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Lymphoma Flashcards

1
Q

90% of lymphomas are …

A

Non-Hodgkin lymphoma

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2
Q

90% of non-hodgkin lymphomas are…

A

B-NHL

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3
Q

What are the 2 most common types of B-NHL?

A

DLBCL 2/3 + follicular lymphoma 1/3

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4
Q

List viruses/pathogens involved in the development of lymphomas

A 
HIV
EBV
HTLV-1
HHV-8
Hep C
H pylori 
Borrela burgdorferi
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5
Q

List the clinical features of lymphoma

A 
LOW >10% 
Night sweats
Fever
lymphadenopathy 
Hepatosplenomegaly
Abdo pain (bulky disease)
Exranodal disease occurs in 20% 
- GIT (mucosa associated lymphoid tissue ie MALT)
- Skin (T cell lymphoma)
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6
Q

Is it common to find abnormal bloods in lymphoma?

A

No (compared to leukaemia)

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7
Q

How to diagnose lymphoma?

A

Excision biopsy (not FNA or core biopsy)
BM biopsy (due to the sensitivity of PET, most HL and DLBCL can be spared BM biopsy)
CTCAP
PET-CT (useful in mid-treatment too)

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8
Q

How do you stage lymphoma?

A

Ann-Arbor staging

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9
Q

What are the stages of Ann-Arbor staging?

A

Stage 1: single group of nodes (single radiation field)
Stage 2: >1 group of nodes but on same side of diaphragm
Stage 3: Cross diaphragm
Stage 4: Diffuse extranodal involvement

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10
Q

List 5 types of B-NHL

A 
Follicular lymphoma
Mantle cell lymphoma
Marginal zone lymphoma
DLBCL
Burkitt cell lymphoma
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11
Q

List 4 indolent/low grade B-NHL

A

Follicular lymphoma
Marginal zone lymphoma
MALT
Small lymphocytic lymphoma (aka CLL)

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12
Q

List 3 aggressive/intermediate grade B-NHL

A

Mantle cell lymphoma
DLBCL
Peripheral T cell lymphomas

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13
Q

List 2 very aggressive/high grade B-NHL

A

Burkitt cell lymphoma

T lymphoblastic lymphoma

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14
Q

Follicular lymphoma is the neoplastic proliferative of …

A

Small B cells

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15
Q

How does follicular lymphoma present?

A

Painless lymphadenopathy

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16
Q

Pathogenesis of follicular lymphoma

A

t(14;18) –> overexpression of Bcl2 –> reduced apoptosis

CD10+/19+/20+ BCL2+ BCL6+

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17
Q

Rx follicular lymphoma

A

Watch and wait ~2 years or so; 10% never need treatment

Rituximab or obintuzumab + CVP (cyclophosphamide, vincristine, pred)

Rituximab or obintuzumab + bendamustine

Rituximab or obintuzumab + CHOP

All acceptable options except obinutuzumab-bendamustine has increased infections in age >70; not favoured in covid era

Ab maintenance is given every 2 months for 2 years - to increase disease response but increases infections

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18
Q

Rx for relapsed follicular lymphoma

A 
Chemo + rituximab --> rituximab maintenance 
Add anthracycline (doxorubicin) if EF >50%

Obinutuzumab + bendamustine is an option

ASCT for young

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19
Q

Marginal zone lymphoma is associated with …

A

Chronic inflammatory states e.g. H.pylori gastritis, Hashimoto thyroiditis, Sjogren’s

MALT - marginal lymphoma in mucosal sites

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20
Q

Rx for marginal zone lymphoma

A

Treat the underlying inflammation

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21
Q

DLBCL can transform from…

A

Follicular lymphoma or arise sporadically

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22
Q

How does DLBCL present?

A

Single growing lymph node/extranodal mass +/- B symptoms +/- BM involvement

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23
Q

What’s the most common type of NHL?

A

DLBCL

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24
Q

Rx for DLBCL

A

Rituximab + CHOP (cyclophosphamide + doxorubicin + vincristine + pred)

