Lymphoma (CH)

Question 1

What is a lymphoma?

Answer 1

Malignant proliferation of lymphocytes (B and T cells) which accumulate in lymph nodes or other organs

Question 2

What are the two main types of lymphoma?

Answer 2

• Hodgkin’s lymphoma
• non-Hodgkin’s lymphoma

Question 3

What is a Hodgkin’s lymphoma?

Answer 3

Malignant lymphoma of B-cell origin with no antibody expression

Question 4

How common is Hodgkin’s lymphoma?

Answer 4

Uncommon

Question 5

What is Hodgkin’s lymphoma characterised by?

Answer 5

Reed-Sternberg cells

Question 6

What types of Hodgkin’s lymphoma are there? (4)

Answer 6

• nodular sclerosing - associated with lacunar cells (most common)
• mixed cellularity
• lymphocyte predominant (best prognosis)
• lymphocyte depleted (worst prognosis)

Question 7

Describe the epidemiology of Hodgkin’s lymphoma.

Answer 7

• M>F
• bimodal age distribution:
  
  • 1st peak: 25-30 years
  • 2nd peak: 50-70 years

Question 8

What is 50% of Hodgkin’s lymphoma associated with?

Answer 8

EBV - viral particles allow infected abnormal B cells to evade apoptosis and/or replicate in an uncontrolled manner

Question 9

What conditions are associated with Hodgkin’s lymphoma? (3)

Answer 9

• EBV (50%)
• immunodeficiency
• autoimmune diseases e.g. RA, sarcoidosis

Question 10

What are some signs of poor prognosis of Hodgkin’s lymphoma? (4)

Answer 10

• B-symptoms (fever, weight loss, night sweats)
• male
• increasing age
• stage IV disease and lymphocyte depleted subtype

Question 11

What is a non-Hodgkin’s lymphoma?

Answer 11

Every other type of lymphoma that is not Hodgkin’s lymphoma (no Reed-Sternberg cells)

May affect B or T cells: 85% B cell, 15% T cell and NK form

Question 12

How common is non-Hodgkin’s lymphoma?

Answer 12

More common than HL

Question 13

What does incidence of non-Hodgkin’s lymphoma increase with?

Answer 13

Age

Question 14

What are some causes of non-Hodgkin’s lymphoma? (4)

Answer 14

• chromosomal translocations
• infections - EBV, HIV, H. pylori
• immunodeficiency
• autoimmune diseases

(and CLL –> Richter’s transformation)

Question 15

What are some subtypes of non-Hodgkin’s lymphoma? (3)

Answer 15

• diffuse large B-cell lymphoma
• Burkitt’s lymphoma
• MALT lymphoma

Question 16

What is the most common form of lymphoma in the UK?

Answer 16

Diffuse large cell B-lymphoma (type of non-Hodgkin’s lymphoma)

Question 17

What is Burkitt’s lymphoma?

Answer 17

Rapidly proliferating B cell tumour - type of non-Hodgkin’s lymphoma

Question 18

What would you see on microscopy in Burkitt’s lymphoma?

Answer 18

‘Starry sky’ appearance

(‘Sky’ due to densely packed malignant B cells which appear blue on H&E stain due to minimal cytoplasm; ‘starry’ due to fixation artefact derived from debris-filled macrophages)

Question 19

Which patients is Burkitt’s lymphoma common in?

Answer 19

HIV patients (or generally immunosuppressed) and young patients

Associated with EBV

Question 20

How can Burkitt’s lymphoma present?

Answer 20

Unusual ways due to rapid proliferation e.g. nerve root compression due to mass effect of lesion

Question 21

What is a common complication of Burkitt’s lymphoma?

Answer 21

Tumour lysis syndrome (PUKe calcium)

Question 22

What is a gastric MALT lymphoma?

Answer 22

• associated with H. pylori infection in 95% of cases
• good prognosis
• if low grade, 80% respond to H. pylori eradication
• paraproteinaemia may be present
• type of non-Hodgkin’s lymphoma

Question 23

What are the clinical features of Hodgkin’s lymphoma? (5)

Answer 23

• painless lymphadenopathy - cervical / supraclavicular nodes
• involvement of single group of lymph nodes
• alcohol-induced pain (at sites of lymphadenopathy)
• pruritus
• B-symptoms - fever >38, weight loss (>10% in 6m), night sweats

Question 24

What are the clinical features of non-Hodgkin’s lymphoma? (6+3)

Answer 24

• rubbery painless lymphadenopathy (associated with fatigue)
• affects multiple nodes
  
  • Waldeyer’s ring - oropharyngeal involvement
• splenomegaly + sometimes hepatomegaly
• B-symptoms (less common vs HL)
• fatigue and malaise
• extranodal disease:
  
  • GI - dyspepsia, dysphagia, abdominal pain
  • bone marrow - bone pain
  • neurological - headache

Question 25

What systems can non-Hodgkin's lymphoma affect? (3)

Answer 25

- GI - dyspepsia, dysphagia, abdominal pain - bone marrow - bone pain - neurological - headache

Question 26

Which type of lymphoma has alcohol-induced pain?

