Leukaemia

Question 1

What is leukaemia?

Answer 1

cancer of stem cells in bone marrow causing unregulated production of a specific type of blood cell (often white blood cells)

Question 2

What is pancytopenia?

Answer 2

1) anaemia
2) leukopenia
3) thrombocytopenia

Question 3

What causes pancytopenia in leukaemia?

Answer 3

the excessive production of a single type of cell can suppress the other lines, causing underproduction of other cell types

Question 4

What is acute lymphoblastic leukaemia?

Answer 4

cancer affecting one of the lymphocyte precursor cells causing an acute proliferation of a single type of lymphocyte (usually B cells)

Question 5

In what patient age group is acute lymphoblastic leukaemia most commonly seen in?

Answer 5

children under 5

Question 6

What chromosomal disease is acute lymphoblastic leukaemia associated with?

Answer 6

Down’s syndrome

Question 7

What is chronic lymphoblastic leukaemia?

Answer 7

slow proliferation of a single type of well-differentiated lymphocyte, usually B cells

Question 8

In what patient age group is chronic lymphoblastic leukaemia most commonly seen in?

Answer 8

adults over 60

Question 9

True or false: chronic lymphoblastic leukaemia is often asymptomatic

Answer 9

True

Question 10

What is a classic haemolytic feature of chronic lymphoblastic leukaemia?

Answer 10

warm autoimmune haemolytic anaemia

Question 11

What is warm autoimmune haemolytic anaemia?

Answer 11

where autoantibodies react with red blood cells at temperatures over 37 degrees C

Question 12

What is the name given to the rare transformation of chronic lymphoblastic leukaemia into a high grade B cell lymphoma?

Answer 12

Richter’s transformation

Question 13

What blood film sign is associated with chronic lymphoblastic leukaemia?

Answer 13

smear/ smudge cells

Question 14

What are smear/smudge cells?

Answer 14

ruptured WBCs

Question 15

In what age group is acute myeloid leukaemia seen in?

Answer 15

middle age onwards

Question 16

Give two myeloproliferative diseases that may progress to acute myeloid leukaemia?

Answer 16

1) polycythaemia ruby vera
2) myelofibrosis

Question 17

Give 2 blood film findings assocaited with acute myeloid leukaemia:

Answer 17

1) high proportion of blast cells
2) auer rods in blast cell cytoplasms

Question 18

What are auer rods?

Answer 18

crystalline cytoplasmic inclusion bodies

Question 19

What chromosomal disease is associated with chronic myeloid leukaemia?

Answer 19

Philadelphia chromosome

Question 20

What are the 3 phases of chronic myeloid leukaemia?

Answer 20

1) chronic phase
2) accelerated phase
3) blast phase

Question 21

Describe the chronic phase of chronic myeloid leukaemia:

Answer 21

often asymptomatic with raised WCC (often incidental finding) which can last several years

Question 22

Describe the accelerated phase of chronic myeloid leukaemia:

Answer 22

where abnormal blast cells take of 10-20% of bone marrow and blood content, allowing symptoms such as anaemia, thrombocytopenia and immunodeficiency to develop

Question 23

Describe the blast phase of chronic myeloid leukaemia:

Answer 23

where an even higher proportion of blast cells (over 20%) is seen in the blood, causing severe and even fatal symptoms

Question 24

Give 8 clinical presentations associated with leukaemia:

Answer 24

1) fatigue
2) fever
3) pallor
4) petechiae/ bruising
5) abnormal bleeding
6) lymphadenopathy
7) hepatosplenomegaly
8) failure to thrive

Question 25

Give the size threshold for petechiae:

Answer 25

less than 3mm

Question 26

Give the size range for purpura:

Answer 26

3-10mm

Question 27

Give the size threshold for ecchymosis:

Answer 27

>1cm

Question 28

What initial investigation should be used within 48hrs of suspected leukaemia?

Answer 28

full blood count

Question 29

Give 7 investigations for leukaemia:

Answer 29

1) FBC 2) blood film 3) lactate dehydrogenase 4) bone marrow biopsy 5) CT and PET scans 6) lymph node biopsy 7) genetic testing and immunotyping

Question 30

What is the gold standard diagnostic test for leukaemia?

Answer 30

bone marrow biopsy

Question 31

Where are bone marrow biopsies usually taken from?

Answer 31

iliac crest

Question 32

What are the two types of bone marrow biopsies?

Answer 32

1) aspiration (liquid sample of cells) 2) trephine (solid core sample of bone marrow)

Question 33

Which provides a better assessment of cells, aspiration or trephine bone marrow biopsies?

Answer 33

trephine

Question 34

Give 3 general management strategies used for leukaemia:

Answer 34

1) chemotherapy 2) targeted therapy 3) bone marrow transplants

Question 35

Give 2 targeted therapies for leukaemia:

Answer 35

1) ibrutinib (tyrosine kinase inhibitor) 2) rituximab (monoclonal antibody against B cells)

Question 36

What complication is associated with chemotherapy rapidly destroying tumour cells?

Answer 36

tumour lysis syndrome

Question 37

Give 4 U+E imbalances seen in tumour lysis syndrome:

Answer 37

1) increased uric acid 2) hyperkalaemia 3) hyperphosphatemia 4) hypocalcaemia

Question 38

Name 2 drugs that can be used to supress uric acid levels in tumour lysis syndrome?

Answer 38

1) allopurinol 2) rasburicase

Question 39

What complication is associated with raised uric acid in tumour lysis syndrome?

Answer 39

AKI (crystal deposits in kidneys)

Question 40

Describe how the Philadelphia chromosome can cause chronic myeloid leukaemia:

Answer 40

the reciprocal translocation between chromosomes 9 and 22 results in the abnormal BCR-ABL1 gene sequence which codes for an abnormal tyrosine kinase enzyme which drives the proliferation of abnormal cells