Clinical Management - Leukaemia

Question 1

Name four Leukmias

Answer 1

1. Acute Myeloid Leukaemia
2. Chronic Myeloid Leukaemia
3. Acute Lymphoblastic Leukaemia
4. Chronic Lymphocytic leukaemia

Question 2

What is leukaemia

Answer 2

Malignant proliferation of haemopoietic cells

Question 3

Blood test for leukaemia

Answer 3

1. Haemoglobin ia low
2. WBC count raised
3. Low platelet count

Question 4

What would a blood film show for leukaemia

Answer 4

1. Blast cells seen

Question 5

What about a bone marrow biopsy indicate

Answer 5

1. Reduced erythropoiesis
2. Reduced Megakaryocytic
3. Blast cell replacement

Question 6

What would the CXR show

Answer 6

Mediastinal widening - shows T-lymphoblastic leukaemia

Question 7

What examination is specific to Acute Lymphocytic Leukaemia

Answer 7

Cerebrospinal Fluid examination - risk of CNS involvement is high

Question 8

What is immunophenotyping

Answer 8

Classifying tumour cells by antigen expression profile

Question 9

Genetic diagnosis for leukaemia

Answer 9

1. Cytogenetics:
   Karyotyping
   FISH
2. Molecular Genetics:
   PCR

Question 10

How does the incidence of Acute Myeloid Leukaemia be effected with age

Answer 10

Increases

Question 11

What risks increase the chances of Acute Myeloid Leukaemia

Answer 11

1. Previous haematological disorders
2. Prior Chemotherapy
3. Exposure to ionising radiation

Question 12

Supportive care for Acute Myeloid Leukaemia

Answer 12

1. HML
2. Blood product support
3. Prompt treatment of infections
4. Recognition of atypical/unusual infections

Chemotherapy/Transplantation

Question 13

What is the approximate cure rates for AML

Answer 13

60% children
50% younger adults
Less than 20% older adults

Question 14

What causes Acute Promyelocytic Leukaemia

Answer 14

PML/PARA gene blocks differentiation of promyelocyte to mature granulocyte

Question 15

Consequence of Acute Promyelocytic Leukaemia

Answer 15

Haematological Emergency - DIC

Question 16

How is Acute Promyelocytic Leukaemia treated

Answer 16

targeted ATRA

Question 17

What age does Chronic Myeloid Leukaemia effect

Answer 17

40-60 yrs age

Question 18

Sign of Chronic Myeloid Leukaemia

Answer 18

Splenomegaly

Pallor

Question 19

What would show in a blood test for Chronic Myeloid Leukaemia

Answer 19

High WBC

Question 20

What is seen on the blood film on CML

Answer 20

Left shift + Basophilia

Question 21

Key diagnostic features of CML

Answer 21

1. Philadelphia Chromosome
2. t(9;22)
3. Resulting in 210-kDa fusion protein (activated tyrosine Kinase)

Question 22

How is t(9;22) diagnosed

Answer 22

A signal pattern found when doing BCR/ABL dual colour dual-fusion probe

(Normal should have four dots on the circumference, CML should have four dots circular and central)

Question 23

What is philedelphia chromosome

Answer 23

Where the q-arm of chromosome 22 has been shortened by translocation with 9 t(22;9)!

Question 24

How is BCR-ABL signalling activated

Answer 24

ATP phosphorylates tyrosine which is bound to membrane-bound substrate on the same cell

Question 25

How is Chronic Myeloid Leukaemia treated

Answer 25

Tyrosine Kinase inhibitors All end in -tinib Nilotinib, Imatinib

Question 26

Survival rate of CML

Answer 26

More than 90% survive first 5 years

Question 27

How can CML be worsened

Answer 27

Accelerated phase due to TKI binding site mutations

Question 28

Where is Acute lymphoblastic Leukaemia common (ALL)

Answer 28

Common in paediatric malignancy (not in adults)

Question 29

Presentation of ALL

Answer 29

1. Bone Marrow Failure | 2. Organ Infiltration (CNS)

Question 30

How is ALL diagnosed

Answer 30

Same as AML

Question 31

Cytogenetics of ALL

Answer 31

Philadelphia chromosome (poor prognosis if present)

Question 32

Treatment phases for ALL

Answer 32

1. Induction 2. Consolidation 3. Delayed intensification 4. Maintenance CNS directed therapy Stem Cell transplant

Question 33

Can most children be cured of ALL

Answer 33

Yes

Question 34

Cure rate in adults for ALL

Answer 34

50%

Question 35

What is the biggest influential factor for ALL

Answer 35

CYtogenetics

Question 36

What is the most common type of leukaemia

Answer 36

Chronic Lymphocytic Leukaemia

Question 37

What is CLL

Answer 37

Gradual accumulation of B lymphocyte

Question 38

Where do B lymphocytes accumulate

Answer 38

Blood Spleen Lymph

Question 39

When is CLL usually diagnosed

Answer 39

FBC

Question 40

What age group does CLL commonly effect

Answer 40

Elderly Sometimes <55 (20%)

Question 41

Signs of CLL

Answer 41

1. Progressive Lymphadenopathy/Hepatosplenomegaly 2. Haemolysis (anaemia) 3. Marrow failure as marrow replacement occurs 4. Hypogammaglobulinaemia + infection

Question 42

What is stage A CLL

Answer 42

Lymphocytosis

Question 43

How long does stage A CLL last for

Answer 43

10-15 yrs

Question 44

What is stage B CLL

Answer 44

Nodal spread

Question 45

How long does Stage B CLL last

Answer 45

5-7 years

Question 46

What is stage C CLL

Answer 46

Anaemia + Thrombocytopaenia

Question 47

How long does stage C CLL last for

Answer 47

2-3 yrs

Question 48

CLL treatment

Answer 48

``` Chemotherapy Monoclonal antibodies (rituximab) Target therapy (ibrutinib)Marrow tranplsant ```

Question 49

What is Autologous transplantation

Answer 49

Transplant of bone marrow from own host

Question 50

What is Allogenic transplantation

Answer 50

From someone else

Question 51

Danger of allogenic transplantation

Answer 51

Toxic 15-30% mortality Stem cells recipient cells as well as tumour

Question 52

When is Allogenic transplantation done

Answer 52

Acute and Chronic leukaemias

Question 53

Benefits of autologous transplantation of stem cells

Answer 53

Allows escalation of chemo Low mortality