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Y5 Pathology > Haem - Lymphoma 1 and 2 > Flashcards

Haem - Lymphoma 1 and 2 Flashcards

1
Q

Define lymphoma

A

Neoplastic (malignant) proliferation/tumour of lymphoid cells to form discrete tissue masses

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2
Q

In which sites may lymphoma be found

A

Lymph nodes, bone marrow and/or blood (the lymphatic system)
Lymphoid organs; spleen or the gut-associated lymphoid tissue
Skin (often T cell disease; e.g. Mycoses Fungoides)
Rarely “anywhere” (Sanctuary sites: CNS, ocular, testes, breast, etc.)

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3
Q

What are the types of lymphoid malignancies

A

Hodgkin’s
- Classical
- Nodular lymphocyte predominant

Non-Hodgkin’s
- B cell (most common - 80%)
- T cell

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4
Q

What are the types of classical Hodgkins lymphoma

A

Nodular sclerosing
Mixed cellular
Lymphocyte depletion
Lymphocyte rich

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5
Q

What are the types of B cell non-Hodgkin lymphoma

A

Low grade
MALToma
Small lymphocytic lymphoma (CLL)
Follicular

High grade
Diffuse large B cell lymphoma (DLBCL)
Mantle cell

Aggressive
Burkitt’s

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6
Q

What are the types of T cell non-Hodgkin lymphoma

A

Anaplastic large cell lymphoma
Adult T cell leukaemia lymphoma (ATLL)
Enteropathy-associated T-cell lymphoma (EATL)
Cutaneous (mycoises fungoides)

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7
Q

What are the positives and negatives of DNA instability in the immune system

A

Positive
Allows for recombination → diversity
Rapid proliferation for rapid response to infection
Apoptosis dependency for specificity, elimination of self-reactive clones

Negative
Recombination → unwanted point mutations
Rapid proliferation → replication errors
Apoptosis dependency: apoptosis may be switched off

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8
Q

What are the stages of immunoglobulin and TCR gene recombination

A
  1. VDJ recombination in the bone marrow involving RAG1 and RAG2
  2. Class switch recombination: somatic hypermutation in the germinal centre - heavy chain of Ig changed and the oncogenes are brought closer to the promoter
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9
Q

What are the divisions of the lymphoreticular system

A
  1. Generative lymphoreticular (LR) tissue - generation/maturation of lymphoid cells in bone marrow and thymus
  2. Reactive LR - immune reaction in lymph nodes and spleen
  3. Acquired LR tissue - local immune reaction in extra-nodal tissue (skin, stomach, lungs)
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10
Q

Describe the histology of a lymph node

A

Germinal centre of B cells and antigen presenting cells
Surrounded by the mantle zone - naive, unstimulated B cells
Surrounded by a paracortical T cell zone
Lymph node sinuses between follicles

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11
Q

What investigations should be done for lymphoma

A

Bloods:
- LDH: raised
- Albumin
- U&Es
- HIV, hep B serology ± HTLV-1 (ensure hep B will not reactivate)

Anatomical stage
- bone marrow biopsy
- CT/PET
- Lumbar puncture if risk of CNS involvement

Cytology (cells aspirated from lump)
Histology (tissue sections)

Cytogenetics
FISH - chromosomal translocations
PCR - chromosomal translocations, gene rearrangement

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12
Q

What are the lymphoma CD markers for NHL

A

CD19, CD20 = B-cells
CD3, CD5 = T-cells

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13
Q

Which Ig are associated with B cell lymphomas

A

Follicular NHL: IgH-BCL2
Mantle Cell lymphoma: IgH-Cyclin D1
Burkitt Lymphoma: IgH-MYC

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14
Q

What are the symptoms of lymphoma

A

Painless progressive lymphadenopathy (neck, axilla groin)
Compression symptoms
- Renal failure
- Obstructive jaundice
- Large blood vessels
- Bowel obstruction
- Trachea
- Oesophageal
Infiltration
- Brain: stroke, lOC, memory problems
- Skin rash: plaques, ulceration, nodules
- Ocular: blindess
- Liver: liver failure
Recurrent infections
B symptoms: fever, night sweats, weight loss

