Haematology & Oncology

Question 1

In beta-thalassaemia major, iron chelation therapy (e.g. desferrioxamine) is important to prevent the complications of _____________ due to repeat transfusions

Answer 1

iron overload

Question 2

Examples of iron chelation agents are:

Answer 2

Deferiprone
Deferoxamine
Deferasirox

Question 3

_______ is used in Wilson’s disease as a copper chelator.

Answer 3

Trientine

Question 4

Beta-thalassaemia minor and major result from mutations in the ___________ on chromosome ___ which lead to reduced (beta-thalassemia minor) or absent (beta-thalassemia major) production of beta-globulin chains.

Answer 4

HBB gene ; 11

Question 5

classical findings in beta-thalassaemia major

Answer 5

frontal and parietal bossing, chipmunk face and hepatosplenomegaly.

[These signs are caused by compensatory extramedullary haematopoiesis.]

Question 6

X ray finding associated with Beta Thalassemia

Answer 6

hair-on-end appearance on skull X-ray.

Question 7

________________ is a key laboratory finding in Beta Thalassemia

Answer 7

Microcytic anaemia (low MCV)

Question 8

The superior vena cava can become obstructed by external compression, most commonly from __________

Answer 8

bronchogenic carcinoma.

Question 9

Features of SVC Obstruction

Answer 9

dyspnoea is the most common symptom
swelling of the face, neck and arms - conjunctival and periorbital oedema may be seen
headache: often worse in the mornings
visual disturbance
pulseless jugular venous distension
[The development of chest wall collaterals (sometimes called ‘caput medusae’) is a classic sign as the body attempts to establish alternative venous drainage pathways]

Question 10

Causes of SVC Obstruction

Answer 10

common malignancies: small cell lung cancer, lymphoma
other malignancies: metastatic seminoma, Kaposi’s sarcoma, breast cancer
aortic aneurysm
mediastinal fibrosis
goitre
SVC thrombosis

Question 11

Methotrexate therapy may result in a ______________ secondary to folate deficiency

Answer 11

megaloblastic macrocytic anaemia

Question 12

Megaloblastic causes of macrocytic anaemia

Answer 12

vitamin B12 deficiency
folate deficiency
e.g. secondary to methotrexate

Question 13

Normoblastic causes of macrocytic anaemia

Answer 13

alcohol
liver disease
hypothyroidism
pregnancy
reticulocytosis
myelodysplasia
drugs: cytotoxics

Question 14

Antithrombin III is a vitamin K-independent protease that inhibits ______, ____, ________

Answer 14

factor II, IX and X

Question 15

Acute haemolytic transfusion reactions are usually the result of RBC destruction by ___________

Answer 15

IgM-type antibodies

Question 16

A low haemoglobin may be seen in CML as ________________________

Answer 16

there is an increased haemolysis of red blood cells.

Question 17

Diagnostic Ix for CML

Answer 17

bone marrow biopsy is diagnostic and is likely to show the t(9;22) chromosomal translocation commonly seen in CML (the Philadelphia chromosome)

Question 18

How does Imatinib treat CML

Answer 18

imatinib, an inhibitor of the BCR-ABL fusion protein created with the t(9;22) chromosomal translocation

Question 19

Haemophilia is an_______________disorder of coagulation.

Answer 19

X-linked recessive

Question 20

Haemophilia A is due to a deficiency of _______ whilst in haemophilia B (Christmas disease) there is a lack of _______

Answer 20

factor VIII ;factor IX

Question 21

Features of haemophilia

Answer 21

haemoarthroses
haematomas
prolonged bleeding after surgery or trauma

Question 22

blood tests for haemophilia show what?

Answer 22

prolonged APTT
bleeding time, thrombin time, prothrombin time normal

Question 23

Smudge/smear cells on blood film are a classical finding of ________

Answer 23

chronic lymphocytic leukaemia (CLL)

Question 24

Burkitt’s lymphoma is common in children and is also associated with exposure to the _______________. Burkitt’s lymphoma also causes a _______________ appearance on blood film.

