Lymphoma + Waldenstrom's Flashcards

1
Q

What are lymphomas?

A

A group of cancer that affect the lymphocytes within the lymphatic system.

These cancerous cells proliferate within the lymph nodes and cause the lymph nodes to become abnormally enlarged leading to lymphadenopathy.

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2
Q

What is lymphadenopathy?

A

Enlargement of the lymph nodes

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3
Q

Aetiology of Hodgkin’s lymphoma?

A
  • Accounts for 1/5 lymphomas
  • Caused by the proliferation of lymphocytes.
  • Bimodal age distribution which peaks around ages 20 and 75 years old.
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4
Q

What are the risk factors for Hodgkin’s lymphoma?

A

Risk factors:

  • Immunosuppression e.g. HIV, inherited immunodeficiency states.
  • Autoimmune disorders e.g. rheumatoid arthritis, sarcoidosis.
  • EBV (epstein-barr virus)
  • Family history
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5
Q

What are the main types of non-hodgkin’s lymphoma?

A

Burkitt lymphoma: associated with EBV, malaria and HIV.

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

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

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6
Q

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

A

Risk factors:

  • Infections: HIV, EBV and H.pylori
  • 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

Clinical features of lymphoma?

A

Lymphadenopathy - the enlarged lymph node or nodes might be in the neck, axilla or inguinal region.
* Characteristically non-tender and fell “rubbery”.
* Some patients will experience pain in the lymph nodes when drinking alcohol.

“B” symptoms

Itch without rash

Symptoms due to involvement of other organs e.g. if lung became involved there may be breathlessness and cough.

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8
Q

What are “B” symptoms in lymphoma?

A

These are the systemic symptoms in lymphoma:

  • Fever
  • Night sweats
  • Weight loss
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9
Q

What blood tests can be done in lymphoma?

A

Lactate dehydrogenase

Certain blood results, notably elevated ESR and/or decreased haemoglobin, associated with poor prognoses in HL.

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10
Q

What is lactate dehydrogenase (LDH)?

A

A blood test often raised in Hodgkin’s lymphoma but is not specific and can be raised in other cancers and many non-cancerous diseases.

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11
Q

What is the key diagnostic test for lymphoma?

A

Lymph node biopsy

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12
Q

What is the key finding on lymph node biopsy for lymphoma?

A

The Reed-Sternberg cell is key finding from lymph node biopsy in Hodgkin’s lymphoma.

Characterised by abnormally large B cells that have multiple nuclei containing nucleoli within them.

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13
Q

Use of CT, MRI and PET scans in lymphoma?

A

Can be used for diagnosis and staging
- The Ann-Arbor system is the staging method used for HL, comprising 4 key stages (I-IV).

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14
Q

Management of Hodgkin lymphoma?

A

Multi-agent chemotherapy +/- radiotherapy

  • ABVD – adriamycin, bleomycin, vinblastine and dacarbazine.
  • Good cure rates particularly in younger patients.
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15
Q

In patients with HL, what is an option for patients not responding to chemo?

A

Immunotherapy/stem cell transplantation is an option for patients not responding to chemo.

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16
Q

In HL, what can bleomycin treatment cause?

A

Pneumonitis (inflammation of alveoli)

17
Q

What is a risk of multi-agent chemotherapy+/- radiotherapy?

A

Long term toxicity including:

  • Secondary cancers
  • Cardiovascular disease
  • Infertility (which occurs with more intensive treatment).
18
Q

What is involved in multi-agent chemotherapy +/- radiotherapy for non-hodgkin lymphoma?

A

R - CHOP

  • Rituximab
  • Cyclophosphamide
  • Hydroxydaunorubicin
  • Vincristine
  • Prednisolone
19
Q

What role can steroids play in lymphoma treatment?

A

Lymphocytes are normally sensitive to steroid therapy and some lymphomas are exquisitely sensitive.

Steroids are an integral part of multi-agent chemotherapy regimens for the routine treatment of lymphoma.

20
Q

Steroids can be used in emergency management of suspected lymphoma. Why is starting steroid therapy before a biopsy discouraged?

A

Starting steroid before biopsy can cause cell necrosis and distort cellular and tissue architecture to confuse the diagnosis.

21
Q

What is Waldenstrom’s macroglobulinaemia?

A

Low-grade lymphoma with monoclonal plasmacytoid lymphocytes that secrete monoclonal IgM found in the bone marrow and other organ.

22
Q

Aetiology of Waldenstrom’s macroglobulinaemia?

A

Exact cause is unknown.

Personal/family history of autoimmune, inflammatory or infective disorders is a risk factor. Specific examples include: Sjogren’s syndrome and autoimmune haemolytic anaemia.

23
Q

Pathophysiology of WM?

A

Lymphoplasmacytoid neoplasm
- Clonal disorder of cells intermediate between a lymphocyte and plasma cell.
- Characteristic IgM paraprotein.

24
Q

Tumour effects of WM?

A

Lymphadenopathy

Splenomegaly

Marrow failure

25
Q

Paraprotein effects of WM?

A

Hyperviscosity, hyperviscosity syndrome - caused by IgM antibody being pentameric.
- Fatigue
- Bleeding
- Cardiac failure

Neuropathy

26
Q

B symptoms of WM?

A

Night sweats

Weight loss

27
Q

Investigations for WM?

A

Initial work up includes FBC, plasma viscosity, serum protein electrophoresis and immunofixation and quantification of IgM paraprotein by densitometry.

Bone marrow assessment - marrow infiltration by LPL.

28
Q

Primary treatment management of WM?

A

Includes: bendamustine-rituximab (BR) or Dex/Cyclo/Ritux (DCR) which are the 2 most frequently used options in the UK.

29
Q

Supportive care for WM?

A
  • Aciclovir prophylaxis if on chemo
  • Co-trimoxazole if on BTK inhibitors
  • Seasonal influenza and COVID-19 vaccines as well as pneumococcal vaccines.
30
Q

What are BTK inhibitors?

A

A type of drug that work to treat cancers caused by defective B cells, such as chronic lymphocytic leukemia, B-cell lymphomas, and Waldenström macroglobulinemia.

31
Q

What is hyperviscosity syndrome?

A

Acondition that occurs when your blood becomes so thick that your body’s overall blood flow decreases.

Hyperviscosity can be caused by your blood cells changing shape or by an increase in serum proteins, red blood cells, white blood cells, or platelets.

32
Q

What is the ann-arbor staging system?

A

The staging system for lymphomas, both in Hodgkin’s lymphoma and non-Hodgkin lymphoma.

Consists of 4 grades.