Answer 26

Hodgkin's lymphoma

Question 27

What are the B symptoms? (3)

Answer 27

- unexplained fevers >38 - unexplained weight loss >10% in 6 months - unexplained night sweats

Question 28

When do B symptoms occur in Hodgkin's vs non-Hodgkin's lymphoma?

Answer 28

Typically occur earlier in Hodgkin's lymphoma

Question 29

What are some risk factors for lymphoma? (5)

Answer 29

- age>50 - male - viruses - EBV, HIV, hep C - hereditary immunodeficiency syndromes - autoimmune conditions - RA, Sjogren's, Coeliac

Question 30

What are the first-line investigations for lymphoma? (4)

Answer 30

- lymph node biopsy - PET/CT scan - PET scan - FBC with differential

Question 31

What does histology and biopsy show in Hodgkin's lymphoma?

Answer 31

Reed-Sternberg cells: - binucleate lymphocytes - large multinucleate cells with eosinophilic nucleoli - mirror image nucleoli

Question 32

What does lymph node biopsy show in non-Hodgkin's lymphoma?

Answer 32

Positive

Question 33

What does FBC show in lymphoma?

Answer 33

Lymphocytosis

Question 34

What is immunohistochemistry useful for in non-Hodgkin's lymphoma?

Answer 34

Determines specific cell type and identifies specific markers

Question 35

When is ESR high in lymphoma?

Answer 35

Non-Hodgkin's lymphoma - ESR high Adverse prognostic factor: >50mm/hr without B symptoms or >30mm/hr with B symptoms

Question 36

What is PET-CT CAP used for in lymphoma?

Answer 36

Staging (Ann-Arbor staging)

Question 36

What might be seen in blood film in lymphoma?

Answer 36

- lymphoma cells may be seen - nucleated RBCs, left-shift (WBC precursors) from bone marrow involvement

Question 37

What is the Ann-Arbor staging system for lymphoma?

Answer 37

- stage I - 1 node affected - stage II - >1 node affected on same side of diaphragm - stage III - nodes affected on both sides of diaphragm - stage IV - extra-nodal involvement e.g. spleen, bone marrow, CNS - A - no B symptoms - B - B symptoms - X - bulky diseases (widening of mediastinum) - E - involvement of extra-nodal site

Question 38

What do we see on CXR in lymphoma? (2)

Answer 38

- widened mediastinum - bilateral hilar lymphadenopathy

Question 39

What is elevated LDH an indicator of in lymphoma?

Answer 39

Poor prognosis

Question 40

What are some differential diagnoses for lymphoma? (10)

Answer 40

- non-Hodgkin's lymphoma (Waldeyer ring + extra-nodal sites) - Hodgkin's lymphoma (Reed-Sternberg cells, alcohol-triggered pain) - CLL - peripheral blood lymphocytosis (precursors but not blasts), lymphadenopathy, hepatosplenomegaly - ALL - acute, purpura, bleeding, infection, blasts - infectious mononucleosis - pharyngitis, rash, myalgia, positive heterophile AB test - hepatitis C - CMV infection - TB - HIV infection - sarcoidosis

Question 41

What is the management plan for Hodgkin's lymphoma?

Answer 41

Chemotherapy +/- adjuvant radiotherapy

Question 42

What specific chemotherapy drugs are taken for Hodgkin's lymphoma?

Answer 42

ABVD - Adriamycin/doxorubicin - Bleomycin - Vinblastine - Dacarbazine

Question 43

What is the management plan for non-Hodgkin's lymphoma?

Answer 43

Chemotherapy +/- radiotherapy

Question 44

What specific chemotherapy drugs are taken for non-Hodgkin's lymphoma?

Answer 44

R-CHOP-21 Rituximab + Cyclophosphamide, doxorubicin, vincristine, Prednisolone for 21 days

Question 45

What are some complications of lymphomas?

Answer 45

- radiotherapy-related thyroid abnormalities (hypothyroidism most common) - chemotherapy and radiotherapy - secondary malignancies (AML, non-Hodgkin's lymphoma) - chemotherapy and radiotherapy --> accelerated diseases e.g. CAD - immunosuppression, infections, myelosuppression, renal failure etc

Question 46

Which lymphoma has better prognosis?

Answer 46

Hodgkin's lymphoma has better prognosis than non-Hodgkin's lymphoma