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Year 3 Haematology Immunology > Lymphoproliferative disorders > Flashcards

Lymphoproliferative disorders Flashcards

1
Q

What are lymphomas?

What do lymphomas tend to present with (hallmark feature)?

A

Cancers of lymphoid origin

Typically present with enlarged lymph nodes (lymphadenopathy)

but occasionally present with:

  • extranodal involvement
  • bone marrow involvement
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2
Q

What are the general symptoms seen with lymphoma?

A

Lymphadenopathy (rubbery)

Systemic symptoms:

  • weight loss
  • fever
  • fatigue
  • drenching night sweats
  • pruritis
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3
Q

How is a diagnosis of lymphoma (or leukaemia) made?

How is it staged?

A

Biopsy - eg lymph node, bone marrow, extranodal mass

Diagnosis made based on malignant cell characteristics - not on clinical presentation or any of that stuff

Staging of disease based on clinical examination and imaging (CT etc)

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4
Q

What groups of lymphoproliferative disorders involve:

a) lymphoid progenitor cells
b) B & T lymphocytes
c) Plasma cells

A

a) Acute Lymphoblastic leukaemias (ALL)
b) Chronic lymphocytic leukaemias (CLL) & Lymphomas
c) Multiple myeloma* (not in this lecture)

*not sure if its a lymphoproliferative disorder

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5
Q

Where do the following groups of disorders tend to occur:

a) ALL
b) CLL
c) Lymphoma
d) MM

A

a) Acute lymphoblastic leukaemia = Bone marrow
b) Chronic lymphocytic leukaemia = Marrow/nodes
c) Lymphoma = lymph nodes
d) Multiple myeloma = bone marrow

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6
Q

What are the types of lymphoma?

A

Hodgkin Lymphoma

Non-hodgkin lymphoma:

  • high grade non-hodgkin
  • low grade non-hodgkin

Hodgkin = specific disease

Non-hodgkin is everything else and has lots of discrete types

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7
Q

What are the most common lymphoproliferative disorders?

(most to least common)

A

1) Non-Hodgkin lymphoma:

  • High grade - most common high grade is diffuse large B cell lymphoma
  • Low grade - most common low grade is follicular and marginal zone

2) Chronic lymphocytic leukaemia (CLL)

3) Hodgkin lymphoma

4) Acute lymphoblastic leukaemia (ALL)

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8
Q

What happens in ALL?

A

Acute lymphoblastic leukaemia

Rapid, uncontrolled proliferation (but no differentiation) of lymphoid progenitor cells

This causes an accumulation of these ‘blast’ cells, usually in the bone marrow

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9
Q

Concerning the epidemiology n stuff of ALL…

a) How common is it?
b) What age group is most commonly affected?
c) What lineage of cells is most commonly affected?

A

a) Rarest lymphoproliferative disorder
b) Usually in children < 6 - most common blood cancer of children
c) B-cell ALL is most common

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10
Q

What is the typical presentation of ALL?

A

2-3 week history of bone marrow failure (anaemia symptoms etc) & bone/joint pain

May have systemic symptoms (as with all leukaemias) of fatigue, weight loss etc

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11
Q

What blood count would you expect to see in ALL?

What would the bone marrow show?

A

Low haemoglobin

Very high white cell count

Very low platelets

Bone marrow would be dominated by lymphoblasts (‘blasts’) - usually B-lymphoblasts

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12
Q

A patient presents with typical symptoms of Acute Leukaemia and has a blood count in keeping with this…

Her blood film shows lots of blasts, however it is difficult to differentiate between lymphoblasts and myeloid blast cells

What testing is needed to diagnose ALL?

What results would give this diagnosis?

A

Immunophenotyping

(shows specific molecules expressed that are unique to the blasts)

CD19 - expressed in B cell lineage

CD34 & TDT - markers of very immature cells which should not be in the blood

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13
Q

How is ALL treated?

A

Multi-agent chemotherapy

3 phases…

1) Induction - which aims to achieve remission
2) Consolidation - patient may receive allogeneic stem cell transplant if high risk
3) Maintenance - for 18 months

CNS directed therapy

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14
Q

What are risk factors for a poor outcome in ALL?

A

Increasing age

Increased white cell count in blood

Slow/poor response to treatment

Cytogenetics:

Philadelphia chromosome (t9;22)

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15
Q

What is the prognosis for ALL in:

adults

children

A

Adults = good but many go into remission and need bone marrow transplant

Children = very good - 90% 5 year survival

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16
Q

How is CLL different from ALL, in terms of:

a) maturity
b) proliferative speed

A

a) maturity
* in CLL, the cells are much more mature
b) proliferative speed
* proliferate slowly (often dont at all) which means CLL has a much more gradual onset than ALL - hence ‘chronic’

17
Q

What lymphocyte count is needed for a diagnosis of CLL?

