malignant haematology

Question 1

what is malignant haemopoiesis characterised by

Answer 1

increased numbers of abnormal and dysfunctional cells

loss of normal activity

Question 2

what causes acute leukaemia

Answer 2

proliferation of abnormal progenitors with a block in differentiation/maturation

Question 3

what causes chronic myeloproliferative disorders

Answer 3

proliferation of abnormal progenitors, but no differentiation/maturation block

Question 4

what is a driver mutation

Answer 4

a mutations that confers a growth advantage on the cells

leads to cancer

Question 5

what is a passenger mutation

Answer 5

a mutation that does not confer growth advantage

doesn’t cause cancer

Question 6

what are clones

Answer 6

population of cells derived from a single parent cell

Question 7

is malignant haemopoiesis polyclonal or monoclonal

Answer 7

malignant haemopoiesis is monoclonal as all cells derived from a single parent cell with malignant mutation

Question 8

how can haematological malignancies be classified

Answer 8

lineage
developmental stage within lineage
anatomical site involved

Question 9

how can haematological malignancy be classified by lineage

Answer 9

myeloid

lymphoid

Question 10

how can haematological malignancy be classified based on developmental stage within lineage

Answer 10

• blastic (primitive precursor cell)

- cytic (mature precursor cell)

Question 11

how can haematological malignancy be classified based on anatomical site involved

Answer 11

leukaemia (blood)

lymphoma (lymph nodes)

Question 12

what is myeloma

Answer 12

plasma cell malignancy in the bone marrow

Question 13

how do acute leukaemia present

Answer 13

histologically and clinically more aggressive than chronic leukaemia

Question 14

signs of histological aggression in acute leukaemia

Answer 14

large cells with high N:C ratio
prominent nucleoli
open chromatin
rapid proliferation

Question 15

signs of clinical aggression in acute leukaemia

Answer 15

rapid progression

short history of symptoms

Question 16

how is acute leukaemia defined

Answer 16

excess of blasts (>20%) in either peripheral blood or bone marrow

Question 17

what is the most common childhood cancer

Answer 17

acute lymphoblastic leukaemia

Question 18

how does ALL present

Answer 18

marrow failure: anaemia, infections, bleeding

obstruction of circulation

involvement of areas outside the marrow and blood eg CNS, testis

bone pain due to rapid expansion of the marrow cavity

Question 19

who gets acute myeloid leukaemia

Answer 19

elderly people

Question 20

how is leukaemia investigated

Answer 20

blood count 
blood film 
coagulation screen 
bone marrow aspirate 
immunophenotyping 
genetics 
trephine

Question 21

treatment for ALL

Answer 21

can last up to 2-3 years
different phases of varying intensity
targeted treatments in certain subsets

Question 22

treatment for AML

Answer 22

intensive

2-4 cycles of chemotherapy (5-10 days followed by 2-4 weeks recovery)

Question 23

how is chemo administered

Answer 23

via Hickman line

Question 24

what are complications of chemo

Answer 24

nausea and vomiting 
hair loss
renal and liver dysfunction 
tumour lysis syndrome 
infection 
loss of fertility 
cardiomyopathy

Question 25

how should infection be managed in a patient on chemo

Answer 25

empirical treatment with broad spectrum antibiotics, particularly covering gram -ve, ASAP