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Path - Haem > Lymphoma 1 > Flashcards

Lymphoma 1 Flashcards

1
Q

Where are Lymphomas usually found?

A

lymph nodes, bone marrow and/or blood (the lymphatic system)

lymphoid organs; spleen or the gut-associated lymphoid tissue (GALT) & MALT

Skin (often T cell disease - Cutaneous T cell lymphoma )

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2
Q

Non-Hodgkin’s Lymphomas __%

Hodgkin Lymphoma __%

A

Non-Hodgkin’s Lymphomas 85%

Hodgkin Lymphoma 15%

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3
Q

how is a diverse variety of different antibodies and T cell receptors made?

A

germ line genes are recombined known as VDJ recombination

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4
Q

where does VDJ recombination happen?

A

bone marrow

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5
Q

how is even more diversity generated in somatic hypermutation?

A

Recombination: Ig class switching at the germinal centre,

the insertion of nucleotide point mutations at the sites of VDJ recombination

this is second stage of DNA alteration

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6
Q

how is autoimmunity prevented in the generation of antibody diversity?

A

apoptosis

Great antibody specificity, eliminate self reactive clones

(90% of normal lymphocytes die in the Germinal centre!)

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7
Q

what increases the risk of DNA replication error in germinal centre?

A

rapid cell proliferation

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8
Q

what are the key enzymes regulation VDJ recombination?

A

RAG1+2

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9
Q

key enzymes regulation class switch recombination?

A

Adenosine induced Deaminase (AID)

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10
Q

which oncogenes can be translocated as an error, at/near the ig H promoter (chromosome 14)?

what does this result in?

A

bcl2, bcl6 (follicular)
Myc (burkitts)
cyclinD1 (mantle cell l)

oncognes have been translocated near a promoter sequence -> uncontrolled proliferation/cancer will occur!
Note that all these lymphomas involve chromosome 14 as the oncogenes arer moved near this promoter sequence!

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11
Q

oncogenes can be translocated as an error, at the ig H promoter. they come in which 2 forms?

A

proliferative or anti-apoptotic oncogenes

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12
Q

3 risk factors of LYMPHOMA?

A

○ Constant antigenic stimulation

	○ Infection (direct viral infection of lymphocytes)

○ Loss of T cell function

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13
Q

which 3 conditions cause lymphoma via

Constant antigenic stimulation?

A
  1. Helicobacter pylori: gastric MALT. MZL
  2. Sjogren syndrome: MZL NHL - parotid
  3. Hashimoto’s : MZl - thyroid
  4. Coeliac disease: small bowel T cell lymphoma, EATL (enteropathy-associated T cell non-Hodgkin lymphoma)

mzl - Marginal zone sub type (MZL)

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14
Q

which virus infects T cells by vertical transmission ?

consequence?

A

HTLV1
human T-lymphotropic virus,

-> adult T cell leukaemia lymphoma - ATLL

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15
Q

EBV infects __ lymphocytes?

A

B lymphocytes

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16
Q

why does HIV cause EBV-driven lymphomas?

A

EBV infects B lymphocytes

When the EBV is quiescent, the healthy carrier state is maintained by cytotoxic T cells killing B cells expressing EBV antigen.

HIV = loss of T cell function = B cell tumours.

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17
Q

HIV causes EBV-driven lymphomas. What else

A

transplant immunosuppression:

PTLD (post transplant lymphoproliferative disorder)

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18
Q

where in the lymphoid follicle are naïve unstimulated B cells located ?

A

mantle zone

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19
Q

○ CD20 = ___ lymphocytes

○ CD3, CD5 = ___ lymphocytes

A

○ CD20 = B cells

○ CD3, CD5 = T cells

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20
Q

what is the basic WHO Classification of Lymphoma?

A

○ Hodgkin Lymphoma
§ Classical one
§ B Lymphocyte predominant one

○ Non-Hodgkin Lymphoma
			§ B cell & TCell
				□ Precursor B cell neoplasms
				□ Peripheral B cell neoplasms 
					® Low grade 
					® High grade
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21
Q

which is the MOST COMMON lymphoma?

