Leukaemia Flashcards

1
Q

What is leukaemia?

A

Leukaemia is a type of cancer caused by the unregulated proliferation of a clone of immature cells derived from mutant haematopoietic stem cells

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2
Q

In what sex is leukaemia more common?

A

Men

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3
Q

What are some of the predisposing factors for leukaemia?

A
Radiation exposure
Previous chemotherapy
Occupational chemical exposure
Genetic disorders
Viral infection
MDS and myeloproliferative disorders
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4
Q

Besides acute and chronic leukaemias, what are some of the less common types of leukaemia?

A

Hairy cell leukaemia
Prolymphocytic leukaemia
T-cell leukaemia lymphoma

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5
Q

What is acute myeloid leukaemia?

A

AML is a malignant disorder of haematopoietic progenitor cells, specifically myeloid cells

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6
Q

In what age group does acute myeloid leukaemia usually occur?

A

> 60 years

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7
Q

What are the possible symptoms of acute myeloid leukaemia?

A

Symptoms of anaemia, infection and haemorrhage due to bone marrow failure

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8
Q

What subtype of acute myeloid leukaemia is particularly. associated with gum infiltration, lymphadenopathy, skin deposits and hepatosplenomegaly?

A

Acute monocytic leukaemia

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9
Q

CNS disease is rare in acute myeloid leukaemia. T/F?

A

True - though it is most common in monocytic / monoblastic leukaemia when it does occur

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10
Q

Which subtype of acute myeloid leukaemia is associated with DIC and a high risk of spontaneous bleeding into vital organs?

A

Acute promyelocytic leukaemia

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11
Q

What results would you expect to see on blood count and film in acute myeloid leukaemia?

A

WCC elevated with anaemia and thrombocytopenia

Leukaemic blast cells present. Possible dysplastic changes in other cells

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12
Q

What diagnostic tests should be performed for possible acute leukaemia?

A
Blood count
Blood film
Bone marrow aspirate and trephine biopsy
Cytochemistry
Immunophenotyping
Cytogenetics
Molecular biology
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13
Q

What is the purpose of cytochemistry and immunophenotyping in suspected acute leukaemia?

A

To help differentiate between AML and ALL

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14
Q

What results would you expect to see on bone marrow aspirate and trephine biopsy in acute myeloid leukaemia?

A

Bone marrow infiltrated by leukaemic blast cells

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15
Q

What is involved in supportive treatment for acute leukaemia?

A

RBC transfusion for anaemia
Platelet concentrates for thrombocytopenia
Broad-spectrum IV antibiotics for infection
Indwelling venous catheter facilitates this support during and after chemotherapy

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16
Q

When would autologous stem cell transplantation be used in the treatment of acute myeloid leukaemia?

A

It is mostly offered to younger patients with high risk of relapsed disease

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17
Q

What is the prognosis of acute myeloid leukaemia?

A

5-year survival is fairly good for younger patients with standard risk disease
Elderly patients have a poorer prognosis as they are less able to tolerate intense chemotherapy

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18
Q

What cell type is predominantly involved in the majority of acute lymphoblastic leukaemia?

A

B lymphocytes

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19
Q

In what age group is acute lymphoblastic leukaemia more common?

A

Children

20
Q

What are the possible symptoms of acute lymphoblastic leukaemia?

A
Symptoms of bone marrow failure: symptoms of anaemia, haemorrhage and infection
Anorexia
Back or joint pain
CNS symptoms
Large mediastinal mass 
Lymphadenopathy
Hepatosplenomegaly
21
Q

CNS involvement is more common in ALL than AML. What CNS symptoms might these patients present with?

A

Symptoms of raised intracranial pressure - headache, vomiting
Cranial nerve palsies - particularly VI and VII

22
Q

What results would you expect to see on blood count and film in acute lymphoblastic leukaemia?

A

WCC can be raised, normal or low
Anaemia and thrombocytopenia
Presence of blast cells

23
Q

What is the relevance, in terms on the implications for treatment, of the fact that CNS disease is more common in ALL than AML?

A

The higher chance of CNS disease in ALL requires prophylactic treatment via intrathecal chemotherapy with the possible addition of cranial irradiation for high risk patients

24
Q

What is the prognosis of acute lymphoblastic leukaemia?

A

The majority of children with ALL are curable with chemotherapy and 5-year survival is good. The majority of adults enter remission but are not curable with chemotherapy alone so <40% are long-term survivors

25
Q

What is chronic myeloid leukaemia?

