Haematological Malignancy Flashcards

1
Q

What percentage of human cancers are accounted for by haematological malignancies?

A

Approx 10%

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2
Q

In adults, what sex is more commonly affected by haematological malignancy?

A

Males > females

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3
Q

How can you identify the population of stem cells in bone marrow?

A

Immune phenotyping

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4
Q

What percentage of bone marrow cells are accounted for by stem cells?

A

< 0.1%

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5
Q

What are the origins of bone marrow malignancies?

A

Myeloid compartment - erythrocytes, platelets, monocytes, granulocytes

Lymphoid compartment - B cell and T cell

Mutations may arise in haematopoietic stem cell or in progenitor cells

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6
Q

What does the phenotype of disease depend on?

A

Features of mutation

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7
Q

What does a mutation resulting in proliferation without differentiation cause?

A

Accumulation of useless progenitor cells which causes acute leukaemia
If this process is in myeloid line then acute myeloid leukaemia
If in lymphoid line then acute lymphoblastic leukaemia

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8
Q

What does a mutation resulting in proliferation and differentiation cause?

A

Overproduction of end cells leading to myeloproliferative disease e.g. chronic lymphocytic leukaemia

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9
Q

What kind of malignancies are CLL, lymphomas and MM?

A

Mature lymphoid malignancies

Mutational events here do not occur in stem cell compartment, they occur as cells pass through germinal centre of secondary lymphoid organs

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10
Q

In what cell does a mutation occur that results in acute lymphoblastic leukaemia?

A

Lymphoid progenitor

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11
Q

In what cell does a mutation occur that results in acute myeloid leukaemia?

A

Myeloid progenitor

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12
Q

What is the difference between leukaemia and lymphoma in terms of where they occur?

A

If disease predominantly occurs in blood and bone marrow then leukaemia

If disease predominantly occurs in lymph nodes and solid organs then lymphoma

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13
Q

What is the commonest type of leukaemia?

A

Chronic lymphocytic leukaemia

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14
Q

If a patient presents with widespread lymphadenopathy, normal FBC, with blood films and genetic testing showing CLL, what is the diagnosis?

A

Small cell lymphocytic lymphoma

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15
Q

What are the major groups of haematological malignancies?

A

Acute leukaemias

  • acute lymphoblastic leukaemia
  • acute myeloid leukaemia

Chronic leukaemias

  • chronic myeloid leukaemia
  • chronic lymphocytic leukaemia

Malignant lymphomas

  • non-Hodgkin lymphoma
  • Hodgkin lymphoma

Multiple myeloma

Myelodysplastic syndromes

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16
Q

What are the features of acute leukaemia?

A

Leukaemic cells do not differentiate - proliferation without differentiation so bone marrow fills up with useless immature cells and bone marrow fails
Rapidly fatal if untreated
Potentially curable

17
Q

What are the features of chronic leukaemia?

A

Leukaemic cells retain ability to differentiate
Proliferation without bone marrow failure
Not presently curable but slow progression with generally good survival

18
Q

What are the clinical features of acute leukaemia?

A

Bone marrow failure
Anaemia
Thrombocytopenic bleeding
Infection due to neutropenia

19
Q

In lymphoma, where do mutational events tend to occur?

A

In more mature cells

In B cell lymphoma mutational events occur during transit through germinal centre

20
Q

Somatic hypermutation of immunoglobulin genes occurs to create a good match for antigen, this requires a lot of

A

cell division - huge pressure on immunoglobulin gene so potential source for mutation

21
Q

What are the presentations of lymphoma?

A

Nodal disease
- lymphadenopathy

Extra-nodal disease

Systemic symptoms

  • fever (B symptom)
  • drenching night sweats (B symptom)
  • loss of weight (10% body weight loss = B symptom)
  • pruritus
  • fatigue
22
Q

What percentage of Hodgkin lymphoma presents with nodal disease?

A

> 90%

23
Q

What percentage of non-Hodgkin lymphoma presents with purely nodal disease?

A

Around 60%

24
Q

What percentage of non-Hodgkin lymphoma presents with extra-nodal component?

A

40%

25
Q

What are the types of lymphadenopathy and their causes?

A

Localised and painful
- bacterial infection in draining site

Localised and painless

  • rare infections e.g. TB
  • metastatic carcinoma from draining site (hard)
  • lymphoma (rubbery)
  • reactive (no cause identified)

Generalised and painful/tender
- viral infections e.g. EBV, CMV, hepatitis, HIV

Generalised and painless

  • lymphoma
  • leukaemia
  • connective tissue diseases
  • reactive
  • drugs
26
Q

What are the clinical features of multiple myeloma?

A
Bone pain and lytic lesions 
Anaemia 
Recurrent infections 
Renal failure 
Amyloidosis 
Bleeding tendency 
Hyperviscosity syndrome