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YR5 Haematology > Lymphoma > Flashcards

Lymphoma Flashcards

1
Q

What is lymphoma?

A

Neoplastic proliferation of lymphoid cells forming discrete tissue masses.

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2
Q

Where are lymphomas usually found?

A

LN +/- blood (Lymphatic system)
Lymphoid organs: spleen or gut associated lymphoid tissue
Skin (Often T cell disease)

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3
Q

Where can lymphomas rarely occur? (5)

A

CNS
Eyes
Testes
Breast
Kidney

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4
Q

What are the 2 classes of lymphoma and their prevalence?

A

Non-Hodgkin’s 80%
Hodgkin’s 20%

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5
Q

Describe the aetiology of lymphoma

A

Mostly sporadic with no known RFs
Some a/w specific infections/ immune disorders

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6
Q

How does immunoglobulin configuration occur? What are the benefits and risks?

A

Ig + TCR genes cut + recombined, then subjected to deliberate DNA mutagenesis

Generates diversity + Ig class switching

Potential for recombination errors + harmful point mutations

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7
Q

What are the benefits and risks of rapid cell proliferation in the germinal centre?

A

Allows rapid response to infection

Rapid multiple cell divisions = increase chance of DNA replication errors

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8
Q

What are the benefits and risks of immune response dependancy on apoptosis?

A

Eliminates self-reactive clones or ineffective Ig clones

Apoptosis is “switched off” in germinal centre
Consequences of mutations in apoptosis regulating genes

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9
Q

What are the 3 mechanisms of risk factors for certain non Hodgkin’s lymphoma subtypes?

A
  1. Constant antigenic stimulation
  2. Viral infection (direct viral integration of lymphocytes)
  3. EBV infection in a scenario of loss of T cell function
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10
Q

Give 3 examples of chronic/ AI antigenic stimulation causing B cell Non Hodgkin Lymphoma marginal zone subtype?

A

H. Pylori: Gastric MALT- mucosa associated lymphoid tissue. MZL of stomach

Sjogren syndrome: MZL of parotid gland

Hashimoto’s Thyroiditis: MZL of thyroid

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11
Q

What chronic antigenic stimulation causes enteropathy associated T-cell non Hodgkin’s lymphoma (EATL)?

A

Poorly controlled coeliac disease:
Small intestine EATL

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12
Q

How does H. pylori cause gastric MALT?

A

Chronic bacterial infection stimulates + irritates gut mucosa
Leads to lymphocytic infiltration
In chronic polyclonal reactive response, may lead to clones being produced
Increasingly malignant
Leads to MZL

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13
Q

Give an example of a direct viral integration causing non Hodgkin lymphoma.
In which populations is this more common?

A

HTLV1 retrovirus infects T cells by vertical transmission
Caribbean, Japanese endemic
Risk of adult T cell leukaemia lymphoma is 2.5% at 70y (ATLL)

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14
Q

How does EBV and loss of T cell function result in B non-hodgkins lymphoma?

A
  1. EBV infects B lymphocytes. Healthy carrier state post glandular fever. EBV driven proliferation a/w surface EBV antigens- targeted by EBV specific cytotoxic T cell response
  2. Loss of T cell function e.g. HIV, transplant immunosuppression
  3. Failure to eliminate EBV driven proliferation of B cells due to absence of cytotoxic T cells
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15
Q

What are almost all lymphomas due to?

A

immunoglobulin mutations (often heavy chain rearrangement)
BCL2
BCL6
Myc
Cyclin D1

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16
Q

Give 3 features of lymphoma presentation

A

Painless progressive lymphadenopathy
Recurrent infections
Constitutional Sx

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17
Q

How can lymphomas cause presentation with other system involvement?

A

Extrinsic compression of any “tube”: ureter, bile duct, large blood vessel, bowel, trachea, oesophagus

Infiltrate/ impair any organ system

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18
Q

Give examples of symptoms caused by lymphoma infiltration in the brain, eyes and skin

A

Brain: Stroke, memory problems
Eyes: blindness in 1 eye
Skin: Rash, inflammation, plaques, nodules

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19
Q

In which type of Lymphoma are Reed Sternberg cells seen?

A

Hodgkin’s Lymphoma

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20
Q

What are the subtypes of B cell non-hodgkins lymphoma?

A

Precursor: B lymphoblastic leukaemia or lymphoma
Mature: B cell neoplasm, DLBCL, Follicular NHL, CLL

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21
Q

What are the subtypes of T or NK cell lymphoma?

A

Precursor: T lymphoblastic leukaemia or lymphoma
Mature: T + NK neoplasm, PTCL, Anaplastic, Cutaneous

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22
Q

Give 3 epidemiological facts about Hodgkin’s lymphoma

A

1% of all cancer
M > F
Bimodal:
Most common 20-29
2nd smaller peak >60s

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23
Q

List the 3 classic B symptoms seen in Hodgkins lymphoma and 2 other less common symptoms

A

B: Fever, Night sweats, Weight loss
Pruritis
Alcohol induced pain in nodes

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24
Q

Which subtype of Hodgkins lymphoma has a peak of incidence in young women more than men?

