Haematology and Oncology

Question 1

ITP + AIHA =

Answer 1

Evan’s syndrome

Question 2

Which immunoglobulin drives ITP?

Answer 2

IgG

Question 3

3 features of craniopharyngioma

Answer 3

headache
visual disturbance
hormone imbalance

Question 4

G6PD blood results

Answer 4

Low - Hb, haptoglobin, spectrophotometric assay <10%

High - reticulocytes, bili, lactate

Present - Heinz cells, bite cells, polychromasia

Question 5

Factor 8 levels in severe haemophilia A

Answer 5

<1%

Question 6

most important treatment in sickle cell

Answer 6

high flow O2 (hypoxia drives sickling)

Question 7

What do Howell Jolly bodies show?

Answer 7

splenic dysfunction e.g. sickle cell

Question 8

onset of high grade astrocytoma

Answer 8

acute

Question 9

sickle cell diagnosis

Answer 9

electrophoresis

Question 10

how does sickle cell cause macrocytosis?

Answer 10

chronic haemolysis = folate deficiency

Question 11

alpha thalassaemia deletions

Answer 11

1 or 2 = trait
3 = HbH disease
4 = foetal hydrops and fatal Bart’s

Question 12

beta thalassaemia mutations

Answer 12

1 = minor
2 = major

Question 13

most common solid tumour in children and location

Answer 13

medulloblastoma (cerebellar)

Question 14

how to confirm aplastic crisis

Answer 14

reticulocytes inappropriately low

Question 15

who gets Hodgkin’s lymphoma?

Answer 15

teenagers

Question 16

G6PD inheritance

Answer 16

X linked recessive

Question 17

hereditary spherocytosis inheritance

Answer 17

AD

Question 18

sickle cell and thalassaemia skull deformities

Answer 18

sickle cell = overbite
thalassaemia = bossing

Question 19

haemophilia coag results

Answer 19

raised aPTT
normal bleeding time

Question 20

supraclavicular LN enlargement?

Answer 20

do CXR for ?mediastinal involement ?cancer

Question 21

tuberous sclerosis presentation

Answer 21

epilepsy
macules
shagreen patch
fibroma/lipoma/harmatomas
dental pits
renal cysts

Question 22

regular Ix for tuberous sclerosis

Answer 22

MRI head and abdo

Question 23

haptoglobin in haemolysis

Answer 23

low

Question 24

ependyoma location

Answer 24

extends into the upper cervical cord through the foramen magnum

Question 25

test for neuroblastoma

Answer 25

urine catecholamines

Question 26

bloods in hereditary spherocytosis

Answer 26

anaemia unconjugated jaundice

Question 27

major haemorrhage management

Answer 27

20ml/kg RBC if still shocked 10ml/kg cryoprecipitate

Question 28

most common childhood leukaemia

Answer 28

ALL

Question 29

first treatment in ALL

Answer 29

correct anaemia and platelets before chemo

Question 30

timing of CNS infiltration in ALL

Answer 30

relapse rather than diagnosis

Question 31

Fanconi's anaemia inheritance

Answer 31

autosomal recessive

Question 32

drug to avoid in haemaphilia

Answer 32

NSAIDs

Question 33

which haemophilia may be managed by desmopressin if mild?

Answer 33

A

Question 34

iron content in milk

Answer 34

Cows milk is higher than breast but more poorly absorbed

Question 35

which iron preparations for children and why

Answer 35

edentate or polysaccharide (others stain teeth)

Question 36

test before steroids for ITP

Answer 36

bone marrow biopsy to exclude ALL

Question 37

when to give a platelet transfusion

Answer 37

life threatening bleed (only raises plt for a few hours)

Question 38

G6PD activity in crisis

Answer 38

high

Question 39

when to do splenectomy in hereditary spherocytosis

Answer 39

>7yo due to sepsis risk

Question 40

2 conditions associated with spherocytes and how to differentiate

Answer 40

hereditary spherocytosis AIHA do DAT

Question 41

LT drug for sickle cell

Answer 41

hydroxycarbamide

Question 42

post stroke management of sickle cell

Answer 42

sibling bone marrow transplant

Question 43

Rx for mild von willebrand

Answer 43

desmopressin

Question 44

Rx for severe von willebrand

Answer 44

plasma derived factor 8 concentrate

Question 45

Which AIHA is more common

Answer 45

warm (IgG)

Question 46

how does warm AIHA cause haemolysis

Answer 46

extravascular

Question 47

AIHA presentation

Answer 47

dark urine anaemia splenomegaly DVT

Question 48

where does foetal haematopoesis occur

Answer 48

liver

Question 49

Fetomaternal alloimmune thrombocytopaenia Pathophysiology Presentation Treatment

Answer 49

Baby inherits dad's platelets with antigens on. Sensitisation event occurs and mum develops attacking Ab that can cross the placenta and attack babies platelets. (Mum attacks baby). Low Plts = purpura and intracranial haemorrhage in otherwise well baby and mum Give antigen negative platelets (irradiated and plasma removed)

Question 50

DAT in hereditary spherocytosis

Answer 50

negative

Question 51

when to do a bone marrow biopsy in ITP

Answer 51

bone pain or limp abnormal lymphadenopathy hepatosplenomegaly persistent fever macrocytosis FH or PMH of bleeding other abnormalities on FBC

Question 52

DIC bloods

Answer 52

low fibrinogen high PT

Question 53

how to differentiate AML and ALL

Answer 53

myeloperoxidase is positive in AML