Haematological Malignancies

Question 1

What occurs in acute haematological malignancies?

Answer 1

Increased rate of apoptosis,
Decreased apoptosis
Differentiation block

Question 2

What occurs in chronic?

Answer 2

Build up of mature differentiated cells
Proliferation increased
NO differential block like in acute

Question 3

Why is acute leukaemia a heterogenous disease?

Answer 3

Because the acute phase can happen at any point of differentiation

Question 4

What % of blasts in the bone marrow is required to diagnose acute leukaemia?

Answer 4

20% (can be less for molecular mechanism in place)

Question 5

What cells are found in hodgekins lymphoma?

Answer 5

Reed-Sternberg cells

Question 6

What are risks for haematological malignancies

Answer 6

Radiation
Benzene
Viruses
Genetic disorders- fanconi anaemia , Down syndrome etc.

Question 7

What malignancy is associated with the Epstein- Barr virus?

Answer 7

Lymphoma

Question 8

What malignancy is associated with heliocobacter pylori infection?

Answer 8

Gastric lymphoma B-cell

Question 9

Explain how FACS works?

Answer 9

Anti surface antigen antibodies are labelled with fluorochromes and used to Bind to CD molecules- analysed on the single cell level by flow cytometry

Question 10

What mutation is found in 30% of AML?

Answer 10

FLIT3

Question 11

What is the most common change found in b and T cell malignancies?

Answer 11

Clonal Ig or TCR rearrangements

Question 12

What is the incidence of CML?

Answer 12

0.9% per 100,000

500/year in uk

Question 13

What is the most frequent age of CML?

Answer 13

40-60 years

Question 14

What are the 3 phases of CML?

Answer 14

Chronic
Accelerated
Blast crisis

Question 15

What are the symptoms of the chronic stage of CML and how long does it last?

Answer 15

Often asymptomatic- can last up to 5 years

Question 16

What are the symptoms of the accelerated stage of CML and how long does it last?

Answer 16

Anaemia and thrombocytopenia and increased blasts in BM

Last several months

Question 17

What is required to move from chronic to accelerated or blast crisis? (CML)

Answer 17

Additional chromosomal abnormalities

Question 18

What is the blast count during blast crisis (CML) ?

Answer 18

> 20%

Question 19

True or false CML can become AML or ALL?

Answer 19

True

Question 20

What is the BCR able translocation?

Answer 20

t(9;22)(q34;q11)

Question 21

What does BCR-ABL generate?

Answer 21

A highly active oncogene tyrosine kinase 210 kda

Question 22

How do you test BCR-ABL negative CML patients?

Answer 22

As if they have a myloproliferative disorder

Question 23

What other disorder is the Philadelphia gene found in?

Answer 23

ALL

Question 24

What does a blood film of a CML patient look like?

Answer 24

Neutrophilic leucocytes is with immature precursors you normally wouldn’t see and lots of basophils

Question 25

What will a PB blood film in CML often resemble?

Answer 25

A Bone marrow asparate

Question 26

What does bone marrow look like inCML?

Answer 26

Hypercellular see all stages of myeloid maturation and an increase in basophils and eosinophils

Question 27

What are the symptoms of CML?

Answer 27

Usually associated with hyper metabolism - splenomegaly or anaemia (normochromic normocytic)

Question 28

How much is leucocytosis?

Answer 28

>50 X 10^9/L

Question 29

If imatinib is not successful what is second generation inhibitor?

Answer 29

Disastinib

Question 30

What is the frequency of AML?

Answer 30

4.1% per 100,000

Question 31

What is the median age of AML?

Answer 31

70

Question 32

What is the most common form of adult leukaemia?

Answer 32

AML

Question 33

Is AML one disease?

Answer 33

No, many diseases as leukaemia occur at different stages

Question 34

How many types of AML based on FAB?

Answer 34

8 types based on morphology moos all abnormality

Question 35

How many classifications of AML based on WHO 2008

Answer 35

4

Question 36

What is class 1 of WHO 2008 and what is the prognosis ?

Answer 36

AML with recurrent genetic abnormalities - good prognosis

Question 37

Name the four genetic abnormalities associated with group 1 of AML?

Answer 37

Inv(16) T(8;21) T(16;6) T(15;17)

Question 38

What is group 2 (who)

Answer 38

AML with myelodysplasia related changes including patients with previous myledysplastic syndromes- POOR PROGNOSIS

Question 39

What is group 3 (who)

Answer 39

Therapy related AML t-AML and t-MDS

Question 40

What is group 4 (who)

Answer 40

All other AML

Question 41

Deletions of what chromosome have a v.poor prognosis in AML ?

Answer 41

Chromosome 5 and chromosome 7

Question 42

Is the FLT interval repeat in AML a good prognosis or bad?

Answer 42

Bad

Question 43

What are the clinical features of AML?

Answer 43

Related to leukaemic cells replacing cells in bone marrow - decrease in rbc, platelets and WBCs Anaemia (normochromic normocytic) Thrombocytopenia Infections

Question 44

What does bone marrow look like in AML?

Answer 44

Hypercellular with blasts- large unusual nucleus and open chromatin

Question 45

What can be seen in AML myeloblasts?

Answer 45

Auer rods

Question 46

What is involved in the induction treatment of AML?

Answer 46

Attempt to reduce blast numbers in BM to less than 5% | Use daunarubican, ara-c and etoposide

Question 47

What is the second stage of AML treatment?

Answer 47

Consolidation- attempt to eliminate the disease using daunarubican , ara-c and etopside

Question 48

What disorder is defined by the 15-17 translocation?

Answer 48

Acute promyloific leukaemia (APL)

Question 49

How do you treat APL ?

Answer 49

Using ATRA

Question 50

What is ATRA?

Answer 50

All trans retinoic acid - a non chemotherapy drug that induces differentiation of promyleocytes and once they differentiate they under go apoptosis