1.2 Acute Leukaemia

Question 1

define leukaemia:

Answer 1

A spectrum of diseases resulting from a malignant neoplastic proliferations of haemopoietic or lymphoid cells. It results from an acquired mutation in a single stem cell, the progeny of which form a clone of leukaemic cells

Question 2

define acute leukaemia

Answer 2

Malignant transformation in the HSC or early progenitor which causes accumulation of blast cells (early HSC) in the bone marrow.

Question 3

What is the dominant feature of acute leukaemia?

Answer 3

Bone marrow failure

Question 4

What is cytopenia?

Answer 4

reduction in the number of blood cells

Question 5

Define chronic leukaemia

Answer 5

A slowly progressive proliferation of haemopoietic or lymphoid cells. The leukaemic clone often retains some functional capabilities and the clinical disease is indolent. Over time, the disease process becomes more aggressive

Question 6

Who normally gets AML?

Answer 6

adults >60 (most common leukaemia in adults)

M:f 5:3

Question 7

Who normally gets ALL?

Answer 7

Highest incidence in childhood with = M:F

Question 8

What are the features of acute leukeamia?

Answer 8

Bone marrow failure

Question 9

What are the features of bone marrow failure?

Answer 9

Bone marrow failure
Leukopenia (low WCC - fever, infection, mouth ulcers)

Thrombocytopenia (low platelets): bleeding, ecchymoses, petechiae

Anaemia (low RCC): fatigue, SOB on exertion, exacerbation of CCF/IHD

Question 10

What are the features of bone marrow failure?

Answer 10

Leukopenia (low WCC - fever, infection, mouth ulcers)

Thrombocytopenia (low platelets): bleeding, ecchymoses, petechiae

Anaemia (low RCC): fatigue, SOB on exertion, exacerbation of CCF/IHD

Question 11

What are the extramedullary infiltration features of acute leukaemia?

Answer 11

Gingival hyperplasia 
Hepatosplenomegaly and lymphadenopathy 
Spinal cord compression and paraparesis 
Headache and seizures 
Nerve palsies 
Skin rashe/infiltration

Question 12

What tests can be used to classify leukaemia?

Answer 12

Morphology of cells 
Immunophenotyping pattern 
Cytogenetics 
Molecular testing 
Disease behaviour and treatment response

Question 13

What are the AML subtypes with recurrent genetic abnormalaties?

Answer 13

AML with t(8;21) RUNX1-RUNX1T1 


AML with inv(16) CBFβ-MYH11 


AML with t(15;17) PML- RAR α

Question 14

What are the AML subtypes with normal cytogenetics?

Answer 14

AML with mutated NPM1 


AML with FLT–3ITD 


AML with mutated CEBPA

Question 15

What are the three main classifications of AML?

Answer 15

AML with recurrent genetic abnormalities

AML with normal cytogenetics

Therpay related AML

Question 16

What are the 3 main classifications of ALL?

Answer 16

B lymphoblastic leukaemia/lymphoma NOS

B lymphoblastic leukaemia/lymphoma with recurrent genetic abnormalities

T Lymphoblastic leukaemia/lymphoma

Question 17

What is the usual FBC results of someone with leukaemia?

Answer 17

Low Hb, Low platelets, High WCC

Question 18

What will you see in a bone marrow biopsy of leukaemia?

Answer 18

Loss of normal architecture
Loss of fat cells
Deep infiltrate of leukaemic blast cells
Deep blue, basophilic neuclei

Question 19

What will you see in immunophenotyping of AML?

Answer 19

CD33+
CD13+
CD14+
myeloperoxidase +
CD7+/-
HLA-DR+

Question 20

What will you see in immunophenotyping of ALL?

Answer 20

B cell lineage (90%)
CD10+
CD 19+
TdT+
surface or cytoplasmic Ig
T cell lineage (10%)
CD2,3,4,8+/-

Question 21

What are the common genetic mutations of AML?

Answer 21

FLT-3, NPM1, CEBPA

Question 22

What is graft vs. leukaemia

Answer 22

Donor T cells eliminate host malignant T cells

Question 23

What is graft vs, host disease and what are the complications

Answer 23

Occurs when the donor immune cells recognise host cells as foreign and begins to attack them. This can lead to skin rash, diarrhoea, thickening of skin and liver abnormalities

Question 24

What is graft vs, host disease and what are the complications

Answer 24

Occurs when the donor immune cells recognise host cells as foreign and begins to attack them. This can lead to skin rash, diarrhoea, thickening of skin and liver abnormalities

Question 25

What is graft vs. leukaemia

Answer 25

Donor T cells eliminate host malignant T cells (good)

Question 26

What is graft vs, host disease and what are the complications

Answer 26

Occurs when the donor immune cells recognise host cells as foreign and begins to attack them. This can lead to skin rash, diarrhoea, thickening of skin and liver abnormalities

Question 27

What are the general treatment principles in acute leukaemia?

Answer 27

Chemotherapy
Supportive care
Bone marrow transplant
Palliation

Good prognosis AML and ALL have reasonable cure rate with chemo alone

Poor prognosis need bone marrow transplant as well

Question 28

What are the general treatment principles in acute leukaemia?

Answer 28

Chemotherapy
Supportive care
Bone marrow transplant
Palliation

Good prognosis AML and ALL have reasonable cure rate with chemo alone

Poor prognosis need bone marrow transplant as well

Question 29

What are the types of B lymphoblastic leukaemia with recurrent genetic abnormalities?

Answer 29

t(9;22) BCR-ABL
t(12;21) TEL-AML-1
Hyperploidy
Hypoploidy