Lymphoma Flashcards

1
Q

What is lymphoma?

A

A malignant growth of WBC’s predominantly in the lymph nodes.

A lymphoproliferative disorder – cancerous cells proliferate within lymph nodes inside the lymphatic system + cause them to enlarge abnormally

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2
Q

Difference between lymphomas and leukaemias?

A

Lymphomas are SOLID, leukaemias are CIRCULATING.
Dx confirmed by tissue sampling.

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3
Q

Although predominantly in the lymph nodes, lymphoma is systemic.
What other organs might it effect?

A
  1. Blood.
  2. Liver.
  3. Spleen.
  4. Bone marrow.
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4
Q

Give 4 risk factors for lymphoma.

A
  1. Primary immunodeficiency.
  2. Secondary immunodeficiency e.g. HIV.
  3. Infection e.g. EBV, HTLV-1, H.pylori.
  4. Autoimmune disorders e.g. RA, SLE.
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5
Q

Describe the pathophysiology of lymphoma.

A

There is impaired immunosurveillance and infected B cells escape regulation and proliferate. (This is just a theory).

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6
Q

Give 4 symptoms of lymphoma.

A
  1. Enlarged lymph nodes in arm/neck.
  2. Symptoms of compression syndromes.
  3. General systemic ‘B’ symptoms e.g. weight loss, night sweats, malaise.
  4. Liver and spleen enlargement.
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7
Q

What investigations might you do in someone who you suspect has lymphoma?

A
  1. Lymph node excision / biopsy
  2. Blood films
  3. Imaging - CT, MRI and PET scans
  4. Immunophenotyping.
  5. Cytogenetics.
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8
Q

What are the two sub-types of lymphoma?

A
  1. Hodgkins lymphoma.
  2. Non-hodgkins lymphoma.
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9
Q

What are the 2 types of Hodgkin’s lymphoma?

A
  1. Classical Hodgkin’s lymphoma (cHL)
    * The hallmark of which is the REED-STERNBERG CELL with mirror image nuclei
    * Accounts for 90-95% of cases
  2. Nodular lymphocyte predominant Hodgkin’s Lymphoma (NLPHL)
    * Characterised by the Reed-Sternberg VARIANT, the ‘POPCORN CELL’
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10
Q

Give 3 risk factors for Hodgkin’s lymphoma.

A
  • EBV infection (glandular fever)
  • HIV
  • Autoimmune conditions e.g. SLE, RA, sarcoidosis
  • Fx – affected sibling
  • Post-transplantation
  • Obesity
  • Male sex predominance
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11
Q

Describe the epidemiology of Hodgkin’s lymphoma.

A
  • Bimodal age = 2 peaks of incidence (teenagers and elderly – 13-19 and >65)
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12
Q

What are the symptoms of Hodgkin’s lymphoma?

A
  1. Painless lymphadenopathy.
  2. Presence of ‘B’ symptoms e.g. night sweats, weight loss, fever.
  3. Hepatosplenomegaly.
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13
Q

What features of a Hx would suggest Hodgkin’s lymphoma?

A
  • Painless supraclavicular or cervical lymphadenopathy (may spontaneously increase/decrease in size)
  • Risk factors
  • B symptoms (25%)
  • Pruritus (10%)
  • Alcohol induced pain - LOOK out for in questions - causes pain in the lymph nodes!! - SPECIFIC
  • Symptoms of mediastinal adenopathy (dry cough, dyspnoea, chest pain, SVC syndrome)
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14
Q

Describe the lymphadenopathy seen in Hodgkin’s lymphoma.

A

Enlarged, painless, rubbery superficial lymphadenopathy.

Most commonly presents with cervical or supraclavicular painless lymphadenopathy
“but I do get pain in my neck when I’ve had a drink doctor…”

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15
Q

What investigations would you carry out in Hodgkin’s lymphoma?

What results would you expect?

A
  1. Lymph node excision / biopsy – diagnostic!!!
    • Reed-Sternberg cells (popcorn cells)
      o Abnormally large B cells that have multiple nuclei that have
      nucleoli inside them.
      o This can give them the appearance of the face of an owl
      with large eyes.
  2. Bloods
    * High ESR or Low Hb - indicate worse prognosis
    * High serum lactate dehydrogenase
    * Neutrophilia
  3. Imaging – CT, MRI and PET scans
    * Of the chest, abdomen + pelvis to see at which stage
  4. Immunophenotyping
  5. Cytogenetics
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16
Q

What is needed for diagnosis of Hodgkins lymphoma?

A

Presence of Reed-sternberg cells.

17
Q

Describe the staging of Hodgkins lymphoma.

A

Used for both HL and NHL.
The system puts importance on whether the affected nodes are above or below the diaphragm.

Each stage is either A or B:
* A: no systemic symptoms other than pruritus (severe itching of skin)
* B: presence of B symptoms such as fever, weight loss and night sweats

Stage 1: Confined to 1 region of lymph nodes.
Stage 2: In > 1 region but on the same side of the diaphragm (either above or below).
Stage 3: Affects lymph nodes both above and below the diaphragm.
Stage 4: Widespread involvement including non-lymphatic organs such as the lungs or liver.

