Lymphoma 1 Flashcards

(83 cards)

1
Q

What is the definition of lymphoma?

A

A malignant neoplasm of lymphoid cells

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2
Q

Where can lymphoma occur?

A

Lymphatic system: Lymph nodes, bone marrow, blood
Lymphoid organs: spleen, gut-associated lymphoid tissue
Skin
Anywhere else (CNS, ocular, testes, breast)

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3
Q

What is incidence of Hodgkins vs Non Hodgkins?

A
Hodgkins = 80%
NHL = 20%
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4
Q

How are lymphomas classified?

A

Based on CELL OF ORIGIN

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5
Q

How does lymphoma generally develop?

A
  • Rapid proliferation/division of cells in the germinal centre - means that there is a higher risk of replication errors
  • highly dependent on apoptosis (most lymphocytes die in the GC) - means that apoptosis can be switched off, causing damage
  • Deliberate gene recombination (somatic hypermutation) - means potential for recombination errors
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6
Q

What is the gene recombination that occurs in bone marrow also called?

A

VDJ recombination

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7
Q

What is the downside of VDJ recombination?

A

Could lead to recombination errors and new point mutations
This means the formation of either OVEREXPRESSION OF PROLIFERATIVE ONCOGENE
or
SUPPRESSION OF APOPTOTIC GENE

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8
Q

What kind of lymphoma is a prime example of an error in recombination?

A

BURKITT’S

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9
Q

What types of malignant genes may be accidentally over expressed in VDJ recombination?

A

Myc (burkitt’s)
Bcl2
Bcl6
cyclin D

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10
Q

What are 3 MAIN causes for lymphoma?

A
  1. CONSTANT ANTIGENIC STIMULATION
  2. VIRAL INFECTION
  3. LOSS OF T cell FUNCTION
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11
Q

Give examples of how constant antigenic stimulation can cause lymphoma.

A

Helicobacter pylori: causes gastric MALT Marginal Zone NHL of stomach

Sjogrens: Marginal zone NHL of parotid

Hashimoto’s: Marginal zone NHL of thyroid

Coeliac: Small Bowel T cell lymphoma

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12
Q

Give examples of how viral infection causes lymphoma

A

HTLV1

EBV

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13
Q

Explain how HTLV1 works

A

HTLV1 infects T cells > develop Adult T cell Leukaemia Lymphoma (ATLL)

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14
Q

Explain how EBV works

A

EBV > infects B lymphocytes > usually quiescent (infected B cells killed by cytotoxic T cells)

If HIV / immunosuppression present, T cells cannot act > EBV causes B cell lymphoma

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15
Q

Give examples of how T cell function can belopst

A

HIV

Iatrogenic (immunosuppression)

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16
Q

What are the three key tissues of lymphoreticular system?

A

Generative LR tissue
Reactive LR tissue
Acquired LR tissue

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17
Q

What is function of Generative LR tissue

A

Generate / maturate B and T lymphoid cells

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18
Q

What are examples of generative LR tissue?

A

Bone marrow

Thymus

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19
Q

What is function of Reactive LR tissue

A

Develop immune reaction

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20
Q

What are examples of reactive LR tissue?

A

lymph node

spleen

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21
Q

What is the function of acquired LR tissue?

A

Develop LOCAL immune reaction

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22
Q

What are examples of acquired LR tissue?

A

Extra nodal lymphoid tissue e.g. skin, stomach, lung

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23
Q

Where are T cells found in the lymph node?

A

In the PARACORTEX

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24
Q

Where are B cells found in the lymph node?

