Lymphoma — Hodgkin and Non-Hodgkin

The answer first

Lymphoma is a clonal proliferation of lymphoid cells that presents most often as painless lymphadenopathy, a mediastinal mass, or an extranodal mass, frequently with the B symptoms of unexplained fever, drenching night sweats, and weight loss. The single most important diagnostic rule: the diagnosis is made on excisional lymph node biopsy, not fine-needle aspiration. Flow cytometry and immunohistochemistry on a whole node preserve architecture; FNA destroys it and misses lymphoma in a meaningful proportion of patients ^[6].

The second rule is a fork in the road that changes everything downstream: Hodgkin versus non-Hodgkin. Hodgkin lymphoma is defined by the Reed-Sternberg cell (a clonal B cell derived from the germinal centre) within an inflammatory background, spreads in a contiguous fashion, is exquisitely chemo-radiosensitive, and is among the most curable of all cancers — five-year survival over 85 per cent in developed countries. Non-Hodgkin lymphoma is heterogeneous: the clinical fork is indolent versus aggressive versus very aggressive, and the management fork is watch-and-wait versus urgent chemoimmunotherapy versus emergency cytoreduction with tumour lysis prophylaxis ^[6].

Three bedside rules that change outcome: ^[1]

1. Suspected Burkitt or lymphoblastic with high LDH? Start tumour lysis prophylaxis now. Burkitt lymphoma has the fastest doubling time of any human tumour (24 hours) and tumour lysis syndrome can develop spontaneously before chemotherapy is given. Aggressive hydration, rasburicase, and close electrolyte monitoring are started on suspicion ^[14].
2. Suspected primary CNS lymphoma? Hold the steroids. Corticosteroids lyse lymphoma cells and can make a stereotactic biopsy non-diagnostic. If you give dexamethasone to reduce cerebral oedema before biopsy, the diagnosis may be lost ^[15].
3. DLBCL with kidney, adrenal, testicular, paranasal sinus, or bone marrow involvement? Assess for CNS prophylaxis. CNS relapse in DLBCL is usually fatal. The CNS-IPI identifies patients who benefit from intrathecal or systemic CNS-penetrant prophylaxis ^[11].

For fit patients with advanced classical Hodgkin lymphoma, the modern backbone is ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine), increasingly modified by interim PET-adapted therapy (escalate if PET-positive after 2 cycles, de-escalate or omit bleomycin if PET-negative) and the newer frontline option of brentuximab vedotin plus AVD ^[3][4]. For DLBCL — the commonest aggressive NHL — the backbone is R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) given every 21 days for 6 cycles ^[8]. For relapsed DLBCL the paradigm has shifted to CAR-T cell therapy (axicabtagene ciloleucel, tisagenlecleucel) after platinum-based salvage, with curative intent in patients previously deemed incurable ^[17].

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References17ShowHide

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