A

B Cell Non-Hodgkin Lymphoma

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22
Q

difference between Hodgkins and other lymphoma?

A

NHL : lymphoid cells circulate in the blood. involves multiple sites.

Hodgkin’s lymphoma tends to only affect one or two lymph node groups and spreads contiguously - local spread.

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23
Q

Over expression of cyclin D1 (from bcl-1) is suggestive of ?

A

Mantle cell lymphoma

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24
Q

how do you know there is normal clonal proliferation in a B cell?

A

will show roughly even amounts of kappa and lambda light chains)

60:40

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25
Q

Bcl 1 overexpression due to t(11;14)

q13;q32
translocation is consistent with which lymphoma?

A

Mantle cell lymphoma

26
Q

t(2;5) (p23;q35)

translocation is consistent with which lymphoma?

A

Anaplastic Large Cell Lymphoma ALCL

27
Q

Every lymphoma you see is LOW grade apart which 2?

A

• Burkitt’s lymphoma
○ high grade histology- Very aggressive

• Diffuse large B cell lymphoma & Mantle cell
○ high grade - aggressive

remember - diffuse means disseminated

28
Q

Histology showed;

Follicular pattern and
positive staining on flow cytometry for CD10 and bcl-6

which lymphoma is this?

A

Follicular Lymphoma

remeber this is B cell so even though CD10 is most common, it can have CD20, CD19, CD22 too as is common with B cells

29
Q

Histology showed;

CD5 and CD23 positive

and can undergo Richter transformation

which lymphoma is this?

A

Chronic Lymphocytic Leukaemia - CLL

30
Q

histology showed:

○ Arise from pre-germinal centre cells
expression of CD5 and cyclin D1 (from bcl-1)

also 11;14 translocation

which lymphoma is this?

A

Mantle Cell Lymphoma

31
Q

Which lymphoma involves 14;18 translocation involving B-cell lymphoma 2 (BCL-2) gene

A

Follicular Lymphoma

follicular - 4licular
Half of four is 2 -> BCL2 GENE

32
Q

another name for Chronic Lymphocytic Leukaemia? CLL

A

Small Lymphocytic Lymphoma - SLL

33
Q

Follicular lymphoma presents as? (origin?)

A

Lymph node enlargement

LNs

34
Q

which lymphoma arises from naïve B cells or post-germinal centre memory B cells ?

A

Chronic Lymphocytic Leukaemia?

35
Q

which lymphoma arises from extranodal sites (e.g. gut, lung, spleen) ?

and from post-germinal centre memory B cells?

A

Marginal Zone Lymphoma/MALT Lymphoma

36
Q

how to treat Marginal Zone Lymphoma/MALT Lymphoma caused by H.pylori

A

can be treated by getting rid of h. pylori/ cause

37
Q

which lymphoma presents as Jaw or abdominal mass in children/young adults

A

Burkitt’s Lymphoma

38
Q

which lymphoma arises from germinal centre cells AND

has a Starry-sky appearance on histology

what is the starry sky?

A

Burkitt’s Lymphoma

note the starry sky appearance can be present in other highly proliferative lymphomas

starry - reactive histiocytes (macrophages)
sky - background of malignant B cells

39
Q

which tumour has c-Myc translocation (8;14)

(2;8 or 8;22) ?

A

Burkitt’s Lymphoma

40
Q

A Diffuse large B cell lymphoma with a germinal centre phenotype is associated with a __ prognosis

A

GOOD

41
Q

A Diffuse large B cell lymphoma with p53 positive and high proliferation fraction is associated with a__ prognosis

A

POOR

42
Q

which lymphoma is Often found with an associated reactive cell population (especially eosinophils) and are

AGGRESSIVE

A

T Cell Lymphomas

43
Q

Cutaneous T cell lymphoma is caused by?

A

mycosis fungoides

44
Q

Anaplastic Large Cell Lymphoma - ALCL

occurs in which age group?

