Lymphoma 2 Flashcards by Ambreen Muhammed

What are the stages of lymphoma?

1 = 1 group of nodes
2 = > 1 group of nodes on the same side of the diaphragm
3 = > 1 group of nodes above and below the diaphragm
4 = extranodal spread
Suffix ‘B’ if B symptoms are present

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Which type of scan is often used to stage lymphoma?

FDG-PET/CT

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Which treatment modalities are used in Hodgkin lymphoma?

All patients receive chemotherapy
Radiotherapy is often used because Hodgkin lymphoma is very responsive
Referred to as ‘combined modality’ if both are used

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Which chemotherapy regimen is usually used for Hodgkin lymphoma?

ABVD: Adriamycin, Bleomycin, Vincristine, Dacarbazine
NOTE: this is usually given at 4-weekly intervals for 2-6 cycles

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What are some possible long-term consequences of chemotherapy for Hodgkin lymphoma?

Pulmonary fibrosis

Cardiomyopathy

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What is a risk of radiotherapy for Hodgkin lymphoma?

Collateral damage to surrounding tissues

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How might a relapse of Hodgkin lymphoma be treated?

High-dose chemotherapy
Autologous stem cell transplant
NOTE: intensifying chemotherapy will lead to an increased cure rate but it will also lead to an increase in secondary cancers

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Describe the curability of Hodgkin lymphoma.

Stage I and II: > 80%

Stage IV: 50%

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What are some important tests to perform in non-Hodgkin lymphoma and why are they important?

LDH – marker of cell turnover
HIV serology – HIV can predispose to NHL (HTLV1 serology may also be important)
Hepatitis B serology – NHL treatment may deplete B cells resulting in fulminant liver failure due to reactivation of hepatitis B in chronic carriers

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Broadly speaking, what are the treatment approaches to non-Hodgkin lymphoma?

Monitor only (in indolent lymphoma)
Urgent chemotherapy
Non-chemotherapy treatment (e.g. antibiotics to eradicate H. pylori)

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What are the two most common types of non-Hodgkin lymphoma?

Diffuse large B cell lymphoma (DLBCL)

Follicular lymphoma

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Which types of lymphoma are associated with other pre-existing diseases?

Gastric MALToma (H. pylori)
Enteropathy-associated T cell lymphoma (coeliac disease)
HIV-associated

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What is the correlation between how aggressive a lymphoma is and how curable it is?

The more aggressive it is, the more curable

Indolent lymphoma is more likely to recur

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What proportion of NHL are diffuse large B cell lymphoma?

30-40%

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Which factors are taken into account by the international prognostic index (IPI) for lymphoma?

Age > 60 
High LDH 
Performance status 2-4 
Stage III or IV 
More than one extranodal site

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Which chemotherapy treatment is usually used for diffuse large B cell lymphoma?

R-CHOP
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone 
NOTE: usually 6-8 cycles 
NOTE: achieves a 50% cure rate

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What treatment option may be considered for patients with diffuse large B cell lymphoma who relapse?

Autologous stem cell transplantation

What proportion of NHL is follicular lymphoma?

35%

Which genetic abnormality is associated with follicular lymphoma?

T(14;18) – resulting in over-expression of Bcl2 (which is an anti-apoptosis gene)
NOTE: follicular lymphoma is incurable but is indolent

What is the usual first-line treatment approach to follicular lymphoma?

Watch and wait

Only treat it clinically indicated (e.g. compression symptoms, massive nodes, recurrent infection)

Which chemotherapy regimen may be used in the treatment of follicular lymphoma?

R-CVP
Rituximab
Cyclophosphamide 
Vincristine 
Prednisolone

Which lymphoid tissue tends to be affected by marginal zone lymphoma?

Extranodal lymphoid tissue (e.g. MALT)

List some diseases that can lead to marginal zone lymphoma.

H. pylori infection – gastric MALToma
Sjogren’s syndrome – parotid lymphoma
Hashimoto’s thyroiditis – thyroid lymphoma
Psittaci infection – lacrimal gland

Outline the process of MALT lymphomagenesis.

Lymphocytes will respond to H. pylori infection and proliferate
At some point, they will over-proliferate and develop cancer-like features but they will still be dependent on antigenic stimulation by H. pylori
At this point, treating H. pylori will treat the lymphoma

24. How might gastric MALToma stage I-II disease be treated?

Triple therapy to eradicate H. pylori (2 antibiotics + 1 PPI) Repeat breath test at 2 months Repeat endoscopy every 6 months for 1-2 years then annually NOTE: failure may require chemotherapy

25. What are the main features of enteropathy-associated T cell lymphoma?

Mature T cells Involves small intestines Aggressive Caused by chronic antigenic stimulation by gliadin/gluten

28. What is the most common leukaemia in the Western world?

Chronic lymphocytic leukaemia

29. What are the typical laboratory findings in a patient with CLL?

Lymphocytosis Smear cells Normocytic normochromic anaemia Thrombocytopaenia Bone marrow lymphocytic replacement of normal marrow elements NOTE: it is indolent so is often only picked up on routine blood tests

30. What distinctive antigen phenotype (presence and absence) is suggestive of: a. Mature B cells b. Mature T cells

``` a. Mature B cells CD19 positive CD5 negative b. Mature T cells CD19 negative CD5 positive CD3 positive CD4 or CD8 positive ```

31. Which antigen phenotype is suggestive of CLL?

CD5+ B cells (i.e. CD19+ and CD5+) | NOTE: this could potentially also be mantle cell lymphoma

32. Which staging system is used for CLL?

Rai and Binet | Binet: stages A-C depending on number of lymphoid areas (< or > 3, Hb and platelets)

33. Which laboratory tests are used in CLL to help gauge prognosis?

CD38 expression (associated with poor prognosis) Cytogenetics (FISH) Immunoglobulin gene mutation status (IgH mutated or unmutated)

34. What are VH genes?

The genes that encode the ‘variable heavy’ chain and undergoes somatic hypermutation by VDJ recombination

35. What is the difference between the VH genes of pre- and post-germinal centre B cells?

Pre-germinal centre: VDJ section is unmutated and looks identical to germline Post-germinal centre: undergone somatic hypermutation so VDJ is mutated and looks different to germline

36. How does VH gene mutations affect prognosis?

``` Unmutated = poor prognosis Mutated = better prognosis ```

37. What is an important chromosomal abnormality in CLL that is tested for using FISH?

Deletion of 17p (Tp53) This is part of the p53 tumour suppressor gene This deletion is associated with a poor prognosis

Describe the immunoglobulin levels you would expect to see in CLL?

Hypogammaglobulinaemia | Because the malignant B cells are suppressing antibody production by other B cells

40. What are some supportive measures used in the treatment of CLL?

Vaccination (flu, pneumococcus) | Infection prophylaxis and treatment (may include aciclovir, PCP prophylaxis, IVIG)

44. What are some indications for treatment of CLL?

Progressive lymphocytosis (more than doubling in < 6 months) Progressive bone marrow failure Massive or progressive lymphadenopathy/splenomegaly Systemic symptoms (B symptoms) Autoimmune cytopaenias

45. What is the first line treatment for TP53 intact CLL?

BCR inhibitor – Ibrutinib (also affective in TP53 mutated cases) BCL2 inhibitor – Venetoclax – allows apoptosis of CLL cells – main SE is tumour lysis syndrome

48. Describe how CAR-T therapy for CLL works.

CAR-T are autologous T cells that are modified to contain chimeric antigen receptors The internal part of the receptor is responsible for cell signalling The external part is designed to target CD19 (on B cells) thereby enabling B cell depletion