Lymphoma 1 Flashcards by RK Splendid

Where are Lymphomas usually found?

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 )

How well did you know this?

Not at all

Perfectly

Non-Hodgkin’s Lymphomas __%

Hodgkin Lymphoma __%

Non-Hodgkin’s Lymphomas 85%

Hodgkin Lymphoma 15%

How well did you know this?

Not at all

Perfectly

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

germ line genes are recombined known as VDJ recombination

How well did you know this?

Not at all

Perfectly

where does VDJ recombination happen?

bone marrow

How well did you know this?

Not at all

Perfectly

how is even more diversity generated in somatic hypermutation?

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

How well did you know this?

Not at all

Perfectly

how is autoimmunity prevented in the generation of antibody diversity?

apoptosis

Great antibody specificity, eliminate self reactive clones

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

How well did you know this?

Not at all

Perfectly

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

rapid cell proliferation

How well did you know this?

Not at all

Perfectly

what are the key enzymes regulation VDJ recombination?

RAG1+2

How well did you know this?

Not at all

Perfectly

key enzymes regulation class switch recombination?

Adenosine induced Deaminase (AID)

How well did you know this?

Not at all

Perfectly

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

what does this result in?

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!

How well did you know this?

Not at all

Perfectly

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

proliferative or anti-apoptotic oncogenes

How well did you know this?

Not at all

Perfectly

3 risk factors of LYMPHOMA?

○ Constant antigenic stimulation

	○ Infection (direct viral infection of lymphocytes)

○ Loss of T cell function

How well did you know this?

Not at all

Perfectly

which 3 conditions cause lymphoma via

Constant antigenic stimulation?

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

mzl - Marginal zone sub type (MZL)

How well did you know this?

Not at all

Perfectly

which virus infects T cells by vertical transmission ?

consequence?

HTLV1
human T-lymphotropic virus,

-> adult T cell leukaemia lymphoma - ATLL

How well did you know this?

Not at all

Perfectly

EBV infects __ lymphocytes?

B lymphocytes

How well did you know this?

Not at all

Perfectly

why does HIV cause EBV-driven lymphomas?

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.

How well did you know this?

Not at all

Perfectly

HIV causes EBV-driven lymphomas. What else

transplant immunosuppression:

PTLD (post transplant lymphoproliferative disorder)

How well did you know this?

Not at all

Perfectly

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

mantle zone

How well did you know this?

Not at all

Perfectly

○ CD20 = ___ lymphocytes

○ CD3, CD5 = ___ lymphocytes

○ CD20 = B cells

○ CD3, CD5 = T cells

How well did you know this?

Not at all

Perfectly

what is the basic WHO Classification of Lymphoma?

○ 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

How well did you know this?

Not at all

Perfectly

which is the MOST COMMON lymphoma?

B Cell Non-Hodgkin Lymphoma

How well did you know this?

Not at all

Perfectly

difference between Hodgkins and other lymphoma?

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.

How well did you know this?

Not at all

Perfectly

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

Mantle cell lymphoma

How well did you know this?

Not at all

Perfectly

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

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

60:40

How well did you know this?

Not at all

Perfectly

Bcl 1 overexpression due to t(11;14) | q13;q32 translocation is consistent with which lymphoma?

Mantle cell lymphoma

t(2;5) (p23;q35) translocation is consistent with which lymphoma?

Anaplastic Large Cell Lymphoma ALCL

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

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

Histology showed; CD5 and CD23 positive and can undergo Richter transformation which lymphoma is this?

Chronic Lymphocytic Leukaemia - CLL

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

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

another name for Chronic Lymphocytic Leukaemia? CLL

Small Lymphocytic Lymphoma - SLL

Follicular lymphoma presents as? (origin?)

Lymph node enlargement LNs

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

Chronic Lymphocytic Leukaemia?

which lymphoma arises from extranodal sites (e.g. gut, lung, spleen) ? and from post-germinal centre memory B cells?

Marginal Zone Lymphoma/MALT Lymphoma

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

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

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

Burkitt's Lymphoma

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

which tumour has c-Myc translocation (8;14) | (2;8 or 8;22) ?

Burkitt's Lymphoma

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

GOOD

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

POOR

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

T Cell Lymphomas

Cutaneous T cell lymphoma is caused by?

mycosis fungoides

Anaplastic Large Cell Lymphoma - ALCL | occurs in which age group?

Kids and Young people

``` 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

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 ```

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

Hodgkin Lymphoma

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

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

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

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

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

what are reed sternberg cells?

giant B cells from germinal centre – bi-nucleate/multinucleate (‘owl eyed’) cell on a background of lymphocytes & reactive cells

what is the most common subtype of HL?

nodular sclerosing 80% - young women > men

what is the nodal involvement in HL?

Nodes tend to be mediastinal / cervical but | not always

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.

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

name the mature B cell NHL?

DLBCL, Follicular NHL, CLL (Chronic Lymphocytic Leukaemia)

name the mature T cell NHL?

Anaplastic, Cutaneous | PTCL

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

what is burkitts leukaemia?

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