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15
Q

What should be looked at for histology of a lymphoma

A
  • Architecture: nodular, diffuse
  • Histology: small round, small cleaved, large cells
    Immunophenotyping:
  • Cell types (CD markers)
  • Cell distribution
  • loss of surface proteins
  • Abnormal expression of proteins
  • Clonality of B cells
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16
Q

What are the features of follicular lymphoma (epidemiology, time course, molecular, histopathology)

A

Middle-aged or elderly
Indolent, relatively incurable
t(14;18) involving bcl-2 gene
Histo: neoplastic follicles, positive CD10 and bcl-2 staining, centroblasts (lymph node biopsy)

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17
Q

What are the features of small lymphocytic lymphoma (association, epidemiology, histopathology)

A

CLL
Associated with Richter transformation (presents with NEW B symptoms)
Middle aged or elderly
Histo: small lymphocytes, arises from naive B cells, CD5 and CD23 positive

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18
Q

What are the features of marginal zone lymphoma/MALToma (cause, timeline, management)

A

Response to chronic antigenic stimulation
Post germinal centre memory B cell
Indolent but could transform
Management: remove the antigenic stimulation

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19
Q

Give examples of MALT and their causes

A

H. pylori → gastric
Sjogren’s → parotid gland
Hashimotos → thyroid MZL
Psittaci infection → lacrimal gland

20
Q

What are the features of diffuse large B cell lymphoma (epidemiology, histopathology, prognosis)

A

Middle aged and elderly
Histo: from germinal centre/post-germinal centre, sheets of large lymphoid cells
p53+: Poor prognosis

21
Q

What are the features of mantle cell lymphoma (epidemiology, histopathology, molecular associations, prognosis)

A

Middle aged males
Histo: seen in the mantle zone, arising from pre-germinal centre cells, expression of CD5 and cyclin D1, clefted nuclei
Molecular: t(11;14), cyclin D1 overexpression
Prognosis: 2-5 years

22
Q

What are the features of Burkitt’s lymphoma (presentation, association, histopathology, molecular associations)

A

Jaw/abdo mass when young
Associated with EBV
Histo: starry-sky appearance, arises from germinal centre cells
Molecular: C-myC translocation (8;14) OR (2;8) OR (8;22)

23
Q

What are the features of anaplastic large cell lymphoma (epidemiology, histopathology, molecular associations, prognostic factors)

A

Younger patients
Histo: large epithelioid lymphocyte, anaplastic
Molecular: t(2;5)
Alk-1 protein expression = better prognosis

24
Q

Describe enteropathy associated T-cell lymphoma (cause and association, histology, presentation, response to treatment, prevention)

A

Caused by chronic antigen stimulation to gluten/gliadin → associated with coeliac disease
Histology: Mature T cells TdT, CD19
Presentation:
- Abdo pain, obstruction, perforation, GI bleeding
- Malabsorption
- Systemic symptoms

Responds poorly to chemotherapy, generally fatal
Can be prevented by strict adherence to a gluten-free diet

25
Q

What are the associations with Adult T cell leukaemia lymphoma (ATLL)

A

HTLV-1 infection
Japanese and Caribbean ethnicity
Histo: flower cells

26
Q

What is cutaneous T cell NHL associated with

A

Mycosis fungoides

27
Q

What is the epidemiology of Hodgkin’s lymphoma

A

1% of all cancer
Less common than non-Hodgkin’s
M > F
Bimodal age - 20-29yo (most common), >60yo (smaller peak)

28
Q

What are the symptoms of Hodgkin’s lymphoma

A

Asymmetric painless lymphadenopathy
Pain in nodules after alcohol ingestion

29
Q

What are the diagnostic features of hodgkin’s lymphoma on investigation

A

From germinal centre OR post-germinal centre
Associated with EBV
Histo: sclerosis, Reed-Sternberg cells (binucleate), lymphoma cells, Eosinophils
Markers: CD30+, CD15+, CD20 -ve

30
Q

Describe the features of nodular sclerosing HL sub-type (epidemiology, symptoms)

A

Young women (20-29)
Neck nodes and a mediastinal mass → SVC or trachea compression
Spreads contiguously

31
Q

What are the features of nodular lymphocyte predominant lymphoma (presentation, association, histopathology, diagnostic markers)

A

Isolated lymphadenopathy
May transform to a high grade B cell NHL
Histo: b cell rich nodules, NO eos/macro

