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Lymphoma 1 Flashcards

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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
Bcl 1 overexpression due to t(11;14) | q13;q32 translocation is consistent with which lymphoma?
Mantle cell lymphoma
26
t(2;5) (p23;q35) translocation is consistent with which lymphoma?
Anaplastic Large Cell Lymphoma ALCL
27
Every lymphoma you see is LOW grade apart which 2?
• Burkitt's lymphoma ○ high grade histology- Very aggressive • Diffuse large B cell lymphoma & Mantle cell ○ high grade - aggressive remember - diffuse means disseminated
28
Histology showed; Follicular pattern and positive staining on flow cytometry for CD10 and bcl-6 which lymphoma is this?
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
Histology showed; CD5 and CD23 positive and can undergo Richter transformation which lymphoma is this?
Chronic Lymphocytic Leukaemia - CLL
30
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?
Mantle Cell Lymphoma
31
Which lymphoma involves 14;18 translocation involving B-cell lymphoma 2 (BCL-2) gene
Follicular Lymphoma follicular - 4licular Half of four is 2 -> BCL2 GENE
32
another name for Chronic Lymphocytic Leukaemia? CLL
Small Lymphocytic Lymphoma - SLL
33
Follicular lymphoma presents as? (origin?)
Lymph node enlargement LNs
34
which lymphoma arises from naïve B cells or post-germinal centre memory B cells ?
Chronic Lymphocytic Leukaemia?
35
which lymphoma arises from extranodal sites (e.g. gut, lung, spleen) ? and from post-germinal centre memory B cells?
Marginal Zone Lymphoma/MALT Lymphoma
36
how to treat Marginal Zone Lymphoma/MALT Lymphoma caused by H.pylori
can be treated by getting rid of h. pylori/ cause
37
which lymphoma presents as Jaw or abdominal mass in children/young adults
Burkitt's Lymphoma
38
which lymphoma arises from germinal centre cells AND has a Starry-sky appearance on histology what is the starry sky?
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
which tumour has c-Myc translocation (8;14) | (2;8 or 8;22) ?
Burkitt's Lymphoma
40
A Diffuse large B cell lymphoma with a germinal centre phenotype is associated with a __ prognosis
GOOD
41
A Diffuse large B cell lymphoma with p53 positive and high proliferation fraction is associated with a__ prognosis
POOR
42
which lymphoma is Often found with an associated reactive cell population (especially eosinophils) and are AGGRESSIVE
T Cell Lymphomas
43
Cutaneous T cell lymphoma is caused by?
mycosis fungoides
44
Anaplastic Large Cell Lymphoma - ALCL | occurs in which age group?
Kids and Young people
45
``` histology showed: ○ T cell or anaplastic cells also has: ○ 2;5 translocation ○ Alk-1 protein expression ``` what condition?
Anaplastic Large Cell Lymphoma - ALCL ○ Alk-1 protein expression is associated with a better prognosis
46
difference between classical and Nodular lymphocyte predominant H lymphoma?
``` 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
Alcohol-induced pain is a classic but rare symptom in?
Hodgkin Lymphoma
48
patient comes with painless enlarging lymph nodes fever, night sweats, weight loss and pruritus. name the symptoms. what's does he have?
B symptoms: Fever, Night sweats, Weight loss >10% in 6 months + unexplained Hodgkin or NH Lymphoma
49
How to treat Hodgkin Lymphoma?
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
what are the features of burkitts lymphoma?
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
presence of itching is associated with which lymphoma? what is the typical distribution?
Hodgkins - 1/3 (more common) Non Hodgkins - 1/10 distribution: around affected LNs or skin or whole body
52
why are some people with lymphoma more susceptible to infection?
somee lymphoma can lead to: Hypergammaglobulinaemia - high IgG ->hyper viscosity syndrome, autoimmune cytopaenia eg haemolytic anaemia Agammaglobulinaemia - no IgG -> increased infections
53
what are reed sternberg cells?
giant B cells from germinal centre – bi-nucleate/multinucleate (‘owl eyed’) cell on a background of lymphocytes & reactive cells
54
what is the most common subtype of HL?
nodular sclerosing 80% - young women > men
55
what is the nodal involvement in HL?
Nodes tend to be mediastinal / cervical but | not always
56
How is lymphoma staged?
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
what are the risks of mediastinal lymph node swelling?
SVC syndrome : - face and neck swelling (worse in morning) - headache, lightheaded - upper limb edema - distended neck veins, cough, SOB Airway obstruction
58
name the mature B cell NHL?
DLBCL, Follicular NHL, CLL (Chronic Lymphocytic Leukaemia)
59
name the mature T cell NHL?
Anaplastic, Cutaneous | PTCL
60
on biopsy of the lymph node what would you find for a diagnosis of mantle cell lymphoma?
malignant cells in the mantle of the follicle
61
what is burkitts leukaemia?
when there is bone marrow involvement leading to a 'leukaemic phase' of BL

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