Lymphoma

Question 1

What is the broad classification of lymphomas?

Answer 1

Hodgkin v non-Hodgkin

Question 2

How is Hodgkin lymphoma classified?

Answer 2

Classical v other

Question 3

How is non-Hodgkin lymphoma classified?

Answer 3

Sub classified according to:

• cell lineage e.g. B v T
• functionally into clinically distinct disease groups based on aggressiveness

Question 4

When should lymphoma be suspected?

Answer 4

• Palpable pathological lymphadenopathy (firm, rubbery, non-tender)
• Dyspneoa and cough w/ subsequent demonstration of mediastinal mass
• Abdo swelling and/or progressive discomfort (non-specific)
• PUO and night sweats

Question 5

Ix in ?lymphoma?

Answer 5

• FBE: anaemia (chronic disease), eosinophilia, leukocytosis
• Biochem: HIV, LFTs, UEC, ESR, LDH
• Imaging: CXR, CT CAP
• Excisional LN biopsy + histopath
• Immunohistochemistry
• Radiological staging
• BMA assessment (morphology, immunohistochemistry, flow cytometry)

Question 6

Microscopic features of lymph node biopsy of classical Hodkgkin lymphoma?

Answer 6

• Nodularity
• Sclerosis
• Reed Sternberg cells
• +/- occasional eosinophils

Question 7

How is diagnosis of Hodgkin lymphoma confirmed?

Answer 7

• CD30 immunostain showing clear membranous and Golgi apparatus staining of the Reed Sternberg cells
• CD15 +ve

Question 8

How is lymphoma staged?

Answer 8

Ann Arbor Staging (I-IV) A or B

Critical for Mx decisions.

Question 9

What is the Ann Arbor staging classification?

Answer 9

I: single region (i.e. one LN and surrounding area)
II: two separate regions, confined to one side of diaphragm
III: lymphoma involves LNs/organs on both sides of diaphragm
IV: diffused/disseminated involvement of extra lymphatic organs

Question 10

What does the A or B of Ann Arbor staging classification?

Answer 10

A or B: the absence of constitutional (B-type) symptoms (weight loss / fever / night sweats) is denoted by adding an A to the stage; adding B indicates presence of B symptoms.

Question 11

What are the considerations in determining treatment of lymphoma?

Answer 11

• Prognostic models to determine intensity
• potential impact on fertility in young women
• increased risk of breast malignancy / CAD due to RT exposure

Question 12

What is the prophylaxis against tumour lysis?

Answer 12

• Hydration
• Monitoring of electrolytes
• Drugs to lower uric acid

Question 13

How does follicular NHL often present?

Answer 13

• Low grade
• Often presents with painless, slowly growing lymphadenopathy (may wax and wane)
• Usually stag 4 at diagnosis (BM often involved)

Question 14

Histology of Burkitt lymphoma?

Answer 14

Cells with vacuolated cytoplasm

Question 15

What is ofatumumab?

Answer 15

Abs against target on B cell: CD22

Question 16

What is blinatumumab?

Answer 16

Anti CD19 (B cell antigen) combined with a receptor which attracts T cells, causing T cell mediated tumour cell kill.

Question 17

What is lymphoma?

Answer 17

Collection of lymphoid malignancies in which malignant lymphocytes accumulate at lymph nodes and lymphoid tissues.

Question 18

What is Hodgkin lymphoma?

Answer 18

Malignant proliferation of lymphoid cells with Reed Sternberg cells (though to arise from germinal centre B cells)

Question 19

Epidemiology of Hodgkin lymphoma?

Answer 19

• Bimodal distribution with peaks at 20y and >50y

- Ass/w EBV

Question 20

How does Hodgkin lymphoma classically present?

Answer 20

Painless, non-tender, firm, rubbery enlargement of superficial LNs most often in the cervical region.

Question 21

CFx of Hodgkin lymphoma?

Answer 21

• Asymptomatic lymphadenopathy (70%)
• Splenomegaly (50%)
• Mediastinal mass (routine CXR, SVC syndroe)
• Systemic symptoms (B Sx)

Question 22

What are the complications of Hodgkin lymphoma treatment?

Answer 22

• Cardiac disease 2” to RT
• Pulmonary disease 2” to bleomycin
• Infertility
• Secondary malignancy in irradiated field (MDS, AML, solid tumours of lung/breast)

Question 23

Prognosis of Hodgkin lymphoma?

Answer 23

Hasenclever adverse prognostic factors:
1. serum albumin 45y
6. Leukocytosis
7. Lymphocytopenia
Each score decreases freedom from progression at 5y.

Question 24

What is Non-Hodgkin lymphoma?

Answer 24

Malignant proliferation of lymphoid cells of progenitor or mature B or T cells

Question 25

What is the WHO classification of NHL?

Answer 25

1. INDOLENT: follicular lymphoma, small lymphocytes lymphoma, mantle cell lymphoma
2. AGGRESSIVE: diffuse large B cell lymphoma
3. HIGHLY AGGRESSIVE: Burkitt’s lymphoma

Question 26

How does NHL usually present?

Answer 26

• Painless superficial lymphadenopathy
• Cytopenia: when bone marrow involved
• Hepatosplenomegaly

Question 27

Treatment indolent NHL?

Answer 27

Goal = Sx Mx

• watchful waiting
• RT for localised disease
• CHOP + rituximab (anti CD20) for advanced disease

Question 28

What is CHOP?

Answer 28

• Cyclophosphamide
• Hydroxydoxorubicin (adriamycin)
• Oncovin (vincristine)
• Prednisone

Question 29

Treatment of aggressive NHL?

Answer 29

Goal = curative.

• combo chemo: CHOP +/- rituximab
• RT for localised disease
• CNS prophylaxis with high dose methotrexate if certain sites involved (testicular, nasopharyngeal)

Question 30

How does treatment differ between HL and NHL?

Answer 30

Treatment for HL depends on stage. Treatment for NHL depends on histologic subtype.

Question 31

Complications of NHL?

Answer 31

• Hypersplenism
• Infection
• AI haemolyti anaemia and thrombocytopenia
• Vascular obstruction (due to enlarged LNs)
• Tumour lysis syndrome

Question 32

What are the major types of NHL?

Answer 32

• Follicular
• Diffuse large B cell (DLBCL)
• Burkitt lymphoma
• Mantle cell

Question 33

What is Richter’s transformation?

Answer 33

5% CLL patients progress to DLBCL