A

Nodular sclerosing

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25
Q

What are the 4 types of classic Hodgkin’s lymphoma? What is the prevalence of each? What is the prognosis in each?

A

Nodular sclerosing 80%: good
Mixed cellularity 17%: good
Lymphocyte rich (rare): good
Lymphocyte depleted (rare): poor

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26
Q

What investigations are used to stage Hodgkin’s lymphoma?

A

FDG-PET/ CT scan
Biopsy of other sites if infiltrated e.g. liver

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27
Q

How do non Hodgkin’s and Hodgkins lymphoma differ in the way they spread?

A

H: Spreads through lymphatic system
NHL: Spreads through circulation

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28
Q

What is the staging system and what are the stages of Hodgkin’s lymphoma?

A

Ann-Arbor
I: 1 group of nodes (can inc. spleen)
II: >1 group of nodes SAME side of diaphragm
III: nodes above + below diaphragm
IV: extra-nodal spread

Suffix A if none of below, B if any of below:
Fever, unexplained weight loss >10% in 6m, night sweats

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29
Q

How may nodular sclerosing Hodgkin’s lymphoma present?

A

Neck nodes + mediastinal mass (may be massive + compress SVC or trachea)

30
Q

What is the chemotherapy regime for Hodgkins lymphoma?

A

ABVD
Adriamycin
Bleomycin
Vinblastine
DTIC

31
Q

Give 2 benefits of ABVD

A

Effective- curative
Preserves fertility (unlike original MOPP regime)

32
Q

Give 2 potential complications of ABVD in the long term

A

Pulmonary fibrosis
Cardiomyopathy

33
Q

Describe the chemotherapy regime for Hodgkins lymphoma

A

4w intervals, 2-6 cycles
PET CT after 2 cycles, response assessment + at end of tx
+/- radiotherapy

34
Q

Describe salvage chemotherapy in cases of relapsed Hodgkin’s lymphoma

A

High dose chemo
Autologous PB stem cell transplant

35
Q

Why is radiotherapy not used in all Hodgkin’s lymphoma patients?

A

ABVD alone can be curative
Radiotherapy increases risk of secondary cancer, so in young try to avoid
(secondary: Breast, Leukaemia, Lung, Skin)

36
Q

What is the prognosis in Hodgkins lymphoma?

A

80% cured
10% die from relapse of HL in 10y
10% die from tx related complications after 10y

37
Q

Which patients generally do less well despite treatment in Hodgkin’s lymphoma?

A

Elderly
Lymphocyte depleted

38
Q

What investigations are used for staging non Hodgkins lymphoma?

A

CT
PET scan (in aggressive lymphomas)
+/- BM biopsy
LP (if CNS involvement)

39
Q

List 4 prognostic markers/ important tests to perform in non Hodgkins lymphoma

A

LDH (rapid cell turnover)
Performance status
HIV serology
Hep B serology (risk of reactivation if B cell depleting therapy given)

40
Q

Which subtypes of non Hodgkin lymphoma are considered very aggressive? What is the median survival without tx and response to chemo?

A

Burkitt’s lymphoma
T or B cell lymphoblastic leukaemia/ lymphoma

2-5w survival if no Tx

Curable

41
Q

Which subtypes of non Hodgkin lymphoma are considered aggressive? What is the median survival without tx and response to chemo?

A

Diffuse Large B cell 30-40% of NHL
Mantle cell

3-12m survival without Tx

Moderately curable

42
Q

Which subtypes of non Hodgkin lymphoma are considered indolent? What is the median survival without tx and response to chemo?

A

Follicular
Small lymphocytic/ CLL
Mucosa associated (MALT)

10-15y survival without Tx

Incurable (long remission)

43
Q

How are very aggressive non Hodgkins lymphoma treated?

A

Treated like acute leukaemias

44
Q

What is prognosis and treatment determined by in diffuse large B cell NHL?

A

IPI (International Prognostic Index):
Age
Stage (Ann Arbor)
LDH
Extra-nodal disease sites
ECOG performance status

45
Q

What is the chemotherapy regime for diffuse large B cell NHL?

A

6-8 cycles of R-CHOP
Rituximab: anti-CD20 monoclonal antibody
Cyclophosphamide
Adriamycin
Vincristine
Prednisolone

46
Q

Give 4 features of the course of follicular NHL

A

Indolent
35% of NHL
Incurable, median survival 12-15y
May require 2-3 different chemotherapy schedules over the 12-15y

47
Q

What translocation is follicular NHL associated with?

A

t(14:18) which results in over expression of bcl2 an anti-apoptosis protein

48
Q

What is the initial treatment for follicular NHL?

