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Haematology > Lymphoma > Flashcards

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
Q

What are side effects of CHOP (cyclophosphamide + vincristine + doxorubicin + pred)?

A

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 +++
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26
Q

Rx for relapse DLBCL

A

ASTC

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27
Q

How to monitor for relapse in DLBCL?

A

End of therapy PET

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28
Q

How does mantle cell lymphoma present?

A

Painless lymphadenopathy
Extranodal occurs in 25% - GI and Waldeyer’s ring, splenomegaly, >70% BM involvement
Starts off indolent but has poor 5 year survival

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29
Q

Overexpression of cyclin D1 occurs in which type of NHL?

A

Mantle cell lymphoma

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30
Q

Rx for mantle cell lymphoma

A

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
Q

Rx for relapsed mantle cell lymphoma

A

Ibrutinib

32
Q

What is Burkitt cell lymphoma associated with?

A

EBV +++
Epidemic in Africa
Immunosuppression esp HIV

33
Q

How does Burkitt cell lymphoma present?

A

Jaw mass in children

Abdo mass in adults

34
Q

Rx for Burkitt cell lymphoma

A

Very chemosensitive

Curable

35
Q

What’s waldenstrom macroglobulinemia?

A

B cell/plasma cell lymphoma with monoclonal IgM production

36
Q

What are the clinical features waldenstrom macroglobulinemia (lots of IgM)?

A

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
Q

How is Waldenstrom’s macroglobulinemia managed?

A

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
Q

How are acute complications of Waldenstrom’s macroglobinemia managed?

A

Plasmapheresis - removes IgM from the serum

39
Q

> 10% of IgM MGUS progresses to Waldenstrom’s macroglobinemia but not myeloma
True or false

A

True

40
Q

What’s another name for Waldenstrom’s macroglobinemia?

A

Lymphoplasmacytic lymphoma

41
Q

What’s the prognosis like for T cell lymphomas?

A

Aggressive

Overall worse prognosis than DLBCL

42
Q

Standard rx for T cell lymphoma

A

CHOP

But poor response

43
Q

How does T cell lymphomas present?

A

More cutaneous disease
More Hepatosplenomegaly
More eosinophilia

Compared to B cell lymphoma

44
Q

Adult T cell leukaemia/lymphoma (ATLL)

Which virus is it associated with?

A

Human T-cell leukemia virus (HTLV-1)

Seen in Japan and Caribbean

45
Q

Adult T cell leukaemia/lymphoma (ATLL)

What are the clinical features?

A

Rash
Generalised lymphadenopathy
Hepatosplenomegaly
Lytic bone lesions with hypercalcaemia (DDx is multiple myeloma but no rash)

46
Q

Adult T cell leukaemia/lymphoma (ATLL) is the neoplastic proliferation of

A

Mature CD4+ T cells

47
Q

Which organ does mycosis fungoides typically infiltrate?

A

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
Q

Hodgkin lymphoma are characterised by … cells

A

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
Q

Hodgkin lymphoma cells are positive for which markers?

A

Classically positive for CD15 and CD30 (lose usual B cell expression CD20) which are not usually expressed by normal B cells

50
Q

What are the 4 subtypes of Hodgkin lymphoma?

A

Nodular sclerosis (most common)
Lymphocyte rich
Lymphocyte depleted
Mixed cellularity

51
Q

Which is the most common subtype of Hodgkin lymphoma?

A

Nodular sclerosis (70% of all cases)

52
Q

How does nodular sclerosis HL present and in which age group?

A

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
Q

Which subtype of HL has the best prognosis?

A

Lymphocyte rich

54
Q

Which subtype of HL has the worst prognosis?

A

Lymphocyte depleted

Usually seen in elderly and HIV positive

55
Q

How is Hodgkin lymphoma treated?

A

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
Q

Name one toxicity of bleomycin

A

Pulmonary toxicity

57
Q

Name one toxicity of anthracycline (e.g. doxorubicin)

A

Cardiotoxicity e.g. cardiomyopathy, IHD, valvular disease

58
Q

List some chemotherapy complications of Hodgkin lymphoma?

A
  • Malignancy - MDS, NHL, solid tumours
  • Pulmonary toxicity (bleomycin)
  • Cardiotoxicity - premature IHD, valvular disease, cardiomyopathy (anthracycline e.g. doxorubicin)
  • Infertility - particularly BEACOPP
59
Q

List some side effects of radiotherapy in Hodgkin lymphoma (diminishing role)

A

Cardiac
Solid tumours - lung, breast, GIT (latent period 7-10 years)
Infertility

60
Q

What’s an important tool in prognosticating and guiding treatment in Hodgkin lymphoma?

A

PET-CT

61
Q

Rx for relapsed/refractory hodgkin lymphoma

A

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
Q

What scan is used routinely to stage FDG avid lymphomas?

A

PET-CT

63
Q

What’s MALT lymphoma?

A

Marginal lymphoma in extranodal sites

64
Q

Mantle cell lymphoma - what surface antigens?

A

Pan-B cell antigens CD19+, CD20+

CD5+ (T cell antigen) like CLL but its CD23- which separates it from CLL

65
Q

Can you get IgM myeloma?

A

No!!

Always Waldenstrom’s macroglobulinaemia

66
Q

DLBCL cell surface markers?

A

Pan B cell markers: CD19+, CD20+, CD22+, CD79a+

67
Q

How to diagnose DLBCL?

A

PET-CT

better than BM biopsy

68
Q

How to monitor response to therapy/relapse in DLBCL?

A

PET-CT

69
Q

Peripheral T cell lymphoma cell marker

A

CD3+

70
Q

CD30+ T cell lymphoma. What’s important?

A

Brentuximab anti-CD30 inhibitor

71
Q

Tumour lysis syndrome Rx

A

Rasburicase

Oxidses uric acid to allantoions, inert molecules that are less toxic to kidneys

72
Q

B-ALL affects who?

A

Children and young adults

73
Q

Brentuximab AE

A

Neuropathy

Haematology (7 decks)

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