Lymphoma

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)

Aetiology

Hodgkin lymphoma - accounts for 1/5 lymphomas

  • Caused by proliferation of lymphocytes
  • There is a bimodal age distribution with peaks around aged 20 and 75 years
  • Risk factors:
    • Immunosuppression e.g. HIV, inherited immunodeficiency states
    • Autoimmune disorders e.g. rheumatoid arthritis, sarcoidosis
    • EBV
    • Family history

Non-Hodgkin lymphona

  • A group of lymphomas, there are almost endless types of lymphoma
  • A few notible ones are:
    • Burkitt lymphoma - associated with Epstein-Barr virus, malaria and HIV
    • MALT lymphoma affects the mucosa-associated lymphoid tissue, usually around the stomach - associated with H. pylori infection
    • Diffuse large B cell lymphoma often presents as a rapidly growing painless mass in patients over 65 years
  • Risk factors:
    • Infections - HIV, EBV, H. pylori
    • Hepatitis B or C infection
    • Exposure to pesticides and a specific chemical called trichloroethylene used in several industrial processes
    • Family history

Clinical presentation

  • Lymphadenopathy - the enlarged lymph node or nodes might be in the neck, axilla, or inguinal region
    • Characteristically non-tender and feel ‘rubbery’
    • Some patients will experience pain in the lymph nodes when they drink alcohol
  • ‘B’ symptoms are the systemic symptoms of lymphoma - fever, night sweats, weight loss
  • Itch without rash
  • Symptoms due to involvement of other organs e.g. if lung became involved there may be breathlessness and cough

Investigations

  • Lactate dehydrogenase (LDH) is a blood test that is often raised in Hodgkin’s lymphoma but is not specific and can be raised in other cancers and many non-cancerous diseases
  • Certain blood results, notably elevated ESR and/or decreased haemoglobin, are associated with poor prognoses in HL
  • Lymph node biopsy is the key diagnostic test
    • The Reed-Sternberg cell is the key finding from lymph node biopsy in patients with Hodgkin’s lymphoma: abnormally large B cells that have multiple nuclei that have nucleoli inside them
notion image
  • CT, MRI and PET scans can be used for diagnosis and staging
    • The Ann-Arbor system is the staging method used for HL, comprising four key stages
       (I–IV)

Management

Hodgkin lymphoma

  • Multi-agent chemotherapy +/- radiotherapy
    • ABVD - adriamycin, bleomycin, vinblastine, dacarbazine
    • Bleomycin in treatment can cause pneumonitis
    • Good cure rates particularly in younger patients
    • Long term toxicity including secondary cancers, cardiovascular disease, infertility (with more intensive treatment)
  • Immunotherapy/stem cell transplantation is an option for patients not responding to chemo

Non-Hodgkin lymphoma

  • Multi-agent chemotherapy +/- radiotherapy
    • R-CHOP - rituximab, cyclophosphamide, hydroxydaunorubicin (doxorubicin), vincristine (Oncovin® ) and prednisolone
    • Risks of neutropenia, cardiotoxicity (also in HL)
  • Monoclonal antibody therapy + chemotherapy e.g. rituximab (CD20) in B cell NHL
  • High-grade NHL is potentially curable, low-grade is incurable but many patients may not need treatment
  • High-dose therapy with autologus stem cell rescue or CART therapy is an option for patients failing chemotherapy

Lymphoma and steroids

  • Lymphocytes are normally sensitive to steroid therapy and some lymphomas are exquisitely sensitive
  • Steroids are an integral part of multiagent chemotherapy regimens for the routine treatment of lymphoma
  • Steroids can be used in the emergency management of suspected lymphoma but starting steroid before biopsy can cause cell necrosis and distort cellular and tissue architecture to confuse the diagnosis