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Haematology > Lymphoproliferative Disorders > Flashcards

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
Q

What are CAR T-cell therapies?

A

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
Q

What may occur as a result of T cell immunotherapy?

A

Cytokine release syndrome - fever, hypotension, dyspnoea

Neurotoxicity - seizure, headache, focal neurology, coma

These occur as there is such a heightened immune response

27
Q

What are the poor risk factors for ALL?

A

Increased age, WCC, more primitive immunophenotype, cytogenetics (e.g. Philadelphia chromosome), slow/poor response to treatment

28
Q

What is required for a diagnosis of CLL?

A

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
Q

What may you see on the blood film of someone with CLL?

A

Smear cells (cells disintegrate)

30
Q

In which gender is CLL more common?

A

2x M

31
Q

What is the most common presentation of CLL?

A

OFTEN ASYMPTOMATIC

32
Q

What symptoms are associated with CLL?

A

Bone marrow failure (anaemia, thrombocytopenia, infection)
Lymphadenopathy
Splenomegaly
Fever and sweats

Less commonly: hepatomegaly, infections, wt loss

33
Q

What are commonly associated with CLL?

A 
Immune paresis (loss of normal Ig) 
Haemolytic anaemia
34
Q

What is the staging system for CLL?

A

Binet
A - <3 LNs - normal survival
B - >3 LNs - 8 year survival
C - >3 LN + anaemia/thrombocytopenia - 6 year survival

35
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 3 months 
Systemic symptoms
Autoimmune cytopenias
36
Q

What are the treatments for CLL?

A

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
Q

What are poor prognostic markers for CLL?

A 
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
Q

How can lymphomas present?

A

Lympadenopathy/hepatosplenomegaly
Extranodal disease
B symptoms
Bone marrow involvement

39
Q

How do you assess lymphomas?

A

LN biopsy
CT scan
Bone marrow aspirate and trephine

40
Q

What is the staging of lymphoma?

A

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
Q

What is NHL classified according to?

A

Lineage (T or B cell) - maj B cell due to germinal centre reaction
Grade of disease (high or low)
Histological features of disease

42
Q

How can you cure low grade lymphomas?

A

You can’t eve though it responds to chemotherapy

43
Q

How do you treat high grade lymphoma?

A

Requires combination chemotherapy

44
Q

What is the commonest subtype of lymphoma of any kind?

A

Diffuse large B cell lymphoma

45
Q

How do you treat diffuse large B cell lymphoma/follicular lymphoma?

A

Combo chemo - usually anti-CD20 monoclonal Ab and chemo

46
Q

In which age group if HL most common?

A

Bimodal peak, 15-35y then 70ys

47
Q

In which gender is HL more common?

A

Males

48
Q

What things are associated with HL?

A

Epstein barr virus, familial and geographic clustering

49
Q

What is the appearance of a HL tumour?

A

Hodgkin cell draws in a lot of reactive cells

50
Q

What is the treatment of HL?

A 
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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