Answer 24

Epstein-Barr virus (EBV) ; ‘starry sky’

Question 25

Pathophysiologyy of Thrombotic thrombocytopenic purpura

Answer 25

abnormally large and sticky multimers of von Willebrand's factor cause platelets to clump within vessels

Question 26

in TTP there is a deficiency of _________ (a metalloprotease enzyme) which breakdowns ('cleaves') large multimers of von Willebrand's factor

Answer 26

ADAMTS13

Question 27

Features of TTP

Answer 27

rare, typically adult females fever fluctuating neuro signs (microemboli) microangiopathic haemolytic anaemia thrombocytopenia renal failure

Question 28

Causes of TTP

Answer 28

post-infection e.g. urinary, gastrointestinal pregnancy drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel, aciclovir tumours SLE HIV

Question 29

The most common form of leukaemia in adults is _______

Answer 29

Chronic lymphocytic leukamaemia (CLL)

Question 30

Chronic lymphocytic leukaemia is the most common adult leukaemia, representing around ___% of cases

Answer 30

25

Question 31

Chronic myeloid leukaemia represents around __% of adult leukaemias

Answer 31

15

Question 32

_____________ is the most common childhood leukaemia.

Answer 32

Acute lymphocytic leukaemia (ALL)

Question 33

______ cancer is the most common primary tumour that metastasises to the bone

Answer 33

Prostate

Question 34

The intrinsic pathway of the clotting cascade is assessed by measurement of ____

Answer 34

aPTT

Question 35

The extrinsic pathway is best assessed by measurement of the ___________

Answer 35

Prothrombin time (PT).

Question 36

Which clotting factors will be most rapidly consumed in disseminated intravascular coagulation ?

Answer 36

Factor V and VIII {Factors V and VIII are both labile factors with relatively short half-lives (12-36 hours for Factor V and 8-12 hours for Factor VIII)}

Question 37

Causes of DIC

Answer 37

sepsis trauma obstetric complications e.g. aminiotic fluid embolism or hemolysis, elevated liver function tests, and low platelets (HELLP syndrome) malignancy

Question 38

DIC : A typical blood picture includes:

Answer 38

↓ platelets ↓ fibrinogen ↑ PT & APTT ↑ fibrinogen degradation products schistocytes due to microangiopathic haemolytic anaemia

Question 39

What happens in sideroblastic anaemia?

Answer 39

It happens when red cells fail to completely form haem (a precursor to haemoglobin) and lead to iron deposits in the mitochondria that form a ring around the nucleus called a ring sideroblast.

Question 40

the presence of anti-intrinsic factor antibodies points to ________________

Answer 40

pernicious anaemia

Question 41

________ increases the HbF levels and is used in the prophylactic management of sickle cell anemia to prevent painful episodes

Answer 41

Hydroxyurea

Question 42

How does Tranexamic acid work?

Answer 42

Tranexamic acid is an anti-fibrinolytic drug, which works to prevent major haemorrhage by binding to plasminogen and prevents plasmin from breaking down fibrin clots

Question 43

Burkitt's lymphoma

Answer 43

t(8:14) positive Epstein-Barr virus 'starry sky' appearance on microscopy ; lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells. Burkitt's lymphoma is associated with the c-myc gene translocation, usually t(8:14).

Question 44

The features of a peripheral blood film in iron deficiency anaemia are:

Answer 44

Microcytic cells Hypochromic cells Pencil cells Target cells

Question 45

Schistocytes can be caused by ____________

Answer 45

mechanical heart valves

Question 46

Rouleaux can be seen in ____________ + _______________

Answer 46

chronic liver disease ; malignant lymphoma.

Question 47

Tear drop poikilocytes can be seen in __________

Answer 47

myelofibrosis

Question 48

Acanthocytes can be seen in ______ and _______________

Answer 48

liver disease ; McLeod blood group phenotype.

Question 49

True or False: Haemophilia does not affect bleeding time

Answer 49

True [Haemophilia is a disorder of secondary haemostasis and does not affect platelets. Therefore bleeding time, a measure of primary haemostasis, is usually normal in haemophilia.]

Question 50

How does Dabigatran work?

Answer 50

Dabigatran is a direct oral anticoagulant (DOAC) that binds to thrombin, also known as clotting factor II. Thrombin normally acts on fibrinogen to produce the fibrin strands that compose a normal clot. By inhibit this process dabigatran impairs clot formation. Additionally in severe bleeding, dabigatran can be reversed with a medication called idarucizumab.