NEGATIVE for CD30 + CD15 (which is seen in classical Hodgkin)
POSITIVE for CD20

32
Q

Describe the staging of lymphoma

A

Ann Arbor Staging
A = B symptoms absent
B = B symptoms present
E = involvement of single, contiguous, or proximal extranodal site

I: One node region involved on 1 side of the diaphragm
II: 2+ ipsilateral regions involved on 1 side of the diaphragm
III: Bilateral node involvement on both sides of the diaphragm
IV:
HL: Involvement of extranodal sites beyond those designated by ‘E’ (below)
NHL: Disseminated/multifocal involvement of ≥1 extralymphatic site OR isolated extralymphatic organ involvement with distant node involvement

33
Q

Describe the treatment of Hodgkin’s lymphoma

A

Chemotherapy: ABVD (2-6 cycles, 4-weekly intervals) - adriamycin, bleomycin, vinblastine, DTIC (Dacarbazine)
± radiotherapy

Second line: salvage chemotherapy (high dose chemo + HSCT)

Third line: anti-CD30 (Brentuximab Vedotin)+ anti-PD1(nivolumab)

34
Q

What is the management for NHL

A

Chemotherapy: R-CHOP (6-8 cycles) - rituximab, cylophosphamide, adriamycin, vincristine, prednisolone

Second line: autologous HSCT

35
Q

What is the prognosis for NHL

A

Burkitt’s lymphoma – fastest growing human cancer
Follicular lymphoma (an indolent disease) – possible 25 year survival
HOWEVER, chance of being cured increases with more aggressive neoplasms

36
Q

What are the prognostic markers for NHL

A

LDH (marker of cell turnover)
Performance status
HIV serology
Hep B serology (treatment for lymphoma may cause a dormant hep B to reactivated → fulminant liver failure)

37
Q

What is a common complication of Chemotherapy + radiotherapy for Hodgkin’s lymphoma

A

Risk of damage to normal tissues (collateral damage)
Associated with increased risk of breast/lung/skin cancer, leukaemia/myelodysplasia

38
Q

What is the prognosis for Hodgkin’s lymphoma

A

Elderly patients do worse than the worst-prognosis HL (lymphocyte-depleted type)
It is a highly curable disease, but the prognosis depends on stage
Over 80% in stage I or II disease are cured
50% of stage IV are cured
10% die from relapse within 10 years and 10% die from complications after 10 years
More likely to die of secondary malignancy of CV complications after 5 years

39
Q

What phenotype is indicative of CLL

A

Normal T cells are CD5 positive, and CD 19 and 20 negative. While normal B cells are the other way around.
CLL B cells have an unusual phenotype (Monoclonal lymphocytosis with abnormal expression of CD5 = highly suggestive of CLL)

40
Q

What is the phenotype of T cells

A

CD3 positive
CD4 or CD8 positive (Th-cell, CTL cell)
CD19 NEGATIVE
CD5 POSITIVE

41
Q

What is the prognosis for CLL

A

In a disorder of elderly
- 1/3 never progress
- 1/3 Progress, respond to CLL Rx (death from unrelated disorder)
- 1/3 Progress, require multiple lines of Rx, refractory disease, death from CLL

42
Q

How is CLL staged

A

Rai and Binet staging
A: <3 lymphoid areas
B: >3 lymphoid areas
C Hb <100, Platelets <100

43
Q

What are the complications of CLL

A

Non-functioning B cell production → hypogammaglobulinaemia → increased infection risk e.g. pneumonia, sinusitis
Metastasis to lymphoid organs e.g. lymph nodes, spleen
Bone marrow failure (anaemia, neutropenia thrombocytopenia)
Richter transformation → high grade lymphoma
Regulation of the surviving normal B cell population → autoimmune disease e.g. autoimmune haemolytic anaemia

44
Q

What are the prognostic lab factors for CLL

A

Cytogenetics (FISH panel)
Immunoglobulin gene (IgHV) mutation status
- IgH mutated
- IgH unmutated (Bad)
17p deletion
p53 muted

45
Q

What are the poor prognostic features of CLL

A

Deletion of 13q (~133 months survival)
Trisomy 12 (~114)
Deletion of 11q (ATM) (~79)
Deletion of 17p (TP53) - loss of p53 tumour

Y5 Pathology (71 decks)

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