Lymphoma (Non-Hodgkin's) Flashcards

1
Q

What is lymphoma?

A

Lymphomas are a group of cancers that affect the lymphocytes inside the lymphatic system. These cancerous cells proliferate within the lymph nodes and cause the lymph nodes to become abnormally large (lymphadenopathy).

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2
Q

Briefly differenttiate between Hodgkin’s and non-Hodgkin’s lymphoma

A

There are two main categories of lymphoma: Hodgkin’s lymphoma and non-Hodgkin’s lymphoma. Hodgkin’s lymphoma is a specific disease and non-Hodgkins lymphoma encompasses all the other lymphomas.

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3
Q

Out of all the lymphomas, how many are Hodgkin’s lymphoma?

A

Overall 1 in 5 lymphomas are Hodgkin’s lymphoma.

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4
Q

What is non-Hodgkin’s lymphoma?

A

Non-Hodgkin’s lymphoma is an umbrella term for a group of malignancies affecting the lymphoid system.

It is defined as all lymphomas without Reed-Sternberg cells.

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5
Q

How is non-Hodgkin’s lymphoma classified?

A

There are over 30 different types, and they may be classified according to cell of origin (B-cell or T-cell) or pathological grade (high-grade or low-grade).

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6
Q

What are the risk factors for non-Hodgkin’s lymphoma?

A

Risk factors for non-Hodgkin’s lymphoma include:

  • HIV
  • Epstein-Barr Virus
  • H. pylori (MALT lymphoma)
  • Hepatitis B or C infection
  • Exposure to pesticides and a specific chemical called trichloroethylene used in several industrial processes
  • Family history
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7
Q

Give examples of the common non-Hodgkin’s lymphoma

A

Non-Hodgkins lymphoma is a group of lymphomas. There are almost endless types of lymphoma. A few notable ones are:

  • Burkitt lymphoma
  • MALT lymphoma
  • Diffuse large B cell lymphoma
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8
Q

What are the specific features of Burkitt lymphoma?

A

Burkitt lymphoma is associated with Epstein-Barr virus, malaria and HIV.

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9
Q

What are the specific features of MALT lymphoma?

A

MALT lymphoma affects the mucosa-associated lymphoid tissue, usually around the stomach. It is associated with H. pylori infection.

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10
Q

What are the specific features of diffuse large B cell lymphoma?

A

Diffuse large B cell lymphoma often presents as a rapidly growing painless mass in patients over 65 years.

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11
Q

How does non-Hodgkin’s lymphoma present?

A

The presentation is similar to Hodgkin’s lymphoma and often they can only be differentiated when the lymph node is biopsied.

Lymphadenopathy is the key presenting symptom. The enlarged lymph node or nodes might be in the neck, axilla (armpit) or inguinal (groin) region. They are characteristically non-tenderand feel “rubbery”.

B symptoms are the systemic symptoms of lymphoma:

  • Fever
  • Weight loss
  • Night sweats

Other symptoms can include:

  • Fatigue
  • Itching
  • Cough
  • Shortness of breath
  • Abdominal pain
  • Recurrent infections
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12
Q

With regards to clinical features, how can non-Hodgkin’s lymphoma and Hodgkin’s lymphoma be differentiated?

A

The lymphadenopathy is more likely to be symmetrical in non-Hodgkin’s lymphoma, at multiple sites, and spread discontinuously across nodal sites.

B symptoms typically occur earlier in Hodgkin’s lymphoma and later in non-Hodgkin’s lymphoma.

B symptoms (fever, night sweats, and weight loss) are more common in non-Hodgkin’s lymphoma than in Hodgkin’s lymphoma and are a poor prognostic marker.

Lymphadenopathy in Hodgkin’s lymphoma can experience alcohol-induced pain in the node.

Extra-nodal disease is much more common in non-Hodgkin’s lymphoma than in Hodgkin’s lymphoma.

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13
Q

What staging is used for non-Hodgkin’s lymphoma?

A

Ann Arbor staging system.

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14
Q

Briefly describe Ann Arbour Staging

A

Stage 1: confined to one region of lymph nodes.

Stage 2: in more than one 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.

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15
Q

What investigations confirm the diagnosis of non-Hodgkin’s lymphoma?

A

Core biopsy or excision biopsy of an affected lymph node or other organ.

