Leukaemia

Question 1

State the five key aspects of normal haemopoiesis

Answer 1

Self-renewal 
Proliferation 
Differentiation 
Maturation 
Apoptosis

Question 2

How does malignancy arise?

Answer 2

Increased proliferation
Lack of differentiation
Lack of maturation
Lack of apoptosis

Question 3

How does acute leukaemia arise?

Answer 3

Proliferation of abnormal progenitors with block of differentiation/maturation

Question 4

How does chronic myeloid leukaemia arise?

Answer 4

Proliferation of myeloid cells but no differentiation/maturation block

Question 5

What causes leukaemia?

Answer 5

Multifactorial

Acquired somatic mutations in regulatory genes (driver mutations)

Question 6

What do driver mutations do?

Answer 6

Select clones and confer a growth advantage

Question 7

What is a clone?

Answer 7

Population of cells derived from a single parent cell

Question 8

Is malignancy polyclonal or monoclonal?

Answer 8

Monoclonal

Question 9

Name four factors that help determine the subtype of malignancy

Answer 9

Lineage
Developmental stage within lineage
Anatomical site
Histology/presentation

Question 10

What are the two key lineages?

Answer 10

Myeloid

Lymphoid

Question 11

What are the two main anatomical site of haematological malignancy?

Answer 11

Blood - leukaemia

Lymph nodes - lymphoma

Question 12

Describe the characteristics of a high grade tumour

Answer 12

Large cells 
High nucleus to cytoplasm ratio 
Prominent nucleoli 
Rapid proliferation 
Rapid progression of symptoms

Question 13

In acute leukaemia what type of cells are in excess?

Answer 13

Blasts

Question 14

Name two types of acute leukaemia

Answer 14

Acute myeloid

Acute lymphoblastic

Question 15

Describe ALL

Answer 15

Disease of primitive lymphoid cells - lymphoblasts. Most common childhood cancer

Question 16

How will ALL present?

Answer 16

Marrow failure - anaemia, infection, bleeding
Leukaemia effects
Bone pain

Question 17

What is meant by leukaemic effects?

Answer 17

High count with obstruction of circulation and involvement of areas outwit the marrow/blood

Question 18

Name two common extra-medullary sites

Answer 18

CNS, testes

Question 19

Describe AML

Answer 19

More common in elderly >60 years old

De novo or secondary

Question 20

How does AML present?

Answer 20

Similar to ALL with dominant marrow failure

Question 21

Name two subgroups of AML

Answer 21

DIC in acute promyelocytic leukaemia

Gum infiltration

Question 22

What investigations should be done in acute leukaemia?

Answer 22

Blood count and film 
Coagulation screen 
D dimer and fibrinogen - DIC 
Bone marrow aspirate
Immunophenotyping 
Cytogenic analysis 
Trephine biopsy

Question 23

What will a blood film of leukaemia show?

Answer 23

Reduced Hb
Neutrophils 
Platelets 
Increased WCC
Blasts 
Aner Rods

Question 24

What are Aner rods?

Answer 24

Cytoplasmic inclusions seen in AML

Question 25

How does immunophenotyping work?

Answer 25

Lineage specific proteins on cell surface | Definitive diagnosis - important for treatment

Question 26

How is leukaemia treated?

Answer 26

Multi-agent chemotherapy

Question 27

How long may ALL last?

Answer 27

2-3 years

Question 28

How is AML treated?

Answer 28

2-4 cycles of chemo followed by 2-4 weeks recovery. Intensive therapy requires prolonged hospitalisation

Question 29

Name the type of long term central venous access used

Answer 29

Hickman Line

Question 30

Where is the tip of Hickman Line?

Answer 30

SVC - right atrium junction

Question 31

What are the complications of leukaemia treatment?

Answer 31

Anaemia Neutropenia - infections Thrombocytopenia

Question 32

Which infections are common in leukaemia patients?

Answer 32

Gram negative bacteria from large bowel can cause fulminant life threatening sepsis Fungal infections can also occur

Question 33

After culture what must be done in leukaemia patients with infection?

Answer 33

Broad spec antibiotics

Question 34

How will thrombocytopenia present and what is the treatment?

Answer 34

Bleeding Petechiae Requires platelet transfusion

Question 35

Name the side effects of chemotherapy

Answer 35

``` Nausea/vomiting Hair loss Liver/renal dysfunction Tumour lysis syndrome Infection (bacterial, fungal, protozoal - PJC) Late effects - infertility/cardiotoxic ```