Overview

Hodgkin’s lymphoma (HL) is a cancer of the lymphatic system characterised by the presence of Reed-Sternberg cells. These are cells that are either nucleated or have a bilobed nucleus with eosinophilic inclusion-like nucleoli. They have an ‘owl’s eye’ appearance.

Epidemiology

  • There are peaks in incidence in patients aged in their 20-30s and >70 years
  • The annual incidence in the UK is 2.7/100,000
  • Males are slightly more affected 

Classification

Classical Hodgkin’s lymphoma can be divided into four subtypes:

  • Nodular sclerosing – the most common type
  • Mixed cellularity
  • Lymphocyte-rich (or lymphocyte predominant) – carries the best prognosis
  • Lymphocyte-depleted – carries the poorest prognosis

Risk Factors

  • History of Epstein-Barr virus (EBV) infection
  • Family history
  • HIV
  • Immunodeficiency
  • Smoking

Example History

A 27-year-old man has a slowly enlarging, painless left neck mass. He has no recent infection or illness. He denies fevers, night sweats, or unintentional weight loss. He has no other medical conditions and no family history. There is no hepatosplenomegaly. He does not smoke and has stopped drinking due to the alcohol causing the mass to hurt.

Presentation

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

  • Painless lymphadenopathy – usually cervical and/or supraclavicular
    • Drinking alcohol can induce pain in areas where there is lymphadenopathy, although this is rare
    • The lymph nodes may feel rubbery on examination
  • B-symptoms – these are usually seen in advanced disease and in around 30% of patients. They are associated with a poorer prognosis:
    • Fevers:
      • These may cyclically increase and then decrease over 1-2 weeks, although this is rare
    • Night sweats
    • Weight loss
  • Generalised pruritus
  • Hepato- and/or splenomegaly are uncommon and usually seen in advanced disease

Differential Diagnoses

Non-Hodgkin’s lymphoma (NHL)

  • They can clinically be difficult to distinguish
  • Alcohol-induced pain at affected sites is not seen in NHL
  • Patients with NHL tend to be older
  • HL tends to spread from one lymph node chain to the next in a sequential manner

Infectious mononucleosis

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

Reactive lymphadenopathy

  • Enlarged lymph nodes occur following infection
  • Reactive lymphadenopathy tends to resolve within a few weeks
  • B-symptoms are not present. Persisting lymphadenopathy and/or B-symptoms should raise suspicion of lymphoma

Referral

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

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

Investigations

All patients

  • Full blood count (FBC) and white cell differential:
    • Haemoglobin: may be low due to bone marrow involvement
    • White cell count: high or low – depending on bone marrow involvement
    • Platelets: low – due to bone marrow involvement
  • Erythrocyte sedimentation rate (ESR):
    • May be elevated
  • Chest x-ray:
    • May show mediastinal mass
  • Immunophenotyping:
    • Can differentiate HL and NHL using flow cytometry– HL is usually CD30 positive and CD15 positive
  • Lymph node biopsy:
    • Shows Reed-Sternberg cells – these are cells that are either nucleated or have a bilobed nucleus with eosinophilic inclusion-like nucleoli. They have an ‘owl’s eye’ appearance

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)

Prognosis

  • With modern treatments, 80-90% of people achieve remission
  • Factors associated with a poorer prognosis are:
    • Increased age
    • Male sex
    • Increased extensiveness of disease
    • B-symptoms
    • Increased ESR
    • Hypoalbuminaemia

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