Haem: Lymphoma 2, CLL and Lymphoproliferative disorder Pt.1

Question 1

Describe the typical presentation of lymphoma.

Answer 1

Painless progressive lymphadenopathy

• Can cause compression symptoms e.g. dysphagia if compressing oesophagus

Inflitration symptoms - skin rash, visual changes, neurological deficits

Recurrent infection (BM supression)

Constitutional symptoms (B symptoms)

Question 2

How is lymphoma diagnosed?

Answer 2

Biopsy with histological WHO classification of lymphoma subtype

Question 3

Which investigations may be used to stage lymphoma?

Answer 3

• Imaging - PET, CT, MRI
• Bone marrow biopsy
• Lumbar puncture (if CNS involvement)

Question 4

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

Answer 4

• 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

Question 5

What are the 2 main types of Hodgkin’s lymphoma

Answer 5

Classical (95%)

Nodular lymphocyte predominant (5%) - causes recurrent disease in elderly

Question 6

What is the main type of classical HL and which type has a poor prognosis?

Answer 6

Nodular sclerosing is the main type of classical HL (predominantly affects young women aged 20-30)

Lymphocyte depleted HL is the type that has a poor prognosis

Question 7

What is the chemotherapy regime used in cHL

Answer 7

ABVD

• Adriamycin
• Bleomycin
• Vinblastine
• Dacarbazine (DTIC)

Given at 4 weekly intervals for 2-6 cycles (dependant on stage and response)

Question 8

How is HL relapse treated

Answer 8

High dose chemotherapy with PB autologous stem cell transplant

Question 9

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

Answer 9

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

Question 10

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

Answer 10

Diffuse large B cell lymphoma (DLBCL) - 30%

Follicular lymphoma - 22%

Question 11

List some types of non-Hodgkin lymphoma that are:

1. Very agressive
2. Aggresive
3. Indolent

Answer 11

1. Very agressive
   
   • Burkitt’s lymphoma
   • T or B cell lymphoblastic lymphoma/leukaemia
2. Aggressive
   
   • Diffuse large B cell lymphoma
   • Mantle cell lymphoma
3. Indolent
   
   • Follicular lymphoma
   • Small lymphocytic lymphoma (CLL)
   • MALToma (marginal zone lymphoma)

Question 12

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

Answer 12

The more aggressive it is, the more curable

Indolent lymphoma is more likely to recur

Question 13

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

Answer 13

• Age
• Ann Arbor stage
• LDH
• Presence of extra-nodal disease
• ECOG performance status

Question 14

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

Answer 14

• R-CHOP
  
  • Rituximab
  • Cyclophosphamide
  • Doxorubucin
  • Vincristine
  • Prednisolone

NOTE: usually 6-8 cycles

NOTE: achieves a 50% cure rate

Question 15

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

Answer 15

Autologous stem cell transplantation

Question 16

Which genetic abnormality is associated with follicular lymphoma?

Answer 16

t(14;18) - over-expression of Bcl-2 (anti-apoptosis gene)

NOTE: follicular lymphoma is incurable but is indolent - median survival 12-15 years

Question 17

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

Answer 17

Watch and wait

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

Question 18

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

Answer 18

R-CVP (rituximab, cyclophosphamide, vincristine, prednisolone)

Question 19

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

Answer 19

Extranodal lymphoid tissue (e.g. MALT)

Question 20

What is the cause of marginal zone lymphoma?

Answer 20

• H. pylori infection - gastric MALToma
• Sjogren’s syndrome - parotid lymphoma
• Hashimoto’s thyroiditis - thyroid lymphoma

Question 21

Where is marginal zone lymphoma most commonly seen and how does it tend to present?

Answer 21

• Usually in the stomach
• Presenting with dyspepsia or epigastric pain
• Usually Stage 1{E} (E=extranodal)
• B symptoms are uncommon

Question 22

Outline the process of MALToma pathogenesis.

Answer 22

• Lymphocytes will respond chronic antigen stimulation from 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

Question 23

What are the symptoms of early stage gastric MALToma

Answer 23

Epigastric pain, ulceration, bleed

B-symptoms uncommon

Question 24

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

Answer 24

• Tripy 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

Question 25

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

Answer 25

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