Hodgkin's Lymphoma

 

• Background

  

  
  

  Definition

  • Lymphoma is a malignant proliferation of mature lymphocytes that accumulate in lymph nodes ± other tissue, often as a solid tumour. Differs from leukaemia, which arises in the bone marrow and is present in the blood.
  • Hodgkin's lymphoma represents 20% of all lymphomas, and is characterised by the presence of 'mirror-image' binucleated Reed-Sternberg cells on light microscopy.
  • Hodgkin's lymphoma is a B-cell cancer.

  Sub-types

  • Common: nodular sclerosis (70%), usually in young patients, and mixed cellularity (25%), usually in old patients. Both have a good prognosis.
  • Rare (5%): lymphocyte-rich (excellent prognosis) or lymphocyte-depleted (bad prognosis).

  Epidemiology

  • Lifetime risk: 1/400.
  • Occurs any age but peaks at 15-30 and 75-80.
  • Commoner in men.

• Signs and symptoms

  Lymphadenopathy:

  • Painless, rubbery, nodes, usually cervical (⅔), axillary, or inguinal.
  • May be adherent to each other and move together ('matted').
  • Increase and decrease spontaneously.
  • Painful on alcohol consumption.
  • Mediastinal lymph nodes can cause SOB, dry cough, and SVC obstruction.

  B symptoms (present in 25%):

  • Weight loss.
  • Fever. The classical Pel-Ebstein cyclical fever every 2-4 weeks is rare.
  • Night sweats.
  • Lethargy
  • Pruritus

  Other features:

  • Anaemia of chronic disease.
  • Hepatosplenomegaly in advanced disease.

• Risk factors

  • Family history.
  • EBV infection.
  • ↑Socioeconomic status.

• Investigations

  Bloods:

  • FBC: pancytopaenia if there is bone marrow infiltration.
  • Blood film
  • ↑ESR
  • LFT, U+E.
  • Protein electrophoresis may show paraproteins, though these are commoner in myeloma.

  Biopsy for diagnosis:

  • Lymph node excision.
  • Bone marrow aspirate and biopsy only if there is altered FBC or B symptoms.
  • Analysis: microscopy (Reed-Sternberg cells), immunohistochemistry (CD30 ± CD15 +ve, EBV in 50% of malignant cells), flow cytometry (CD30 ± CD15 +ve), cytogenetics.

  Imaging for staging:

  • CXR for mediastinal disease (widening).
  • Staging: contrast CT head-neck-abdo or PET-CT.

• Ann Arbor staging system

  Used for both Hodgkin's and non-Hodgkin's. Comprises 2 components.

  By location:

  1. 1 lymph node (LN) area.
  2. ≥2 LN areas on 1 side of the diaphragm.
  3. LN areas on 2 sides of the diaphragm.
  4. Beyond LN e.g. liver, bone marrow.

  By presence of systemic symptoms:

  • A: no systemic symptoms, except pruritus.
  • B: systemic symptoms.

• Management

  • Chemotherapy plus radiotherapy.
  • Consider chemo alone if not bulky, or radiotherapy alone for early lymphocyte-rich disease.
  • Common chemo regimens: ABVD (doxorubicin [Adriamycin], Bleomycin, Vinablastine, Dacarbazine), BEACOPP (Bleomycin, Etoposide, doxorubicin [Adriamycin], Cyclophosphamide, Vinicristine, Procarbazine, Prednisolone).
  • Sperm bank pre-chemo for men.
  • Autologous stem cell transplantation – in which the patient's bone marrow is removed, chemo given, then the marrow returned – is used in resistant and relapsed disease.
  • Pneumococcal and flu vaccine.

• Complications and prognosis

  Treatment complications:

  • Radio- or chemo-therapy: AML, NHL, infertility.
  • Radiotherapy damage to neighbouring tissue: secondary cancer (especially lung, breast), IHD, hypothyroidism, pulmonary fibrosis.
  • Chemotherapy-specific: all the usual chemo side effects, including immunosuppression, nausea, and hair loss, plus pulmonary fibrosis from bleomycin.

  Oncological emergencies:

  • Infection
  • SVC obstruction.

  Prognosis:

  • 5 year survival: stage 1-2 90%, stage 3-4 75%.
  • Poor prognosis: B symptoms, ↑ESR, lymphocyte-depleted type.