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7. Haematology > Lymphoma > Flashcards

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
Q

What are Non-Hodgkin’s lymphomas?

A

Includes all lymphomas without Reed-Sternberg cells
- 80% = B cell origin
- 20 % = T cell origin
- Not all centre on nodes

26
Q

What histological feature differentiates HL from NHL?

A

Presence of Reed-Sternberg cells (large multinucleated mirror-image nuclei) in HL

(They look like little owls)

27
Q

What AIDS defining illness is associated with NHL?

A

Burkitt’s lymphoma - jaw lymphadenopathy

28
Q

List some subtypes of NHL.

A

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
Q

What conditions is NHL associated with?

A

Autoimmune disorders - Sjogrens, RA, coeliac, SLE.

Immunodeficiency.

30
Q

Describe low grade non-hodgkins lymphoma.

A

Slow growing, advanced at presentation, often incurable.
Median survival is 10 years.

31
Q

Describe high grade non-hodgkins lymphoma.

A

Aggressive. Nodal presentation, patient unwell.
Often curable.

32
Q

How does NHL present? Give some key signs + symptoms.

A

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
Q

What investigations would you do in NHL?
What would you expect to see?

A
  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
Q

Describe the staging of lymphomas (same is used for NHL and HL)

A

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
Q

What combination chemotherapy regime is commonly used in patients with non hodgkin’s lymphoma?

A

RCHOP.

36
Q

How would you manage NHL?

A

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
Q

What is the treatment for low grade NHL?

A

If symptomless - do nothing.
Radiotherapy, combination chemotherapy and mAb may be used if symptomatic.

38
Q

Describe the treatment for high grade NHL.

A
  • Early: short course chemotherapy and radiotherapy.
  • Advanced: combination chemotherapy and mAb.

7. Haematology (15 decks)

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