Blood tests to check for underlying causes (HIV serology) and complications (FBC, U&E, LFT, calcium and phosphate, LDH, immunoglobulins, serum electrophoresis).

A staging CT scan (with iv contrast) of neck, chest, abdomen and pelvis.

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16
Q

What is the gold standard diagnostic test for Non-Hodgkin’s lymphoma?

A

Excisional node biopsy is the diagnostic investigation of choice.

17
Q

How does Burkitt’s lymphoma appear on excisional node biopsy?

A

Classical appearance on biopsy such as Burkitt’s lymphoma having a ‘starry sky’ appearance.

18
Q

Why is LDH important in diagnosing non-Hodgkin’s lymphoma?

A

The main blood result to remember is an elevated LDH, which is a poor prognostic marker as it reflects greater red cell turnover.

19
Q

How does a blood film of non-Hodgkin’s lymphoma present?

A

The blood film typically reveals nucleated red cells and left shift (the presence of early white blood cell precursors) - particularly in the high grade cancers. This occurs due to marrow involvement and increased marrow turnover.

20
Q

What are the treatment options for non-Hodgkin’s lymphoma?

A

Management involves a combination of treatments depending on the type and staging of the lymphoma:

  • Watchful waiting
  • Chemotherapy
  • Monoclonal antibodies such as rituximab
  • Radiotherapy
  • Stem cell transplantation

Management is dependent on the specific sub-type of non-Hodgkin’s lymphoma.

21
Q

Briefly describe the treatment of low grade non-Hodgkin’s lymphoma

A

Treatment is not indicated if the disease is asymptomatic so a watch-and-wait strategy may initially be adopted.

Early stage disease (stage I or II) may be treated with local radiotherapy only if local tissues are not very radio-sensitive.

Disseminated disease is treated with chemotherapy, often in combination with a monoclonal antibody e.g. R-CVP.

Low-grade NHL is not generally considered curable. At relapse retreatment with further chemotherapy or radiotherapy can be undertaken, but response rates are lower.

Recently new kinase inhibitors (ibrutinib, which inhibits the Bruton’s tyrosine kinase, and idelalisib, which inhibits phosphoinositide 3-kinase) have been licensed for use in low grade NHL, which have higher response rates than previous treatments.

22
Q

Briefly describe R-CVP regime

A

Can be used in low grade non-Hodgkin’s lymphoma.

Rituximab, an anti-CD20 monoclonal antibody, combined with cyclophosphamide, vincristine and prednisolone.

23
Q

Briefly describe the treatment for high grade non-Hodgkin’s lymphoma

A

High grade NHL is potentially curable in patients fit enough to tolerate treatment.

For those with localised disease, a short course of chemotherapy may be combined with local radiotherapy.

Most patients have disseminated disease and require combination chemotherapy e.g. R-CHOP.

Those with central nervous system involvement or breast involvement should also receive intrathecal treatment, such as intrathecal methotrexate or systemic high dose methotrexate.

If patients relapse, they can receive a course of salvage chemotherapy followed by high dose chemotherapy with autologous stem cell rescue (also known as an autograft).

For those too frail or co-morbid for high dose therapy, lower intensity chemotherapy regimens may be used to slow progression.

24
Q

Briefly describe R-CHOP regime

A

Can be used in high-grade non-Hodgkin’s lymphoma.

As R-CVP but with the addition of doxorubicin, an anthracycline.

R-CVP: rituximab, an anti-CD20 monoclonal antibody, combined with cyclophosphamide, vincristine and prednisolone.

25
Q

What are the complications of non-Hodgkin’s lymphoma

A
  • Spinal cord compression and cauda equina syndrome
  • Transformation to high grade lymphoma (‘Richter’s Transformation’)
    • For low grade lymphoma
  • Tumour lysis syndrome
  • Hypercalcaemia
  • Hypogammaglobulinaemia
  • Hyperviscosity syndrome
  • Paraneoplastic autoimmune conditions
    • Autoimmune haemolytic anaemia, ITP and neuropathy
  • Neutropenic sepsis
26
Q

Briefly describe the prognosis of non-Hodgkin’s lymphoma

Note: low and high grade

A

Low-grade non-Hodgkin’s lymphoma has a better prognosis. Prognosis for 5 year survival for treated patients is around 30%.

High-grade non-Hodgkin’s lymphoma has a worse prognosis but a higher cure rate. Prognosis for 5 year survival for treated patients is around 50%.