A

Lymphocyte count > 5

Normal is < 4

18
Q

Describe the epidemiology n stuff of CLL

A

Commenest leukaemia worldwide - but remember NHL is more common than this

Tends to affect adults

More common in men (2:1)

Occasionally familial

Rare in far east

19
Q

How does CLL present?

A

Often asymptomatic

If symptomatic:

  • bone marrow failure (anaemia, thrombocytopenia)
  • lymphadenopathy
  • splenomegaly
  • fever & sweats

Others - hepatomegaly, infections, weight loss

20
Q

What 2 conditions are associated with CLL, and may be part of the presentation?

A

Immune paresis

  • loss of normal immunoglobulin production which leads to infections

Autoimmune cytopenias - specifically haemolytic anaemia

21
Q

How is CLL staged?

A

Binet staging system

  • A - <3 lymph node areas involved
  • B - 3 or more lymph node areas
  • C - Stage B + anaemia or thrombocytopenia
22
Q

What are the indications for treatment for CLL?

A

Progressive bone marrow failure

Massive lymphadenopathy

Progressive splenomegaly

Rapid lymphocyte proliferation:

  • doubling time < 6 months
  • > 50% increase in 2 months

Systemic symptoms

Autoimmune cytopenias

23
Q

Give an overview of the treatment for CLL

A

“Watch and wait”

  • if indications for treatment:*
    1) Cytotoxic chemotherapy
    2) Monoclonal antibodies
    3) Novel agents - eg Bruton TKIs ‘i**brutinib’
24
Q

What are poor prognostic factors for CLL?

A

Advanced disease (Binet B or C)

Atypical lymphocyte morphology

Rapid lymphocyte proliferation (doubling time <12 months)

Genetics:

  • p53 mutations
  • CD38+ expression
25
Q

Describe the classic presentation of lymphoma

A

Lymphadenopathy

Hepatosplenomegaly

Extranodal disease

Systemic or ‘B’ symptoms - fever, fatigue etc

Sx of Bone marrow involvement

26
Q

How are lymphomas staged?

A

Lymph node biopsy (more for diagnosis)

CT scan

Bone marrow aspirate and trephine

Staging is classed using the Ann Arbor system

27
Q

What are the different stages of lymphoma?

A

Ann arbor staging (IA - IVB)

  • I - 1 nodal site of involvement
  • II - 2 nodal sites both above or below diaphragm
  • III - multiple nodal sites above and below diaphragm
  • IV - any extranodal involvement eg liver
  • A - no systemic symptoms
  • B - systemic “B” symptoms - fever, sweats etc
28
Q

What are the different classes of Non-hodgkin lymphoma (NHL)?

A

Classified according to:

  • Lineage (B or T cell) - B cell most common (90%)
  • Grade (High or Low)
29
Q

What are the main differences between High and Low grade NHL?

A

Low grade lymphoma:

  • indolent, often asymptomatic
  • responds to chemo, but uncurable

High grade lymphoma:

  • aggressive, fast growing
  • requires combination chemotherapy
  • is curable
30
Q

What is the most common type of High grade NHL?

How is it treated?

A

Diffuse large B cell (NH) Lymphoma

Treated with combination chemotherapy:

Anti-CD20 monoclonal antibody + chemo

31
Q

What is the most common type of low grade NHL?

A

Follicular lymphoma

Treated same as Diffuse large B cell lymphoma- ie combination chemotherapy:

Anti-CD20 monoclonal antibody + chemo

EXCEPT - “watch and wait” approach similar to CLL. Leave alone if not causing problems

32
Q

For Hodgkin lymphoma:

a) How common is it?
b) What age does it commonly present at?
c) What sex is most likely to get it?
d) What infection is it associated with?

A

a) 30% of all lymphomas
b) Bimodal age peak:

  1. 15-35 years old
  2. older adults

c) Males (1.9:1)
d) EBV

33
Q

Give an overview of the treatment of Hodgkin lymphoma

A

Very curable disease…

Management:

1) Combination chemotherapy - ABVD

+/- Radiotherapy

2) Monoclonal antibodies
3) Immunotherapy

34
Q

In the management of Hodgkin lymphoma - how is the response to treatment assessed?

A

PET Scanning

Year 3 Haematology Immunology (22 decks)

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