18
Q

How would you manage Hodgkin’s lymphoma?

A

Combination chemotherapy: ABVD
A – Adriamycin
B – Bleomycin
V – Vinblastine
D – Dacarbazine

19
Q

What is the treatment for stage 1 - 2A Hodgkins lymphoma?

A

Short course of ABVD followed by radiotherapy

20
Q

What is the treatment for stage 2B - 4 Hodgkins lymphoma?

A

Longer course of ABVD (combination chemotherapy)

21
Q

What are the possible complications of treatment for Hodgkin’s lymphoma?

A
  1. Secondary malignancies.
  2. IHD.
  3. Infertility.
  4. Nausea.
  5. Alopecia.
22
Q

What complications can chemotherapy for HL cause?

A
  • Chemotherapy -> creates a risk of leukaemia and infertility.
23
Q

What complications can radiotherapy for HL cause?

A
  • Radiotherapy -> creates a risk of cancer, damage to tissues and hypothyroidism.
24
Q

What are complications of HL?

A
  • Myelosuppression and neutropenic sepsis.
  • Tumour lysis syndrome.
  • Impaired immunity.
25
What are Non-Hodgkin's lymphomas?
Includes all lymphomas without Reed-Sternberg cells - 80% = B cell origin - 20 % = T cell origin - Not all centre on nodes
26
What histological feature differentiates HL from NHL?
Presence of Reed-Sternberg cells (large multinucleated mirror-image nuclei) in HL (They look like little owls)
27
What AIDS defining illness is associated with NHL?
Burkitt's lymphoma - jaw lymphadenopathy
28
List some subtypes of NHL.
1) Diffuse large B-cell lymphoma (30%) - aggressive/high grade 2) Follicular lymphoma (20%) - indolent/low grade 3) Burkitt’s (HG) (1%) - EBV - characteristic jaw lymphadenopathy (child) 4) Primary CNS lymphoma (1%) - EBV with AIDS Also: 5) MALT (LG) (gastric mucosa associated lymphoid tissue) - H.pylori 6) Marginal zone - massive splenomegaly 7) T cell
29
What conditions is NHL associated with?
Autoimmune disorders - Sjogrens, RA, coeliac, SLE. Immunodeficiency.
30
Describe low grade non-hodgkins lymphoma.
Slow growing, advanced at presentation, often incurable. Median survival is 10 years.
31
Describe high grade non-hodgkins lymphoma.
Aggressive. Nodal presentation, patient unwell. Often curable.
32
How does NHL present? Give some key signs + symptoms.
1) Nodal signs - lymphadenopathy (can be rapid) 2) Extranodal signs: - Bone marrow - pancytopenia (fatigue, dyspnoea) - Splenomegaly (massive in marginal zone) + hepatomegaly - Dry cough - mediastinal mass/pneumonia - Skin (T cell) - Gut: diarrhoea, vomiting, abdominal pain (MALT) - Bone - bone pain - CNS 3) B symptoms (less common than Hodgkin’s) - Night sweats - Fever - Weight loss > 10%
33
What investigations would you do in NHL? What would you expect to see?
1. FBC - Raised ESR/LDH ( lactose dehydrogenase) - reflects worse prognosis since its a sign of increased cell turnover and thus cell proliferation - Normocytic anaemia, thrombocytopenia or pancytopenia 2. Lymph node excision or bone marrow biopsy - Will NOT see mirror-image nuclei REED-STERNBERG CELLS or Reed-Sternberg variant cells - POPCORN CELLS 3. Marrow and node biopsy - For classification 4. CT/MRI - Of chest, abdomen and pelvis for staging (Ann Arbor) 5. Immunophenotyping 6. Cytogenetics
34
Describe the staging of lymphomas (same is used for NHL and HL)
Ann Arbor system (get a number and a letter): 1 - Single lymph node group 2- Multiple on same side diaphragm 3 - Multiple on opposite side diaphragm 4 - Multiple extranodal sites or lymph nodes and extranodal disease E - extranodal extension B - weight loss > 10%, fever, drenching night sweats A - no B symptoms B symps = worse prognosis
35
What combination chemotherapy regime is commonly used in patients with non hodgkin's lymphoma?
RCHOP.
36
How would you manage NHL?
R-CHOP-21 regimen: * R - RITUXIMAB (monoclonal antibody - minimal side effects) * C - CYCLOPHOSPHAMIDE * H - HYDROXY-DAUNORUBICIN * O - VINCRISTINE (Oncovin brand name) * P - PREDNISOLONE for 21 days
37
What is the treatment for low grade NHL?
If symptomless - do nothing. Radiotherapy, combination chemotherapy and mAb may be used if symptomatic.
38
Describe the treatment for high grade NHL.
- Early: short course chemotherapy and radiotherapy. - Advanced: combination chemotherapy and mAb.