Haem: Acute Leukaemia Pt.2 Flashcards

1
Q

Outline the tests that may be used to diagnose AML.

A
  • Blood film (DIAGNOSTIC) - can see circulating blast cells
  • Bone marrow aspirate
  • Cytogenetic studies (done in EVERY patient)
  • Molecular studies and FISH
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2
Q

What is the significance of cytogenetic and molecular analysis in AML

A

Prognostic value and guides treatment

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3
Q

What is aleukaemia leukaemia?

A

When there are no leukaemic cells in the peripheral blood but the bone marrow has been replaced

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4
Q

Outline the treatment for AML.

A

Chemotherapy

BM transplant if treatment resistant

Supportive (important for patient to survive chemo)

  • Red cells
  • Platelets
  • FFC/cryoprecipitate in DIC
  • Antibiotics
  • Allopurinol (prevent gout)
  • Fluid and electrolyte balance
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5
Q

Describe the chemotherapy regime in AML

A
  • Combination chemotherapy is ALWAYS used
  • Usually given as 4-5 courses: 2x remission induction + 2/3x consolidation
  • Treatment usually lasts around 6 months
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6
Q

List some determinants of prognosis in AML.

A
  • Patient characteristics
  • Morphology
  • Immunophenotyping
  • Cytogenetics
  • Genetics
  • Response to treatment
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7
Q

How is AML differentiated from ALL

A

Immunophenotyping: identifies cell surface and cytoplasmic antigens

  • Flow cytometry
  • Immunocytochemistry
  • Immunohistochemistry
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8
Q

Describe the epidemiology of ALL

A

Peak incidence in childhood (most common childhood malignancy)

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9
Q

Outline the clinical features of ALL.

A

Systemic symptoms

Bone marrow failure

  • Anaemia
  • Neutropenia
  • Thrombocytopenia

Local infiltration

  • Lymphadenopathy
  • Splenomegaly
  • Hepatomegaly
  • Bone marrow
  • Testes, CNS
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10
Q

What is seen on peripheral blood smear and BM biopsy in ALL?

A

Lymphoblasts

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11
Q

What is a key difference in the origin of B-lineage and T-lineage ALL?

A
  • B-lineage starts in the bone marrow
  • T-lineage can start in the thymus (which may be enlarged)
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12
Q

List some possible leukaemogenic mechanisms in ALL.

A

Protooncogene dysregulation due to chromosomal abnormalities

  • Fusion genes
  • Wrong gene promotor
  • Dysregulation due to proximity to TCR or Ig heavy chain loci
  • Hyperdiploidy - mechanism unknown
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13
Q

List some investigations used in the diagnosis of ALL.

A
  • FBC and blood film
  • Bone marrow aspirate
  • Immunophenotyping
  • Cytogenetic/molecular analysis
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14
Q

Why is immunophenotyping important in ALL

A
  • Differentiate between AML and ALL (treated differently)
  • Differentiate between B cell and T cell lineage (treated differently)
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15
Q

Why is cytogenetic/molecular analysis important in ALL

A

Prognosis and treatment guidance:

  • Philidephia chr positive ALL requires imatinib
  • Treatment must be tailored to prognosis
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16
Q

What are the general principles of ALL treatment

A

Specific therapy

  • Systemic chemotherapy
  • CNS-directed therapy
  • Targeted molecular therapy
  • BM transplant

Supportive care

  • Blood products
  • Antibiotics
  • General medical care (central line, gout management, hyperkalaemia management, sometimes dialysis)
17
Q

What are the four phases of chemotherapy for ALL?

A
  • Remission induction
  • Consolidation and CNS therapy
  • Intensification
  • Maintenance
18
Q

How long does chemotherapy for ALL usually take? Why is it longer in boys?

A

2 years for girls, 3 years for boys

Longer in boys because the testes are a site of accumulation of lymphoblasts

19
Q

Who receives CNS-directed chemotherapy? How can this be given?

A
  • All patients should receive CNS-directed chemotherapy
  • This can be given intrathecally or a high dose of chemotherapy could be given such that it penetrates the BBB
20
Q

Give 2 examples of targeted molecular treatments in ALL

A
  • Tyrosine kinase inhibitors for Ph-positive ALL
  • Rituximab for CD20 positive ALL