haem

Question 1

chronic myeloid leukaemia is caused by what mutation?

Answer 1

(T9;22) philadelphia

BCR-ABL1 re-arrangement

Question 2

what types of leukaemia’s have blocked differentiation/maturation

Answer 2

Acute leukaemia’s

Question 3

define acute leukaemia

Answer 3

rapidly progressive clonal malignancy of the marrow/blood with maturation defect(S)

Question 4

outline ALL

Answer 4

• Malignant disease of primitive lymphoid cells
• most common childhood cancer

Question 5

ALL can involve areas outside bone marrow true/false

Answer 5

true

Question 6

some patients with AML can present with what?

Answer 6

Coagulation defect (DIC)

Question 7

what condition can Auer rods be seen

Answer 7

AML only

Question 8

what investigation is needed for a definitive diagnosis of AML/ALL

Answer 8

immunophenotyping

Question 9

how long can ALL treatment last

Answer 9

2-3 years (varying intensity)

Question 10

outline AML treatment

Answer 10

intensive chemo (3-4 cycles)

Question 11

what type of bacteria can cause fulminant life-threatening sespsis in neutropenic patients?

Answer 11

Gram negative bacteria

Question 12

if there is a prolonged neutropenia and persisting fever unresponsive to anti- bacterial agents give ________

Answer 12

Anti- fungal

Question 13

define CLL

Answer 13

A clonal (malignant) lymphoproliferative disorder of the mature B lymphocyte compartment

Question 14

‘smudge/Smear Cell’

Answer 14

CLL

Question 15

what is the difference between leukaemia and lymphoma

Answer 15

leukaemia - bone marrow and blood
lymphoma - lymph nodes

Question 16

differential diagnosis for lymphadenopathy

Answer 16

‘reactive’
bacteria infection (regional)
viral infection (generalised)
metastatic malignancy
lymphoma

Question 17

what IGM are associated with warm and cold AHA

Answer 17

COLD - IgM
WARM - IgG

Question 18

what is the first line prophylactic antibiotic following splenectomy

Answer 18

Phenoxymethylpenicillin

Question 19

smudge cells

Answer 19

CLL

Question 20

reed-steernberg cells

Answer 20

hodgkins lymphoma

Question 21

diagnosis of sickle cell anaemia involves

Answer 21

haemoglobin electrophoresis

Question 22

teardrop poikilocytes

Answer 22

myelofibrosis

Question 23

Which factors do protein C and S inhibit in the clotting cascade?

Answer 23

1985
10a
9
8
5

Question 24

Normocytic anaemia with normal ferritin, in the presence of an inflammatory or malignant condition + treatment

Answer 24

anaemia of chronic disease
supportive treatment+ treat underlying cause

Question 25

prolonged PT, while aPTT and TT are normal

Answer 25

factor VII deficiency

Question 26

the first type of white blood cell to reach areas of acute inflammation

Answer 26

neutrophils

Question 27

CLL can transform into non-hodgins lymphoma what is this called (B-Cells)

Answer 27

richters transformation

Question 28

first line treatment for immune thrombocytopenic purpura

Answer 28

cortico steroids - increase platelet production and decrease platelet destruction

Question 29

what is a common cause of erythema infectiosum 'fifth disease'

Answer 29

parvovirus B19

Question 30

dry tap on bone marrow aspiration

Answer 30

myelofibrosis

Question 31

iron results due to anaemia of chronic disease

Answer 31

Low serum iron, high ferritin and low transferrin saturation

Question 32

schistocytes

Answer 32

intravascular haemolysis

Question 33

what virus is associated with burkitts lymphoma

Answer 33

EBV

Question 33

Raised red cell mass, low serum erythropoetin, JAK2 mutation present

Answer 33

PCV

Question 33

howell-jolly bodies

Answer 33

hyposplenism

Question 34

typical history for g6pd deficiency

Answer 34

* Neonatal jaundice * Infection/drugs precipitate haemolysis * Gallstones

Question 34

give the typical gender + ethnicity for g6pd deficiency

Answer 34

male (x-linked recessive) african + mediterranean descent

Question 34

gender and ethnicity of hereditery spherocytosis

Answer 34

Male + female (autosomal dominant) northern European descent

Question 34

typical history for hereditary spherocytosis

Answer 34

* Neonatal jaundice *aplastic crisis precipitated by parvovirus infection * Gallstones * Splenomegaly is common * MCHC elevated

Question 35

blood film and diagnostic test for g6pd deficiency

Answer 35

heinz bodies, bite and blister cells Measure enzyme activity of G6PD

Question 36

blood film and diagnostic test for hereditary spherocytosis

Answer 36

spherocytes EMA binding

Question 37

give long term treatment for hereditery spherocytosis

Answer 37

folate replacement splenectomy

Question 38

pathophysiology of g6pd deficiency

Answer 38

↓ G6PD → ↓ reduced NADPH → ↓ reduced glutathione → increased red cell susceptibility to oxidative stress

Question 39

outline extrinsic pathway

Answer 39

tissue factor/factor VII - monitered by PTT

Question 40

outline intinsic pathway

Answer 40

XII/XI => VIII/IX – monitored by APTT

Question 41

sickle cell inheritance pattern

Answer 41

autosomal recessive

Question 42

starry sky appearance

Answer 42

burkitts lymphoma (non-hodgkins)

Question 43

tumour lysis investigationresults

Answer 43

ECG, U&E (potassium and phosphate, uric acid are typically raised), calcium (low),

Question 44

Normal platelets; prolonged bleeding time; prolonged APTT, normal PT and TT

Answer 44

von willebrand

Question 45

first-line clinical treatment for tumour lysis syndrome

Answer 45

Rasburicase - recombinant urate oxidase, meaning it metabolises uric acid to allantoin

Question 46

which pathogen can cause a sickle-cell crises

Answer 46

strep. pneumoniae

Question 47

burkitts lymphoma mutation

Answer 47

Translocation between chromosomes 8 and 14 is associated with Burkitt's lymphoma

Question 48

CLL treatment and MOA

Answer 48

rimatunib - tyrosine kinase inhibitor

Question 49

mylodyplastic syndrome can turn into

Answer 49

aml

Question 50

what can polycythaemia vera turn into

Answer 50

AML or myelofibrosis

Question 51

prosthetic heart valves may result in

Answer 51

haemolytic anaemia

Question 52

Isolated rise in GGT in the context of a macrocytic anaemia suggests

Answer 52

alcohol excess

Question 53

thalasseamia diagnosis

Answer 53

HPLC

Question 54

burr cells

Answer 54

uraemia

Question 55

Schistocytes or helmet cells

Answer 55

microangiopathic haemolytic anaemia

Question 56

Rouleaux formation

Answer 56

multiple myeloma

Question 57

Drugs causing folate deficiency

Answer 57

phenytoin, methotrexate, trimethoprim

Question 58

treatment for warm haemolytic anaemia

Answer 58

prednisolone, splenectomy

Question 59

Widespread petechiae in child 2-week post infection

Answer 59

immune thrombocytopaenia

Question 60

RAT FN (renal failure, anaemia, thrombocytopaenia, fever, neuro dysfunction)

Answer 60

TTP

Question 61

treatment for TTP

Answer 61

plasma and prednisolone

Question 62

increased basophils

Answer 62

CML

Question 63

Most common Hodgkin’s lymphoma

Answer 63

nodular sclerosing