocrit below 0.45 in men + 0.43 in women
• Aspirin
• Hydroxcarbamide

Question 25

Rx for essential thrombocythaemia

Answer 25

- Aspirin | - Hydroxyurea

Question 26

What antibiotic + anti-fungal prophylaxis is given to reduce the risk of sepsis in haematological malignancy

Answer 26

- Ciprofloxacin (antibiotic) | - Fluconazole (anti-fungal)

Question 27

What anti-viral+ PJP prophylaxis is given to reduce the risk of sepsis in haematological malignancy

Answer 27

- Aciclovir (anti-viral) | - Co-trimoxazole

Question 28

5 supportive measures that reduce the risk of sepsis in haematological malignancy (not prophylactic therapy)

Answer 28

- Stem cell rescure/transplant - Protective enviroment (laminar flow rooms) - IV immunoglobulin replacement - Vaccination - Growth factors (G-CSF) (and prophylactic therapy)

Question 29

Management of neutropenic sepsis

Answer 29

- Broad spectrum IV antibiotics (Gentamicin + Tazocin) - If no response in 72hrs add IV antifungal (Caspofungin) - CT chest/abdo/pelvis to find source - Modify Rx based on culture results

Question 30

In neutropenic sepsis, if a gram +ve organism is identified what should be added to the Rx

Answer 30

Vancomycin or Teicoplanin

Question 31

Rx of type I hypersensitivity

Answer 31

- Antihistamines - Corticosteroids - Adrenaline - Allergen avoidance

Question 32

Rx for contact dermatitis

Answer 32

- Topical steroid | - Allergen avoidance

Question 33

Rx of rejection

Answer 33

- Corticosteroids - Anti-thymocyte globulin - Plasma exchange