A 22-year-old man is noted to have a swelling in the right supraclavicular region. He has complained of cough some 3 months ago and was diagnosed to have a chest infection which was appropriately treated with antibiotics. He has noticed a 3-kg weight loss over the past 6 months. He has otherwise been in generally good health. There is no other relevant history. He is a non-smoker.
On examination, he has a palpable lymph node in the right supraclavicular region measuring 1.5 cm. He has multiple scratch marks over his upper thorax and back and gives a history of pruritus.
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(a) What is the differential diagnosis?
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Correct answer: This man has a significantly enlarged lymph node and requires biopsy. There is no local explanation for the lymphadenopathy. Biopsy showed the appearances of nodular sclerosing Hodgkin disease.
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(b) What further investigations are required?
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Correct answer: Further investigation must include accurate staging of the tumour. In addition to clinical evaluation, he should undergo CT scan of chest and abdomen to look for lymphadenopathy and to assess whether or not other non-lymphoid organs are infiltrated. He should also undergo bone marrow aspirate and trephine biopsy. A PET scan should also be undertaken at presentation. In the current case, his CT scan showed extensive intra-abdominal and intrathoracic lymphadenopathy with evidence of infiltration of the lungs. PET scan confirmed widespread lymphadenopathy and active uptake was demonstrated in lung and liver. He therefore has Stage 4B Hodgkin disease.
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(c) How would you manage this patient?
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Correct answer: He should be referred for expert management and his care should be discussed at a multidisciplinary meeting so that the histology and imaging results can be reviewed and a management plan is formulated by clinical haematologists and clinical oncologists.
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(d) What is the likely treatment?
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Correct answer:
He should be treated with combination chemotherapy. In most centres this would be in the form of ABVD (adriamycin, bleomycin, vinblastine, dacarbazine).
Sperm cryopreservation should be undertaken prior to commencement of chemotherapy.
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(e) How is the effectiveness of the treatment monitored?
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Correct answer:
It would be customary to repeat the PET scan after two courses and to change to more intensive therapy if the scan remained positive, but to continue to six cycles of ABVD if the scan is negative.
See Chapter 32for further details.
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(f) What are the long-term complications of the treatment?
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Correct answer:
Chemotherapy carries a risk of inducing infertility. There is a risk of second malignancy, particularly acute myeloid leukaemia; this risk is greater in patients who receive radiotherapy as well as chemotherapy and is greater with some chemotherapy regimes (e.g. those containing stem cell toxins including mustine, melphalan, BCNU and CCNU).
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(g) What is his prognosis?
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Correct answer:
Long-term prognosis in Hodgkin lymphoma is excellent. However, he has Stage 4 disease and long-term cure rate is approximately 75%; if he relapses, he could still be cured by salvage chemotherapy and stem cell transplant.
