Lymphoma and Myeloma

Question 1

Define lymphoma.

Answer 1

Cancer of lymphoid cells, commonly found in lymph nodes, but also occurring in extra-nodal sites

Question 2

What are the 2 main types of lymphoma?

How common is each?

Answer 2

Hodgkin’s lymphoma (15%)

Non-Hodgkin’s lymphoma (85%)

Question 3

What are the 2 types of Hodgkin’s lymphoma?

Answer 3

Nodular lymphocyte predominant (9%)
Classical HL (90%)

Question 4

Which virus is associated with classical Hodgkin’s lymphoma?

Answer 4

Epstein Barr virus

Question 5

Describe the cellular features of Hodgkin’s lymphoma on a biopsy. (2)

Answer 5

Mononuclear Hodgkin cell

Reed-Sternberg cell

• Arise from B cells
• Large, pale cytoplasm
• Oval, lobulated nuclei

Question 6

Describe the clinical features of classical Hodgkin’s lymphoma. (5)

Answer 6

Painless lymphadenopathy
Mass on CXR
B symptoms (unexplained fever, night sweats, weight loss)
Generalised pruritus
Alcohol induced lymph node pain

Question 7

How is Hodgkin’s lymphoma diagnosed?

Answer 7

Core biopsy (lymph node)
Excision biopsy (lymph node)

Question 8

Which staging system is used for Hodgkin’s and non-Hodgkin’s lymphoma?

Describe how lymphomas are staged with this system. (6)

Answer 8

Ann Arbor system

Number staging:
1 - single lymph node region affected
2 - 2+ lymph node areas affected on the SAME side of diaphragm
3 - lymph node areas affected on BOTH sides of diaphragm
4 - distant metastases

Letter staging:
A - absence of B symptoms
B - presence of B symptoms

Question 9

What are “B symptoms”?

Answer 9

Symptoms commonly caused by lymphoma involving B cells

Include:

• Unexplained fever
• Night sweats
• Weight loss

Question 10

How would you treat Hodgkin’s lymphoma?

Answer 10

Chemotherapy

Radiotherapy

Question 11

Describe the commonest chemotherapy regimen used in Hodgkin’s lymphoma. (4)

Answer 11

ABVD

Adriamycin (doxorubicon)
-Cytotoxic antibiotic

Bleomycin
-DNA synthesis inhibitor

Vinblastine
-Spindle formation inhibitor

Dacarbazine
-Alkylating agent

Question 12

What are the 2 main types of non-Hodgkin’s lymphomas?

Give an example of each.

Answer 12

Low grade lymphoma, e.g.
-Follicular lymphoma

High grade lymphoma, e.g.
-Diffuse large B cell lymphoma

Question 13

What is follicular lymphoma caused by?

Answer 13

T14;18 translocation

Question 14

Describe the clinical features of follicular lymphoma. (4)

Answer 14

Painless lymphadenopathy
Widespread lymph node involvement
Hepatosplenomegaly
B symptoms (fever, weight loss, night sweats)

Question 15

Which investigations would you do if you suspect follicular lymphoma? (3)

How would you diagnose it? (1)

Answer 15

Blood tests (including LDH)
CT scan
Bone marrow aspirate and trephine

Diagnosis:
-B cells in lymph node follicles staining for CD20+

Question 16

How would you treat follicular lymphoma? Consider:

a) Early stage (1)
b) Late stage (2)

Answer 16

EARLY STAGE:
Localised radiotherapy

ADVANCED STAGE:
If asymptomatic: watch and wait
If symptomatic, with organ compromise: R-chemotherapy

Question 17

What is R-chemotherapy?

Give 2 examples of R-chemotherapy regimens.

What is it used for?

Answer 17

Chemotherapy combined with rituximab, a monoclonal antibody against CD20 antigen on B cells

Examples:

• R-CHOP
• R-CVP

Used for:

• Follicular lymphoma (NHL)
• Diffuse large B cell lymphoma (NHL)

Question 18

Describe the clinical features of diffuse large B cell lymphoma. (4)

Answer 18

Extranodal symptoms, e.g.

• Skin discolouration/ulceration
• Gut/kidneys
• Lungs
• CNS
• Salivary glands
• Bones

Painless lymphadenopathy
Hepatosplenomegaly
Unexplained fever

Question 19

Which blood tests would you do for diffuse large B cell lymphoma? (2)

Answer 19

LDH - increased

Calcium - increased

Question 20

Which drugs are included in the R-CHOP chemotherapy regimen?

Answer 20

Rituximab
-Monoclonal antibody against CD20+

Cyclophosphamide
-Alkylating agent

Hydroxyaunoycin (adramycin/doxorubicin)
-Cytotoxic antibiotic

Oncovin (vincristine)
-Microtubule inhibitor

Prednisolone
-Steroid

Question 21

Define myeloma.

Answer 21

Cancer of the bone marrow plasma cells

Question 22

Describe the clinical features of myeloma. (8)

HINT: there are 2 categories of clinical features.

Answer 22

Non-specific symptoms, e.g.

• Back pain
• Fatigue
• Hypercalcaemia
• Recurrent infections
• Kidney impairment

Classic myeloma triad:

• Increased plasma cells in bone marrow
• Clonal immunoglobulin (paraprotein)
• Lytic bone lesions

Question 23

Describe the pathophysiology of myeloma. (4)

Answer 23

1. Myeloma causes formation of abnormal plasma cells
2. Myeloma cells produce abnormal proteins, called monoclonal proteins/paraproteins/M proteins, e.g.
   - IgA
   - IgD
   - IgE
3. Sometimes the myeloma cells don’t produce the whole Ig paraprotein but instead only an abnormal light chain
   a. This can cause kidney damage
4. Rarely, myeloma is secretory and there is NO Ig produced

Question 24

Which blood tests would you do if you suspected myeloma? (6)

Answer 24

FBC
ESR
U&Es
Serum calcium
Serum protein electrophoresis (SPE)
Serum free light chain levels (SFLC)

Question 25

Which urine tests would you do if you suspected myeloma? (1)

Answer 25

Paraproteins (M proteins) in urine

-Especially Bence-Jones protein

Question 26

Which feature might you see on a peripheral blood film in myeloma? (1)

Answer 26

RBC rouleaux

Question 27

How would you treat multiple myeloma? (5)

Answer 27

Autologous transplant
Chemotherapy
Radiotherapy
Bone marrow transplant
Supportive therapy

Question 28

Myeloma is incurable.

List 4 indications for myeloma therapy.

Answer 28

CRAB features:

Calcium elevation
Renal dysfunction
Anaemia
Bone disease (lytic lesions or osteoporisis)