A

Watch + wait
Treat only if clinically indicated e.g. nodal extrinsic compression, massive painful nodes or recurrent infections

49
Q

What combination immunotherapy regime can be used in follicular NHL?

A

R-COP
or
R-CHOP
Tx not curative

50
Q

Give 5 features of extra nodal marginal zone lymphomas

A

Involve extra-nodal lymphoid tissue e.g. gastric mucosa, parotids
8% of NHL
Chronic antigen stimulation
Median age 55-60y
H. pylori eradication may cure 75%

51
Q

What is the most common presentation of marginal zone lymphoma?

A

Epigastric pain
Ulceration
Bleeding into the gut
Usually present at Stage I
B Sx uncommon

52
Q

What is enteropathy associated T cell lymphoma?

A

T cell NHL in patients with coeliac due to chronic antigen stimulation (gluten)
Mature T cells
Involves jejunum + ileum
Aggressive

53
Q

Give 6 ways in which enteropathy associated T cell lymphoma may present

A

Abdo pain
Obstruction
Perforation
GI bleeding
Malabsorption
Systemic Sx

54
Q

What is the prognosis in enteropathy associated T cell lymphoma?

A

Generally fatal
Responds poorly to chemo
Aim to prevent with strict GF diet

55
Q

What is chronic lymphocytic leukaemia? Give 3 epidemiological facts

A

Proliferation of mature B lymphocytes, predominantly involves BM + blood
Commonest leukaemia in western world
RF: Caucasian, FH
Median age 72 (10% <55)

56
Q

Give 5 laboratory findings in CLL

A

Lymphocytosis 5-300 x10^9
Normocytic normochromic anaemia
Thrombocytopenia
BM lymphocytic replacement of normal marrow elements
Smear cells

57
Q

What technique is used to diagnose CLL?

A

Immunophenotype by flow cytometry of peripheral blood
CD5 +ve B cells in peripheral blood (should only be transiently expressed in immature)

58
Q

What is the common clinical course of CLL?

A

5-10y good health until progression to 2-3y rapid phase to death

59
Q

Do all CLL patients progress?

A

1/3 never progress
1/3 progress, respond to tx, die from unrelated disorder
1/3 progress, require multiple lines of Rx, refractory disease, die from CLL

60
Q

Give 3 cell based prognostic factors in CLL

A

IgHV mutation status
CLL FISH cytogenetic panel
TP53 mutation status (Chr17p deletion +/- TP53 point mutation)

61
Q

Describe the normal development of B cells

A

Start with germline immunoglobulin gene configuration
Pass into germinal center
Undergo recombination, generating antibody diversity

62
Q

How does immunoglobulin heavy chain status affect prognosis in CLL?

A

If IGHV are mutated (post GC) = better prognosis as cells have undergone somatic hypermutation, thus differ from germline

63
Q

What staging systems are used for CLL?

A

Binet
or
Rai

64
Q

What are the clinical issues in CLL? What are these caused by?

A

Increased infection risk (population of malignant non functional mature B cells + hypogammaglobinaemia)
BM failure (proliferation in BM)
Lymphadenopathy +/- splenomegaly, lymphocytosis (circulates to nodes, spleen, blood)
AI complications e.g. AIHA (disease of immune cells)

65
Q

How can acquiring further mutations complicate CLL?

A

Transform to high grade lymphoma (Richter transformation)

66
Q

What supportive care is given to prevent Sino-pulmonary infections in CLL?

A

Early tx with abx
Pneumocystis prophylaxis +/- zoster prophylaxis
Recurrent infection + IgG< 5g/l give IVIG replacement therapy

67
Q

What vaccines are given to CLL patients?

A

Pneumococcal
SARS COV 2
Seasonal flu
(AVOID live vaccines)

68
Q

In which 5 circumstances do you not watch and wait in CLL?

A

Progressive lymphocytosis
Progressive marrow failure
Massive/ progressive lymphadenopathy/ splenomegaly
Systemic Sx (B Sx)
AI cytopenias (treat with immunosuppression not chemo)

69
Q

Give 3 options for therapy in CLL

A

Combination immune-chemotherapy
Targeted: BTK inhibitor or BCL2 inhibitor
Cellular: Allogenic SCT, CAR-T therapy

70
Q

Give an example of a BCR kinase and BCL2 inhibitor

A

BCR: Ibrutinib PO
BCL2: Venetoclax PO

71
Q

What do the targeted therapies for CLL affect?

A

Constant signalling through B cell receptor to promote survival (we maintain memory B cells for protective immunity)
BCR signalling requires BCR + downstream signalling proteins e.g. BTK
Prevent cells dying by increasing presence of anti-apoptotic proteins e.g. BCL2

72
Q

What is the main risk of Venetoclax?

A

Tumour lysis syndrome

YR5 Haematology (20 decks)

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