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Lymphoproliferative Disorders Flashcards

1
Q

Define lymphoma

A

Cancers of lymphoid origin

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2
Q

How can lymphomas present?

A

Lympadenopathy
Extranodal involvement
Bone marrow involvement
Systemic B symptoms

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3
Q

What are the systemic B symptoms?

A

Weight loss (>10% in 6 months), swinging fever, drenching night sweats, pruritus, fatigue

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4
Q

How many types of lymphoma are there?

A

> 50s

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5
Q

How do you find out what type of lymphoma it is?

A

Biopsy (e.g. lymph node/bone marrow)

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6
Q

How do you find out where the lymphoma is?

A

Imaging and clinical Ex (e.g. CT)

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7
Q

What are lymphomas generally split into?

A

Hogdkins and non-hodgkins

NHL is split into high grade and low grade

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8
Q

Why is splitting lymphoma into HL and NHL not very useful?

A

Because HL is only 1/2 specific entities and NHL is everything else

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9
Q

What is the nature of the high grade lymphomas?

A

Fast growing and aggressive - will make you quite ill

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10
Q

What is the nature of the low grade lymphomas?

A

Slow growing, incidental findings

Look more similar to normal lymphocytes

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11
Q

What are the lymphoproliferative disorders?

A

Acute lymphoblastic leukaemia
Chronic lymphocytic leukaemia
Hodgkin lymphoma
Non-Hodgkin lymphoma - high or low grade

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12
Q

Give an example of a high grade NHL and describe what it looks like

A

Diffuse large B cell lymphoma

Appears as big sheet of cells (fast growing and aggressive)

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13
Q

Give two examples of low grade lymphomas

A

Follicular

Marginal zone

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14
Q

What is the most common lymphoma?

A

High grade NHL

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15
Q

Define acute lymphoblastic leukaemia

A

Neoplastic disorder of the lymphoblasts

Diagnosed by >20% lymphoblasts present in bone marrow

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16
Q

In what age do most cases of ALL occur?

A

<6y

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17
Q

ALL tends to affect which lineage of cells?

A

B cell lineage

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18
Q

What is a typical presentation for ALL?

A

2-3 week history of bone marrow failure (anaemia, fatigue, pallor, bleeding, infection) or bone/joint pain

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19
Q

Why might you get bone pain in ALL?

A

Tumour invading bone marrow so quickly it causes necrosis –> bone pain

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20
Q

What investigations should you do in ALL?

A

FBC - will show low Hb, high WCC, low platelets
Blood film
Bone marrow aspirate/biopsy

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21
Q

What is the standard treatment for ALL?

A

Induction and consolidation chemotherapy
CNS (intrathecal chemotherapy)
Maintenance for 18 months
Stem cell transplant if high risk

+ supportive therapy (as you are rendering them v. susceptible to infection, anaemia, thrombocytopenia etc.)

22
Q

Why might some patients require CNS directed therapy?

A

Spread to CNS v common

23
Q

What are some of the newer therapies for ALL?

A

BiTe molecules - bispecific T cell engagers

CAR T-cell therapies

24
Q

What are BiTe molecules?

A

Molecules with a binding site for the tumour and one for T cells

Helps host’s immune system to fight the tumour

25
What are CAR T-cell therapies?
Chimeric antigen receptor T cells Patient/healthy donor T-cells are transfected to express specific T cell receptor on leukaemia cells (CD19) Expanded in vitro Re-infused into patient
26
What may occur as a result of T cell immunotherapy?
Cytokine release syndrome - fever, hypotension, dyspnoea Neurotoxicity - seizure, headache, focal neurology, coma These occur as there is such a heightened immune response
27
What are the poor risk factors for ALL?
Increased age, WCC, more primitive immunophenotype, cytogenetics (e.g. Philadelphia chromosome), slow/poor response to treatment
28
What is required for a diagnosis of CLL?
Blood >5x10^9/L lymphocytes Bone marrow >30% lymphocytes Characteristic immunophenotyping (B cell markers CD19, 20, 23), and CD5+ve (t cell antigen that shouldn't be there)
29
What may you see on the blood film of someone with CLL?
Smear cells (cells disintegrate)
30
In which gender is CLL more common?
2x M
31
What is the most common presentation of CLL?
OFTEN ASYMPTOMATIC
32
What symptoms are associated with CLL?
Bone marrow failure (anaemia, thrombocytopenia, infection) Lymphadenopathy Splenomegaly Fever and sweats Less commonly: hepatomegaly, infections, wt loss
33
What are commonly associated with CLL?
``` Immune paresis (loss of normal Ig) Haemolytic anaemia ```
34
What is the staging system for CLL?
Binet A - <3 LNs - normal survival B - >3 LNs - 8 year survival C - >3 LN + anaemia/thrombocytopenia - 6 year survival
35
What are indications for treatment in CLL?
``` Progressive bone marrow failure Massive lymphadenopathy Progressive splenomegaly Lymphocyte doubling time <6 months or >50% increase over 3 months Systemic symptoms Autoimmune cytopenias ```
36
What are the treatments for CLL?
Wait and watch Cytotoxic chemo - e.g. fludarabine Monoclonal Abs - e.g. rituximab Novel agents - TKIs e.g. ibrutinib; PI3K inhibitors e.g. idelsalisib, BCL inhibitor, e.g. venetoclax
37
What are poor prognostic markers for CLL?
``` Binet B or C Atypical lymphocyte morphology Rapid lymphocyte doubling time (<12 months) CD 38+ expression Loss/mutation of p53, del 11q23 Unmutated IgVH gene status ```
38
How can lymphomas present?
Lympadenopathy/hepatosplenomegaly Extranodal disease B symptoms Bone marrow involvement
39
How do you assess lymphomas?
LN biopsy CT scan Bone marrow aspirate and trephine
40
What is the staging of lymphoma?
Stage 1 - 1 lymph node enlarged Stage 2 - two lymph nodes enlarged on the same side of the diaphragm Stage 3 - lymph nodes enlarged on either side of the diaphragm Stage 4 - metastatic disease
41
What is NHL classified according to?
Lineage (T or B cell) - maj B cell due to germinal centre reaction Grade of disease (high or low) Histological features of disease
42
How can you cure low grade lymphomas?
You can't eve though it responds to chemotherapy
43
How do you treat high grade lymphoma?
Requires combination chemotherapy
44
What is the commonest subtype of lymphoma of any kind?
Diffuse large B cell lymphoma
45
How do you treat diffuse large B cell lymphoma/follicular lymphoma?
Combo chemo - usually anti-CD20 monoclonal Ab and chemo
46
In which age group if HL most common?
Bimodal peak, 15-35y then 70ys
47
In which gender is HL more common?
Males
48
What things are associated with HL?
Epstein barr virus, familial and geographic clustering
49
What is the appearance of a HL tumour?
Hodgkin cell draws in a lot of reactive cells
50
What is the treatment of HL?
``` Combination chemo (ABVD) +/- radiotherapy Monoclonal Ab (anti-CD30) Immunotherapy (checkpoint inhibitors) Use of PET scan to assess response to Rx and limit chemo ```

Haematology (14 decks)

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