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E CAN ext. > Chronic Lymphocytic Leukaemia > Flashcards

Chronic Lymphocytic Leukaemia Flashcards

1
Q

What is chronic lymphocytic leukaemia?

A

A monoclonal expansion of B-lymphocytes with accumulation of abnormal lymphocytes

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2
Q

Where do abnormal lymphocytes accumulate in chronic lymphocytic leukaemia?

A
  • Blood
  • Bone marrow
  • Spleen
  • Lymph nodes
  • Liver
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3
Q

How do the lymphocytes in CLL appear morphologically?

A

Normal

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4
Q

What is abnormal about the lymphocytes in CLL?

A

They are immature and non-reactive

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5
Q

What is the result of the lymphocytes being immature and non-reactive in CLL?

A

Results in immunological compromise

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6
Q

How common is CLL compared to other leukaemias?

A

It represents about 1/4 of all leukaemias seen in clinical practice

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7
Q

When does CLL present?

A

It is largely a disease of older people

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8
Q

What causes CLL?

A

Unknown

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9
Q

What are the known risk factors for CLL?

A
  • Age

- Radiation exposure

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10
Q

Are people in the UK exposed to radiation levels high enough to increase the risk of CLL?

A

No, it occurs after nuclear accidents etc

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11
Q

Describe the presentation of CLL?

A

Variable, with insidious onset

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12
Q

Are most people symptomatic at presentation of CLL?

A

No

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13
Q

How are people diagnosed with CLL asymptomatically?

A

Following routine blood tests

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14
Q

What are the symptoms of CLL?

A
  • Susceptibility to infection
  • Symmetrically enlarged lymph nodes
  • Abdominal discomfort from an enlarged spleen
  • Bleeding or petechiae in skin or mucous membranes
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15
Q

What are the signs of CLL?

A
  • Localised or generalised lymphadenopathy
  • Splenomegaly
  • Hepatomegaly
  • Petechiae
  • Pallor
  • Skin infiltration
  • Tonsillar enlargement
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16
Q

What are the differential diagnoses for CLL?

A
  • Other forms of leukaemia
  • Lymphoma
  • Myelodysplasia and myeloproliferative diseases
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17
Q

What investigations should be done in CLL?

A
  • Blood tests
  • Lymph node biopsy
  • Other tests
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18
Q

What blood tests should be done in CLL?

A
  • FBC
  • Peripheral blood smear
  • Direct anti-globulin test
  • Bone marrow aspirate
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19
Q

What is found on FBC in CLL?

A
  • Minimal clonal B cell lymphocytosis

- Normochromic, normocytic anaemia in advanced diseaes

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20
Q

What is found on peripheral blood smear in CLL?

A

Lymphocytosis, often with smudge cells

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21
Q

Why is a direct anti globulin test required in CLL?

A

Required before treatment to identify autoimmune-related haemolytic anaemias

22
Q

What is found on bone marrow aspirate in CLL?

A

Lymphocytic replacement of normal marrow elements

23
Q

When is a lymph node biopsy required in CLL?

A

If lymph nodes enlarge rapidly

24
Q

Why is a lymph node biopsy required in CLL if lymph nodes enlarge rapidly?

A

To assess the possibility of transformation to a high-grade lymphoma

25
Q

What is Richter’s syndrome?

A

The transformation of CLL into a high grade-lymphoma with enlarged lymph nodes, fever, weight loss, and pain

26
Q

What other tests may be done in CLL?

A
  • Immunophenotyping
  • Measurement of immunoglobulin levels if repeated infection
  • Testing for TP53 gene deletion before treatment
  • Status of relevant infections
27
Q

What infections should you check for in CLL?

A
  • Hep B and C

- Cytomegalovirus

28
Q

When should you check for hep B and C and cytomegalovirus in CLL?

A

Before chemo-immunotherapy or stem cell transplantation

29
Q

What staging system is used for CLL in Europe?

A

Binet system

30
Q

What is stage A CLL?

A
  • Hb at least 10g/L
  • Platelets at least 100x109/L
  • Fewer than 3 lymph nodes areas are involved
31
Q

What is stage B CLL?

A
  • Hb and platelets levels as in stage A

- Three or more lymph nodes areas involved

32
Q

What is stage C CLL?

A

Hb <10g/dL, platelets <100x109/L, or both

33
Q

What is the curative treatment for CLL?

A

Apart from possibly stem cell transplants, there is no curative treatment for CLL

34
Q

What is the role of chemotherapy in CLL?

A

Current standard chemotherapy regimes have been shown to prolong survival

35
Q

When should chemotherapy be given in CLL?

A

Only to patients with active, symptomatic disease

36
Q

What are the indications for chemotherapy in CLL?

A
  • Weight loss >10%
  • Extreme fatigue
  • Fever related to leukaemia
  • Night sweats
  • Progressive marrow failure
  • Autoimmune anaemia or thrombocytopenia not responding to prednisolone
  • Progressive splenomegaly
  • Massive lymphadenopathy
  • Progressive lymphocytosis
37
Q

What is the role of molecular markers in CLL?

A

Molecular markers now make it possible to identify patients more likely to have rapid progression of CLL, or be more resistant to standard treatment

38
Q

What chemotherapy agents are currently used in CLL?

A
  • Alkylating agents
  • Purine analogues
  • Monoclonal antibodies
  • Steroids
39
Q

What might steroids be used for in CLL?

A
  • Treat autoimmune conditions
  • Improve bone marrow function prior to chemotherapy when there is significant bone marrow infiltration
  • Treat CLL that has not responded well to standard chemotherapies
40
Q

What is the only known curative therapy for CLL?

A

Allogenic stem cell transplantation

41
Q

What is the limitation of allogenic stem cell transplantation in CLL?

A

Majority of CLL patients are elderly, and the increased morbidity and mortality of such an intensive approach mean it is rarely justified

42
Q

When might allogenic stem cell transplantation be a useful approach in CLL?

A

In younger patients

43
Q

What treatment is required when there is splenomegaly and pancytopenia in CLL?

A

Splenectomy

44
Q

What is the role of radiotherapy in CLL?

A

May be used palliatively, either for splenic irradiation or external beam radiotherapy for bulky nodal masses

45
Q

When should treatment for CLL relapse be started?

A

Only in symptomatic patients

46
Q

When can first-line therapy be repeated to treat relapse of CLL?

A
  • If relapse or progression occurs at least 24-36 months after chemo-immunotherapy
  • If TP53 deletion/mutation was excluded
47
Q

How much does the median survival of CLL vary?

A

From 18 months to >10 years. depending on the stage

48
Q

Describe the usual course of CLL?

A

Initial course that is relatively benign, followed by a terminal progressive and resistant phase lasting a year or two, followed by late phase

49
Q

What causes morbidity in late phase CLL?

A

Disease and complications of treatment

50
Q

What are older patients likely to die of with CLL?

A

Other causes, including second primary malignancies

E CAN ext. (47 decks)

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