Hodgkin lymphoma

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Hodgkin lymphoma (HL) is a distinctive group of B-cell lymphoid neoplasms that are characterized by the presence of a special cell type called Reed-Sternberg cell (RS cells). It’s also characterised by the fact that the tumour consists of mostly inflammatory, responsive cells, and just a few cancerous cells. For this reason, HL has sometimes been called Hodgkin disease rather than lymphoma.

There’s a bimodal incidence peak, affecting either young adults (20 – 30) or elderly (50+). Hodgkin lymphoma is highly treatable and curable, even when disseminated.

Etiology

Several risk factors are known:

  • EBV infection (in 50%+ of cases)
  • Immunodeficiency
  • Autoimmune disease

Classification

There are five subtypes of HL, four of which are considered “classic Hodgkin lymphoma”:

  • Classic Hodgkin lymphoma (95% of cases)
    • Nodular sclerosis HL – most frequent subtype
    • Mixed cellularity HL
    • Lymphocyte rich HL
    • Lymphocyte depletion HL – least frequent subtype
  • Nodular lymphocyte predominant HL (5% of cases)

Clinical features

It presents with painless cervical or mediastinal lymphadenomegaly. “B symptoms” may also be present. The following findings are rare but very specific for HL:

  • Pel-Ebstein fever – an intermittent high fever which has an abrupt onset and resolution and lasts 1 – 2 weeks at a time.
  • Pain in lymph nodes when drinking alcohol

Hodgkin lymphoma causes secondary T-cell immune deficiency, which increases the risk for viral and fungal infections.

Diagnosis and evaluation

Lymph node biopsy showing Reed-Sternberg cells is diagnostic.

Treatment

The basis of treatment is chemotherapy with or without involved field radiotherapy (IFRT). The most frequently used chemotherapy regimen is ABVD (Adriamycin, bleomycin, vinblastine, and dacarbazine).