Malignancies

Question 1

How do we identify normal progenitors / stem cells?

Answer 1

Cell surface antigens through immunophenotyping

Question 2

What are the two main features of malignant haemopoeisis?

Answer 2

Increased number of abnormal dysfunctional cells

Loss of normal activity

Question 3

In acute myeloid leukemia what occurs?

Answer 3

There is neoplastic proliferation of abnormal progenitors (blast cells)
Block in maturation and differentiation further down

Question 4

In chronic myeloid leukemia, what occurs?

Answer 4

Proliferation of abnormal progenitors BUT there is still maturation and differentiation further down

Question 5

List some potential causes of hematological malignancies:

Answer 5

Genetic Hits
Environmental interaction
Driver mutations
Recurrent cytogenetic abnormalities

Question 6

Malignant haemopoeisis is poly / mono clonal?

Answer 6

Monoclonal

Question 7

What is the significance of driver mutations in haem malignancy/

Answer 7

Driver mutations can select clones and confer a growth advantage to the cells

Question 8

What type of leukaemia is histologically and clinically more aggressive ?

Answer 8

Acute

Question 9

How does an acute leukemia typically present?

Answer 9

Bone marrow failure symptoms

Question 10

List some features of histological aggression in leukaemia

Answer 10

Large cells
High nuclear - cytoplasmic ratio
Prominent nucleoli
Rapid proliferation

Question 11

How is acute leukaemia defined?

Answer 11

> 20% of blasts in either the bone marrow or peripheral blood

Question 12

`Which leukaemia is the most common childhood cancer?

Answer 12

Acute lymphoblastic leukaemia

Question 13

How would ALL present?

Answer 13

Bone marrow failure: anaemia, infections, bleeding
Bone pain
Lymphadenopathy 
Hepato - spleno - megaly
Orchidomegaly 
CNS involvement: meningism, CN palsies

Question 14

What age group does acute myeloid leukemia affect?

Answer 14

Elderly >60y/o

Question 15

What are some of the common infections seen in ALL?

Answer 15

Pneumocystis pneumonia
Candidiasis 
Measles
CMV
VZV

Question 16

What investigations are done for ALL?

Answer 16

FBC - WCC raised despite neutropaenia
Blood film - blasts
Coagulation screen
Bone Marrow Aspirate 
CT / MRI for mediastinal lymphadenopathy

Question 17

What is seen on a blood film that is characteristic of AML?

Answer 17

Auer Rods

Question 18

What is required to make a definitive diagnosis of either AML/ALL?

Answer 18

Immunophenotyping

this defines the lineage of the abnormal cells

Question 19

What is trephine?

Answer 19

A piece of bone used to assess the cellularity of the bone marrow when an aspirate sample is sub-optimal

Question 20

What is the prognosis for ALL?

Answer 20

Children - 70-90% curative

Adults - 40% curative

Question 21

What is the definition of hematological remission?

Answer 21

<5% blasts in a normal regenerating bone marrow with no evidence of leukaemia in the blood and a normal / recovering blood count

Question 22

How is ALL treated?

Answer 22

Supportive therapies
Chemotherapy - remission induction
Allogenic Marrow transplants
Can last 2 - 3 years

Question 23

How is AML treated?

Answer 23

Intensive cycles of chemo lasting 5-10 days, every few weeks
Bone marrow transplant - HLA matched donors for allogenic transplant in 1st remission

Question 24

How is chemotherapy given in hematological patients?

Answer 24

Via a Hickman line

Question 25

What are some consequences of bone marrow suppression?

Answer 25

Anaemia Neutropaenia - severe infections Thrombocytopaenia - severe bleeding

Question 26

What type of infections are common in neutropaenic patients?

Answer 26

Gram Negative bacteria

Question 27

How are patients undergoing treatment managed for bacterial infections?

Answer 27

Broad spectrum antibiotics the minute the develop a neutropaenic fever

Question 28

Patients with a neutropaenic fever who are not responding to antibiotics may have what?

Answer 28

A fungal infection

Question 29

What is the cure rates for AML?

Answer 29

<60 y/o 40-50% | >60 y/o 10%

Question 30

What AML sub type is associated with disseminated intravascular coagulopathy ?

Answer 30

Chromosome translocation of c15:c17

Question 31

When might you see ring sideroblasts in a blood film?

Answer 31

In myelodysplastic syndromes

Question 32

What cells are characteristic of Hodgkin's Lymphoma?

Answer 32

Reed-Sternberg cells

Question 33

What cells are most non Hodgkin's lymphoma's derived from?

Answer 33

B cells

Question 34

What is the most common form of Non-Hodgkin Lymphoma?

Answer 34

Diffuse large B cell lymphoma

Question 35

What is Burkitt's Lymphoma?

Answer 35

Childhood disease | Characterised by jaw lymphadenopathy

Question 36

What is MALT?

Answer 36

Mucosa - associated - lymphoid - tissue

Question 37

What investigations are done for Lymphoma?

Answer 37

FBC and film Marrow and lymph node biopsy Staging - Ann Arbor system

Question 38

What type on Non-Hodgkin's is more aggressive?

Answer 38

High grade

Question 39

What type on Non-Hodgkin's is more likely to be curable?

Answer 39

High grade