A

In the LYMPHOID FOLLICLE

In the CORTEX

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25
What are the components of the lymphoid follicle?
Mantle zone - in the periphery, contains naive unstimulated B cells Germinal centre - in the centre, where B cells which bind to antigen epitopes are selected and activated
26
What are the main immunomarkers that we use in immunohistochemistry to detect whether cells are B or T cells?
B cells = CD20 | T cells = CD5, CD30
27
How is lymphoma classified?
``` Hodgkin (20%) Non Hodgkin (80%) ```
28
What are the types of Hodgkin Lymphoma?
Classical | Lymphocyte predominant
29
What are the types of NHL?
B cell | T cell
30
What is the most common type of Lymphoma?
B cell. NHL
31
What are histological features related to architecture that occur in lymphoma?
Nodular | Diffuse
32
What abnormalities are visible in immunophenotyping for lymphoma?
Cell distribution Loss of normal surface proteins Abnormal expression of proteins
33
What molecular tools are used for lymphoma?
FISH | PCR
34
What can these molecular tools identify?
Chromosome TRANSLOCATIOn
35
What are common types of B cell NH LOW GRADE lymphoma?
Follicular Small lymphocytic / chronic lymphocytic Marginal zone Mantle cell
36
What are common types B cell NH HIGH grade lymphoma?
Diffuse large B cell lymphoma | Burkitt's
37
Who does follicular lymphoma occur in?
middle aged/elderly
38
What is the histopathology of follicular lymphoma?
``` Follicular pattern (Follicles are NEOPLASTIC) Germinal centre cell origin ```
39
What translocation occurs in follicular lymphoma?
t(14,18) Involves Bcl2 gene (There is over expression of Bcl2 in the GERMINAL CENTRE (Bcl2 is usually only found in the mantle zone))
40
What is follicular lymphoma usually classified as in terms of aggression, and what can it become?
Indolent | Can become high grade lymphoma
41
What is the difference between Small and Chronic Lymphocytic Leukaemia?
``` Small = peripheral blood involved Chronic = bone marrow involved ```
42
Who does S/CLL present in?
middle aged/elderly
43
What is the histopathology of S/CLL?
Small lymphocytes Arises from naive B cells or port germinal centre memory B cells These replace the entire lymph node so you can no longer see follicles/T cells > become entire rounded sheets
44
What is Richter transformation?
The transformation of S/CLL from low grade to high gene
45
What proteins are B cells in S/CLL positive for?
CD5 | CD23
46
What is another name for MALT Lymphoma?
Marginal zone lymphoma
47
Where does MALT lymphoma arise?
It arises mainly at extranodal sites (gut, lung, spleen)
48
What does MALT lymphoma arise in response to?^
In response to chronic antigenic stimulation e.g. H pylori
49
What cells does MALT lymphoma arise from?
Post-geminal centre memory B cells
50
How can MALT lymphoma be treated?
With NON CHEMOTHERAPEUTIC modalities | By REMOVING THE ANTIGEN (eradicate H pylori)
51
How does Mantle cell lymphoma present?
In middle aged males, Affects lymph nodes, GI tract Presents as disseminated disease
52
What is the histopathology of Mantle Cell Lymphoma?
In Mantle zone of Lymph nodes
53
What cells does mantle cell L arise from?
From Pre-germinal centre cells
54
What proteins are abnormally expressed in Mantle Cell Lymphoma?
Cyclin D1
55
What translocation is responsible for Mantle cell lymphoma=?
11,14
56
What is the presentation for Burkitts lymphoma?
Jaw/abdo mass in children/adults respectively
57
What virus is burkitt's associated with?
EBV
58
Explain Burkitt histopathology?
Arises from germinal centre cells | Starry sky appearance
59
What is the molecular translocation and gene mutation with Burkitt's?
8,14 2,8 8,22 Resulting in c-Myc TRANSLOCATIon
60
What is the aggressiveness of Burkitt's?
HIGHLY AGGRESSIVE
61
What area does Diffuse Large B cell lymphoma arise from?
From germinal centre OR post-germinal centre B cells
62
What are 4 types of T cell lymphoma?
Adult T cell leukaemia lymphoma (ATLL) Enteropathy associated T cell lymphoma (EATL) Cutaneous T cell lymphoma Anaplastic large cell lymphoma
63
What virus is ATLL associated with?
HTLV-1
64
What comorbidity does EATL occur with=
COELIAC disease
65
How is Hodgkin presentation different to NHL?
Hodgkin: localised (usually just one node site), spreads contiguously to adjacent lymphocytes NHL: involves multiple sites, spreads discontinuously
66
Describe 3 features of classic HL
Nodular sclerosino Mixed cellularity with REED STERNBERG CELLS Lymphocyte rich/depleted
67
What are diagnostic markers for HL?
CD30+, CD15+ | CD20-
68
Whats the presentation of lymphocyte predominant HL=
Isolated lumphadenopathy
69
What virus is lymphocyte predominant HL associated with?
NONE
70
What are diagnostic markers for lymphocyte predominant HL?
CD30-, CD15- | CD20+
71
What do you need to know for diagnosis and staging of HL?
``` Histological Hx Anatomical stage (CT/MRI/PET) Prognostic factors (LBH; beta2 microglob, albumin, Hep B serology, BM function) ```
72
What is the age distribution for Hodgkins?
BIMODAL 1st peak 20-29 2nd peak >60
73
What is the gender distribution for Hodgkins?
1st peak - females more likely 2nd peak- males more likely overall - males slightly more likely
74
What is the general presentation of Hodgkins?
Painless lymphdenopathy B symptoms Alcohol induced lymph node pain
75
What are the 3 B symptoms?
Fever night sweats WL
76
What staging system is used for HL?
Ann Arbour staging
77
Explain stage 1-4 of Ann Arbour staging for HL
1: one group f nodes 2: >1 group of nodes on the same side of diaphragm (above/below) 3: nodes above AND below diaphragm 4: extra nodal spread A If no B symptoms B if min 1 B symptom
78
What is treatment for Hodgkins?
CHEMOTHERAPY for ALL PTSD Radiotherapy depending on circumstances
79
What is the chemotherapy combination used for HL?
ABVD Adriamycin Bleomycin Vincristine DTIC (dacarbazine)
80
What is a benefit of this type of chemotherapy?
It preserves fertility
81
What are long term disadvantages of ABVD?
Pulmonary fibrosis | Cardiomyopathy
82
What are negative consequences for radiotherapy?
DAMAGE TO NORMAL TISSUE ENCOUNTERED High risk of breast cancer leukaemia /myelodysplastic syndrome lung/skin cancer
83
What is a risk of combination radio-chemotherapy?
Increases rate pf relapse