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Haematology > Chronic Lymphoblastic Leukaemia > Flashcards

Chronic Lymphoblastic Leukaemia Flashcards

1
Q

What is chronic lymphoblastic leukaemia?

A

https://www.youtube.com/watch?v=Wn3fylOqUZU

Results from the clonal expansion of small lymphocytes and is almost invariably (95%) B cell in origin.

Accumulation of mature B cells that have escaped programmed cell death and undergone cell cycle arrest in the G0/G1 phase is the hallmark of CLL

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2
Q

What is the difference in cell behaviour between CML and CLL?

A
  • CML - cells divide to quickly
  • CLL - cells don’t die when they should
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3
Q

Which sex is CLL more common in?

A

Males

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4
Q

What can be a trigger for the development of CLL?

A

Pneumonia

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5
Q

What are symptoms of CLL?

A

Mainly asymptomatic

  • Recurrent infection
  • Symptoms of Anaemia
  • Painless lumps
  • Left upper quadrant discomfort
  • Weight loss
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6
Q

Why might someone with CLL get recurrent infections?

A

Due to functional leucopenia and immune failure (reduced immunoglobulins)

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7
Q

What can cause anaemia in CLL?

A
  • Autoimmune haemolytic anaemia
  • Marrow infiltration
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8
Q

Why might someone with CLL have upper left quadrant discomfort?

A

Splenomegaly

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9
Q

What signs might someone with CLL have?

A
  • Anaemia
  • Fever
  • Lymphadenopathy - may be single area or generalised
  • Hepatosplenomegaly - sometimes massive
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10
Q

What is the characteristic feature of lymphadenopathy seen in CLL?

A

Enlarged, rubbery, non-tender nodes

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11
Q

What investigations might you do for someone with suspected CLL?

A
  • Blood count
  • Blood film
  • Bone marrow.
  • Immunophenotyping
  • Direct Coombs’ test
  • Immunoglobulins
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12
Q

What might you see on Blood count in someone with CLL?

A
  • Hb - normal or low
  • WBC - raised and may be very high; with lymphocytosis
  • Platelets - normal or low.
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13
Q

What might you see on blood film in someone with CLL?

A
  • Small/medium sized mature and normal appearing lymphocytes
  • Smudge cells
  • No immature blasts
  • Haemolysis
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14
Q

Why would you do direct coombs test in someone with CLL?

A

To check for autoimmune haemolysis

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15
Q

What might you find when investigating serum immunoglobulins?

A

Hypogammaglobulinaemia - prone to infection

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16
Q

What might you find in the bone marrow of someone with CLL?

A

Reflects peripheral blood, often very heavily infiltrated with lymphocytes.

17
Q

What might you find when immunophenotyping someone for CLL?

A

CD19+, CD5+, CD23+ B cells with weak expression of CD20, CD79b and surface immunoglobulin

18
Q

What are complications of CLL?

A
  • Autoimmune haemolysis
  • Increased risk of infection due to hypogammaglobulinaemia
  • Marrow failure
19
Q

What are individuals most likely to die of in CLL?

A

Infection

20
Q

What organism most commonly cause death in those with CLL?

A
  • Pneumococcus
  • Meningococcus
  • Haemophilus
  • Candidiasis
  • Aspergillosis
21
Q

What are indications for treatment in CLL?

A
  • Progressive bone marrow failure
  • Massive lymphadenopathy
  • Progressive splenomegaly
  • Lymphocyte doubling time <6 months or >50% increase over 2 months
  • Systemic symptoms
  • Autoimmune cytopenias
22
Q

How would you manage someone with CLL?

A
  • None/supportive care/IV immunoglobulins
  • Cytotoxic chemotherapy
  • Monoclonal antibodies
  • Corticosteroids
  • Radiotherapy
  • Novel agents
    • Bruton tyrosine kinase inhibitor
    • PI3K inhibitor
    • BCL-2 inhibitor
23
Q

What cytotoxic chemotherapies are used in treating CLL?

A
  • Fludarabine
  • Bendamustine
24
Q

What monoclonal antibodies are used in treating CLL?

A
  • Rituximab
  • Obinutuzamab
  • Alemtuzumab
  • Ofatumumab
25
Q

Why might steroids be used in management of CLL?

A

Managing autoimmune haemolysis

26
Q

What is radiotherapy used for in treating CLL?

A

Helps lymphadenopathy and splenomegaly

27
Q

What is the prognosis for CLL?

A
  • 1/3 never progress
  • 1/3 progress slowly
  • 1/3 progress actively
28
Q

What are poor prognostic markers in CLL?

A
  • Advanced disease (Binet stage B or C)
  • Atypical lymphocyte morphology
  • Rapid lymphocyte doubling time (<12 mth)
  • CD 38+ expression
  • Loss/mutation p53; del 11q23 (ATM gene)
  • Unmutated IgVH gene status
29
Q

What is the first line treatment for CLL?

A

Chlorbumacil combined with prednisone - associated with 60-90% intital response rate and a complete response in up to 20% of all patients.

30
Q

What is the characteristic cell type seen on blood film in CLL?

A

Smudge cells

Haematology (34 decks)

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