Overview

Non-Hodgkin’s lymphoma (NHL) encompasses all cancers of the lymph nodes except for Hodgkin’s lymphoma. It can be classed as:

  • Low-grade – relatively good prognosis but not curable if advanced
  • High-grade – onset is faster and prognosis poorer but many patients can be cured with intensive chemotherapy

This section will cover a few of the large number of types of NHL.

Epidemiology

  • NHL is 5 times more common than Hodgkin’s lymphoma (HL)
  • DLBCL and follicular lymphomas are the most common
  • Peak incidence is seen in >50 years 

Types

High-grade B-cell neoplasms:

  • Diffuse large B-cell lymphoma (DLBCL)
  • Burkitt’s lymphoma
    • Associated with the Epstein-Barr virus (EBV)
  • Primary central nervous system lymphomas (PCNSLs)

Low-grade B-cell neoplasms:

  • Follicular lymphomas
  • Mucosa-associated lymphoid tissue lymphoma (MALT)
    • Associated with H. pylori infection
  • Waldenström’s macroglobulinaemia

High-grade T-cell neoplasms:

  • Enteropathy-type T-cell lymphoma

Risk Factors

  • >50 years of age
  • Male sex
  • Epstein-Barr virus – associated with Burkitt’s lymphoma and HL itself
  • Helicobacter pylori – associated with gastric MALT
  • Coeliac disease – associated with enteropathy-type T-cell lymphoma
  • HIV – associated with PCNSLs
  • Sjogren’s syndrome – significantly increased risk of NHL

Presentation

Patients most commonly present with painless cervical and/or supraclavicular lymphadenopathy. Features are:

  • Painless cervical and/or supraclavicular lymphadenopathy
    • The lymph nodes may feel rubbery and lymphadenopathy tends to be asymmetrical
    • Drinking alcohol does not induce pain in areas where there is lymphadenopathy in NHL
  • B-symptoms – these tend to occur later in NHL compared to HL. They are associated with a poorer prognosis:
    • Fevers – these usually cyclically increase and then decrease over 1-2 weeks
    • Night sweats
    • Weight loss
  • Splenomegaly
  • Hepatomegaly
  • Extra-nodal involvement – more common in NHL compared to HL:

Differential Diagnoses

Hodgkin’s lymphoma (HL)

  • In HL, alcohol can induce pain in the areas where lymphadenopathy is present, in NHL this does not happen
  • HL has pruritus, which is less common in NHL
  • B-symptoms tend to arise earlier in HL
  • Extranodal involvement is more common in NHL

Infectious mononucleosis

  • The enlarged lymph nodes are tender
  • A sore throat is present
  • Patients given penicillins usually develop a maculopapular pruritic rash

Referral

Adult patients should have a suspected cancer pathway referral (within 2 weeks) for Hodgkin’s lymphoma if they have unexplained lymphadenopathy or splenomegaly with associated symptoms which may be:

  • Fever
  • Night sweats
  • Weight loss
  • Shortness of breath
  • Pruritus

Children and young people should have a very urgent referral (within 48 hours) for specialist assessment.

Investigations

Overview

  • Full blood count (FBC) and white cell differential:
    • Bone marrow involvement may lead to pancytopenia
    • Some subtypes of NHL can cause lymphocytosis
  • Blood film:
    • May reveal abnormal cells associated with different types of NHL
  • Lymph node biopsy
    • Diagnostic test

Staging

HL can be staged using the Ann Arbor staging system which consists of a numbered stage from I-IV and ‘A’ if no B-symptoms are present and ‘B’ if B-symptoms are present:

  • Stage I: single lymph node affected
  • Stage II: ≥2 lymph nodes on the same side of the diaphragm are affected
  • Stage III: lymph nodes on both sides of the diaphragm are affected
  • Stage IV: disseminated and spread beyond lymph nodes

The ‘A’ or ‘B’ is added as well:

  • A – no systemic symptoms
  • B – B-symptoms are present (e.g. fever, night sweats, weight loss) 

Management

Overview

Patients are generally managed by an oncology multidisciplinary team through combined therapies involving chemotherapy, radiotherapy, stem cell transplants etc.

Complications

  • Secondary tumours
  • Radiotherapy and chemotherapy-induced complications e.g. development of acute myeloid leukaemia (AML)
  • Bone marrow infiltration and pancytopenia

Prognosis

  • Low-grade NHL has a relatively good prognosis but is usually not curable when advanced
  • High-grade NHL is more aggressive but more responsive to chemotherapy
  • Most cases of relapse happen within the first two years of treatment

Author