Leukamia-Part 2

Question 1

Describe the nature of the leukaemic cells in CML.

Answer 1

These are mature lymphocytes – their cell kinetics and function are not as seriously affected as they are in AML but the cells do become independent of external signals, there are alterations in the interaction with stroma and there is reduced apoptosis so that cells survive longer and the leukaemic clone expands progressively

Question 2

How is the production of end cells affected in AML and CML?

Answer 2

AML – decrease in the production of end cells CML – increase in the production of end cells

Question 3

What are the metabolic effects of leukaemic cell proliferation?

Answer 3

Hyperuricaemia ,Renal failure ,Weight loss, Low grade fever ,Sweating

Question 4

Which type of leukaemia increases the risk of intraventricular haemorrhage and why?

Answer 4

Acute promyelocytic leukaemia (APML) – this is associated with DIC so the platelet count and fibrinogen are low leading to increased risk of fatal haemorrhage

Question 5

How can leukaemia cause proliferation of the gums?

Answer 5

Infiltration of leukaemic cells and monocytes can lead to inflammation of the gums There will be small haemorrhages due to thrombocytopenia

Question 6

What does epidemiology suggest that B lineage acute lymphoblastic leukaemia may result from?

Answer 6

It may result from delayed exposure to a common pathogen

Question 7

What are some factors that relate to risk of leukaemia?

Answer 7

Family size, new towns, socio-economic class, early social interactions

Question 8

What can leukaemias in infants and young children result from?

Answer 8

Irradiation in utero In utero exposure to certain chemicals

Question 9

What investigations are performed in acute lymphoblastic leukaemia?

Answer 9

Blood count and film Check of liver function and renal function and uric acid Bone marrow aspirate Cytogenetic/molecular analysis Chest X-ray

Question 10

What are the uses of cytogenetic and molecular genetic analysis in ALL?

Answer 10

It is useful for managing the individual patient because it gives us information about prognosis It permits the discovery of leukaemogenic mechanisms

Question 11

What are the implications of hyperdiploidy in in the cytogenetic analysis of ALL?

Answer 11

Good prognosis

Question 12

What features of the cytogenetic analysis are associated with a poor prognosis?

Answer 12

Chromosomal translocations resulting in the formation of a bad fusion gene

Question 13

What translocation causes ALL? State the fusion gene.

Answer 13

Translocation between chromosome 12 and chromosome 21 Fusion gene: ETV6-RUNX1 on chromosome 12

Question 14

What technique is used to detect the fusion genes in ALL?

Answer 14

Fluorescence in situ hybridisation (FISH)

Question 15

What are the treatment options for ALL?

Answer 15

Supportive: red cells, platelets, antibiotics Systemic chemotherapy Intrathecal chemotherapy

Question 16

Why do people get leukaemia?

Answer 16

•Since some mutations that contribute to leukaemogenesis appear to be random events rather than caused by an exogenous influence, they may result from the nature of the human genome

Question 17

•Acute lymphoblastic leukaemia is largely a disease of childrenT/F

Answer 17

T

Question 18

Describe the different mechanism through which leukamia can causes it’s clnical presentations

Answer 18

Accumulation of abnormal cells

Metabolic effects of leukaemic cell proliferation(takes a lot of effort to support this proliferation

Crowding out of normal cells

•Loss of normal immune function as a result of loss of normal T cell and B cell function—a feature of chronic lymphoid leukaemia

Question 19

What are the clinical features of acute lymphoblastic leukaemia that result from the accumulation of abnormal cells

Answer 19

Bone pain Hepatomegaly Splenomegaly Lymphadenopathy Thymic enlargement Testicular enlargement These all result from the accumulation of abnormal cells

Question 20

What are some clinical features of acute lymphoblastic leukaemia that result from the crowding out of normal cells

Answer 20

Caused by anaemia – fatigue, lethargy, pallor, breathlessness Caused by neutropenia – fever and other features of infection Caused by thrombocytopenia – bruising, petechiae, bleeding