Hodgkin Lymphoma

Hodgkin lymphoma is a B cell malignancy with Reed-Sternberg cells in an inflammatory background.

• Bimodal incidence (young adults 15 to 35 and over 55); incidence 2.5 per 100,000.

• Subtypes: nodular sclerosis (70%), mixed cellularity, lymphocyte rich, lymphocyte depleted, nodular lymphocyte predominant.

• EBV association in 30 to 50% (higher in HIV, developing regions).

• Ann Arbor staging with B symptoms (fever, night sweats, weight loss over 10%); cure rates 80 to 90%.

• Late effects: second cancers (breast, lung), cardiovascular disease, hypothyroidism, infertility.

• Painless cervical or supraclavicular lymphadenopathy, mediastinal mass with cough or dyspnoea, B symptoms, pruritus, alcohol induced node pain.

• Splenomegaly, hepatomegaly less commonly.

• Cytopenias, hypercalcaemia.

• Excisional lymph node biopsy with CD30+, CD15+, PAX5 weak, CD20 variable Reed-Sternberg cells.

• PET-CT staging (Deauville score), blood count, ESR, LDH, albumin, liver and renal function, HIV, hepatitis B and C.

• Echocardiography, pulmonary function tests (DLCO), pregnancy test, fertility counselling and sperm banking.

• Bone marrow biopsy not required if PET-CT.

• Early stage favourable: 2 cycles ABVD (doxorubicin 25 mg/m2, bleomycin 10 units/m2, vinblastine 6 mg/m2, dacarbazine 375 mg/m2 on days 1 and 15) plus 20 Gy involved site radiotherapy.

• Early unfavourable: 4 cycles ABVD with 30 Gy radiotherapy; PET-adapted approaches omit radiotherapy if PET negative.

• Advanced stage (III to IV): brentuximab vedotin 1.2 mg/kg with AVD (doxorubicin, vinblastine, dacarbazine) for 6 cycles; or BEACOPP escalated in selected high risk; PET-adapted de-escalation; nivolumab-AVD under evaluation.

• Relapse: salvage chemotherapy (ICE, DHAP) followed by high dose chemotherapy with autologous stem cell transplant, brentuximab maintenance after transplant; pembrolizumab 200 mg every 3 weeks or nivolumab for post-transplant relapse.

• Nodular lymphocyte predominant: observation or rituximab, radiotherapy for stage IA.

• Supportive: antiemetics, growth factors, G-CSF, fertility preservation, vaccination, screening for second cancers (breast MRI from 8 years after chest radiation), cardiovascular follow up.