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25
What are side effects of CHOP (cyclophosphamide + vincristine + doxorubicin + pred)?
Cytopenia (mid cycle D7-11; especially first cycle) - Febrile neutropenia - Add C-GSF (filgastrim) to reduce risk especially in elderly and 1st cycle - Hair loss - N&V - Vincristine: peripheral neuropathy, constipation - Anthracycline (doxorubicin): cardiotoxic; CI if EF<50% - Steroid AEs +++
26
Rx for relapse DLBCL
ASTC
27
How to monitor for relapse in DLBCL?
End of therapy PET
28
How does mantle cell lymphoma present?
Painless lymphadenopathy Extranodal occurs in 25% - GI and Waldeyer's ring, splenomegaly, >70% BM involvement Starts off indolent but has poor 5 year survival
29
Overexpression of cyclin D1 occurs in which type of NHL?
Mantle cell lymphoma
30
Rx for mantle cell lymphoma
Incurable Young: R-DHAP (rituximab + dexamethasone + high dose cytarabine + cisplatin) --> ASTC --> rituximab maintenance *Only lymphoma that has good evidence for early ASTC Old: rituximab + bendamustine
31
Rx for relapsed mantle cell lymphoma
Ibrutinib
32
What is Burkitt cell lymphoma associated with?
EBV +++ Epidemic in Africa Immunosuppression esp HIV
33
How does Burkitt cell lymphoma present?
Jaw mass in children | Abdo mass in adults
34
Rx for Burkitt cell lymphoma
Very chemosensitive | Curable
35
What's waldenstrom macroglobulinemia?
B cell/plasma cell lymphoma with monoclonal IgM production
36
What are the clinical features waldenstrom macroglobulinemia (lots of IgM)?
Generalised lymphadenopathy Fatigue ++++ due to significant anaemia IgM with hyperviscosity Bleeding (viscous serum results in defective platelet aggregation) Vision loss (retinal haemorrhage) Stroke (hyperviscosity)
37
How is Waldenstrom's macroglobulinemia managed?
Treatment often needed for years - very indolent progression 1st line: rituximab + bendamustine + dexamethasone + cyclophosphamide Elderly: dexamethasone + small dose cyclophosphamide + rituximab Ibrutinib has good PFS but not yet approved in ANZ (compassionate access) *similar to CLL*
38
How are acute complications of Waldenstrom's macroglobinemia managed?
Plasmapheresis - removes IgM from the serum
39
>10% of IgM MGUS progresses to Waldenstrom's macroglobinemia but not myeloma True or false
True
40
What's another name for Waldenstrom's macroglobinemia?
Lymphoplasmacytic lymphoma
41
What's the prognosis like for T cell lymphomas?
Aggressive | Overall worse prognosis than DLBCL
42
Standard rx for T cell lymphoma
CHOP | But poor response
43
How does T cell lymphomas present?
More cutaneous disease More Hepatosplenomegaly More eosinophilia Compared to B cell lymphoma
44
Adult T cell leukaemia/lymphoma (ATLL) | Which virus is it associated with?
Human T-cell leukemia virus (HTLV-1) | Seen in Japan and Caribbean
45
Adult T cell leukaemia/lymphoma (ATLL) | What are the clinical features?
Rash Generalised lymphadenopathy Hepatosplenomegaly Lytic bone lesions with hypercalcaemia (DDx is multiple myeloma but no rash)
46
Adult T cell leukaemia/lymphoma (ATLL) is the neoplastic proliferation of
Mature CD4+ T cells
47
Which organ does mycosis fungoides typically infiltrate?
Neoplastic proliferation of mature CD4+ T cells that infiltrate the skin - Skin rash, plaques, nodules When the cells spread to the blood = Sezary syndrome
48
Hodgkin lymphoma are characterised by ... cells
Reed-Sternberg cells - large B cells with multilobed nuclei and prominent nucleoi ('owl-eyed nuclei') RS cells secrete cytokines --> attract lymphocytes, plasma cells, macrophages, eosinophils --> produce mass --> fibrosis (inflammatory cells make up the bulk of the tumour)
49
Hodgkin lymphoma cells are positive for which markers?
Classically positive for CD15 and CD30 (lose usual B cell expression CD20) which are not usually expressed by normal B cells
50
What are the 4 subtypes of Hodgkin lymphoma?
Nodular sclerosis (most common) Lymphocyte rich Lymphocyte depleted Mixed cellularity
51
Which is the most common subtype of Hodgkin lymphoma?
Nodular sclerosis (70% of all cases)
52
How does nodular sclerosis HL present and in which age group?
Enlarging cervical (75%) or mediastinal (60%) lymph node in a young adult (age 20-30s), F>M But also 1/3 in elderly Bimodal distribution
53
Which subtype of HL has the best prognosis?
Lymphocyte rich
54
Which subtype of HL has the worst prognosis?
Lymphocyte depleted | Usually seen in elderly and HIV positive
55
How is Hodgkin lymphoma treated?
Very curable 2 different chemotherapy approaches 1) ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) 2) Escalated BEACOPP (escalated dose bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisolone) - Cure more patients - Causes infertility - Can't really use above age 40
56
Name one toxicity of bleomycin
Pulmonary toxicity
57
Name one toxicity of anthracycline (e.g. doxorubicin)
Cardiotoxicity e.g. cardiomyopathy, IHD, valvular disease
58
List some chemotherapy complications of Hodgkin lymphoma?
- Malignancy - MDS, NHL, solid tumours - Pulmonary toxicity (bleomycin) - Cardiotoxicity - premature IHD, valvular disease, cardiomyopathy (anthracycline e.g. doxorubicin) - Infertility - particularly BEACOPP
59
List some side effects of radiotherapy in Hodgkin lymphoma (diminishing role)
Cardiac Solid tumours - lung, breast, GIT (latent period 7-10 years) Infertility
60
What's an important tool in prognosticating and guiding treatment in Hodgkin lymphoma?
PET-CT
61
Rx for relapsed/refractory hodgkin lymphoma
Pembrolizumab (PD1 checkpoint inhibitor) Brentuximab (anti-CD30 conjugated with MMAE, a tubulin toxin, which gets delivered to the cell) Salvage chemotherapy then high dose chemotherapy with ASCT Poor 5 year survival <30%
62
What scan is used routinely to stage FDG avid lymphomas?
PET-CT
63
What's MALT lymphoma?
Marginal lymphoma in extranodal sites
64
Mantle cell lymphoma - what surface antigens?
Pan-B cell antigens CD19+, CD20+ CD5+ (T cell antigen) like CLL but its CD23- which separates it from CLL
65
Can you get IgM myeloma?
No!! | Always Waldenstrom's macroglobulinaemia
66
DLBCL cell surface markers?
Pan B cell markers: CD19+, CD20+, CD22+, CD79a+
67
How to diagnose DLBCL?
PET-CT | better than BM biopsy
68
How to monitor response to therapy/relapse in DLBCL?
PET-CT
69
Peripheral T cell lymphoma cell marker
CD3+
70
CD30+ T cell lymphoma. What's important?
Brentuximab anti-CD30 inhibitor
71
Tumour lysis syndrome Rx
Rasburicase | Oxidses uric acid to allantoions, inert molecules that are less toxic to kidneys
72
B-ALL affects who?
Children and young adults
73
Brentuximab AE
Neuropathy

Haematology (7 decks)

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