A

CML is a clonal myeloproliferative disorder which results from an acquired genetic change in a pluripotent stem cell.

26
Q

Which cell in particularly is overproduced in chronic myeloid leukaemia?

A

Neutrophils

27
Q

What are the three phases of chronic myeloid leukaemia?

A

Chronic phase
Accelerate phase
Blast crisis

28
Q

In what age group is chronic myeloid leukaemia most common?

A

50s

60s

29
Q

Describe the pathophysiology of chronic myeloid leukaemia

A

CML is characterised by the presence of the Philadelphia chromosome (Ph) which is a modified chromosome 22 caused by a translocation between chromosomes 9 and 22. In the Ph chromosome, the Bcr and ABL genes end up sitting next to each other to form the fusion gene BCR-ABL. This gene codes for a protein of the same name that acts as a tyrosine kinase and is constitutively active. This causes a rapid division of myeloid cells leading to a build-up of premature leucocytes in the bone marrow, which eventually spill out into the blood

30
Q

Explain how chronic myeloid leukaemia can progress to the blast crisis stage

A

The rapid division of myeloid cells in CML, increases the opportunity for further genetic mutations to occur, These further mutations can cause SML to progress to. a more serious acute leukaemia. This is often due to a doubling of the Philadelphia chromosome or a trisomy of chromosome 8

31
Q

In what phase of chronic myeloid leukaemia do patients usually present?

A

Chronic phase

32
Q

What are the typical symptoms of the chronic phase of chronic myeloid leukaemia?

A

Anaemia
Anorexia
Weight loss
Splenomegaly. - can cause pain, bloating and satiety

33
Q

Neutropenia and thrombocytopenia are commonly seen in the chronic phase of chronic myeloid leukaemia. T/F?

A

False - this is why infection and haemorrhage are rare for these patients

34
Q

The high WCC in chronic myeloid leukaemia can lead to what syndrome?

A

Hyperviscosity syndrome

35
Q

What are the expected blood count and film results in. chronic myeloid leukaemia?

A

Elevated WCC
Increase in morphologically normal myeloid cells at different stages of differentiation, with the greatest numbers of myeloblasts and neutrophils (in chronic phase)
Presence of myeloblasts / lymphoblasts (in blast crisis)
Absolute basophilia

36
Q

What is the key diagnostic test for chronic myeloid leukaemia?

A

Genetic testing for the Philadelphia chromosome

37
Q

What is chronic lymphocytic leukaemia?

A

A disease characterised by a clonal proliferation of antigen-stimulated mature B-lymphocytes

38
Q

What is the most common type of leukaemia in the western world?

A

Chronic lymphocytic leukaemia

39
Q

In what age group is chronic lymphocytic leukaemia most common?

A

> 60 years

40
Q

Describe the pathophysiology of chronic lymphocytic leukaemia?

A

CLL is caused by mutations which interfere with B cell receptor pathways, allowing these cells to resist apoptosis. This causes a build up of cells in the bone marrow which then spills out into the blood. Many of these cells will settle in the lymph nodes and spleen causing lymphadenopathy and splenomegaly

41
Q

What are the common symptoms of chronic lymphocytic leukaemia?

A
Anaemia
Lymphadenopathy
Unusually persistent or severe ifnections
Weight loss
Splenomegaly
42
Q

In advanced cases of chronic lymphocytic leukaemia, what tissue may be infiltrated by leukaemic cells?

A
Skin
GI tract
CNS
Lungs
Kidneys
Bone
43
Q

What causes immunodeficiency in chronic lymphocytic leukaemia?

A

Hypogammaglobulinaemia

44
Q

Unexplained persistent lymphocytosis in an elderly person should always suggest which leukaemia?

A

Chronic lymphocytic leukaemia

45
Q

What are the expected findings on blood count and film in chronic lymphocytic leukaemia?

A

High lymphocyte count

Smear cells on blood film - these are large lymphocytes which. have burst during the preparation process

46
Q

Many patients with chronic lymphocytic leukaemia don’t require treatment initially. When should treatment be commenced?

A

Patient develops significant symptoms
Disease is progressing rapidly
Disease is already at an advanced clinical stage

47
Q

What drugs can be used for patients with chronic lymphocytic leukaemia with pancytopenia or autoimmune complications?

A

Steroids