A

Kids and Young people

45
Q 
histology showed:
		○ T cell or anaplastic cells
also has:
	○ 2;5 translocation 
		○ Alk-1 protein expression

what condition?

A

Anaplastic Large Cell Lymphoma - ALCL

○ Alk-1 protein expression is associated with a better prognosis

46
Q

difference between classical and Nodular lymphocyte predominant H lymphoma?

A 
Hodkin L /classical:
young people
group of nodes
EBV
Reed-Sternberg
sclerosis
stain cells with CD30 CD15
NLP - 5%:
CD20
1 lymph node
only lymphocytes
ELDERLY
and more
47
Q

Alcohol-induced pain is a classic but rare symptom in?

A

Hodgkin Lymphoma

48
Q

patient comes with painless enlarging lymph nodes

fever, night sweats, weight loss and pruritus.

name the symptoms.
what’s does he have?

A

B symptoms: Fever, Night sweats, Weight loss >10% in 6 months + unexplained

Hodgkin or NH Lymphoma

49
Q

How to treat Hodgkin Lymphoma?

A

Chemotherapy

ABVD
		○ Adriamycin - Doxorubicin
		○ Bleomycin 
		○ Vincristine 
		○ DTIC (Dacarbazine)

ABVD, is given at 4-weekly intervals.
all patients should get this. +/ radiotherapy (increases risk of 2nd cancer elsewhere eg breast if chemo +radio)

Intensive chemo + Autologous Stem cell transplant - relapsed patients

2nd relapse: anti CD30-MAME + PD1 checkpoint inhibitor

50
Q

what are the features of burkitts lymphoma?

A

Histologically, Burkitt lymphoma (BL) is characterized by a monoclonal proliferation of medium-sized, noncleaved B-cells that are uniform in appearance and that produce a diffuse pattern of tissue involvement

51
Q

presence of itching is associated with which lymphoma?

what is the typical distribution?

A

Hodgkins - 1/3 (more common)

Non Hodgkins - 1/10

distribution: around affected LNs or skin or whole body

52
Q

why are some people with lymphoma more susceptible to infection?

A

somee lymphoma can lead to:

Hypergammaglobulinaemia - high IgG ->hyper viscosity syndrome, autoimmune cytopaenia eg haemolytic anaemia

Agammaglobulinaemia - no IgG -> increased infections

53
Q

what are reed sternberg cells?

A

giant B cells from germinal centre
– bi-nucleate/multinucleate (‘owl eyed’) cell

on a background of lymphocytes & reactive cells

54
Q

what is the most common subtype of HL?

A

nodular sclerosing 80% - young women > men

55
Q

what is the nodal involvement in HL?

A

Nodes tend to be mediastinal / cervical but

not always

56
Q

How is lymphoma staged?

A

Staging (Ann-Arbor)

Stage 1 – one LN region (LN region can include spleen)
Stage 2 – two or more LN regions on the same side of the diaphragm
Stage 3 – two or more LN regions on opposite sides of the diaphragm
Stage 4 – extranodal sites (liver, BM) spleen not included here

A: No constitutional symptoms B: Constitutional symptoms (1 or more)

E.g. Stage 2a – patient with involvement in 3 LN regions above the diaphragm, pain after alcohol
and SVC syndrome but no weight loss, night sweats etc.

57
Q

what are the risks of mediastinal lymph node swelling?

A

SVC syndrome :

  • face and neck swelling (worse in morning)
  • headache, lightheaded
  • upper limb edema
  • distended neck veins, cough, SOB

Airway obstruction

58
Q

name the mature B cell NHL?

A

DLBCL, Follicular NHL, CLL (Chronic Lymphocytic Leukaemia)

59
Q

name the mature T cell NHL?

A

Anaplastic, Cutaneous

PTCL

60
Q

on biopsy of the lymph node what would you find for a diagnosis of mantle cell lymphoma?

A

malignant cells in the mantle of the follicle

61
Q

what is burkitts leukaemia?

A

when there is bone marrow involvement leading to a ‘leukaemic phase’ of BL

Path - Haem (13 decks)

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