Initial management of follicular lymphoma (FL) in adults

CT: computed tomography; FDG: 18F-fluorodeoxyglucose; FL: follicular lymphoma; O-CHOP: obinutuzumab, cyclophosphamide, doxorubicin, vincristine, prednisone; O-CVP: obinutuzumab, cyclophosphamide, vincristine, prednisone; PET: positron emission tomography; R-CHOP: rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone.

* Baseline imaging can use whole-body FDG PET/CT or contrast-enhanced CT of diagnostic quality. FDG PET/CT is strongly preferred for patients with suspected stage 1 disease being evaluated for radiation therapy. Unilateral bone marrow biopsy is performed to rule out bone marrow involvement in patients with suspected stage 1 disease. We generally do not perform a bone marrow biopsy in patients with more advanced disease.

¶ In the small subset of patients with coexisting hepatitis C virus infection, treatment directed at hepatitis C may result in regression of FL.

Δ Single-agent rituximab and initial observation ("watch and wait") are equally acceptable alternatives; the choice between these depends on patient values and preferences. When compared with watchful waiting, single-agent rituximab delays progression and postpones cytotoxic chemotherapy and may reduce anxiety, although without impacting overall survival. Rituximab imposes risks associated with immunosuppression. In the small subset of patients with coexisting hepatitis C virus infection, treatment directed at hepatitis C may result in regression of FL.

◊ For patients with stage 2 FL, we prefer a management approach similar to that used for stage 3 or 4 FL. Other clinicians offer radiation therapy to a subset of these patients in which the disease can be encompassed by a reasonable radiation field (eg, stage 2 disease with groin and ipsilateral iliac involvement).

§ Histologic transformation should be suspected if there is a rapid progression of lymphadenopathy (eg, lymph nodes enlarging over weeks to months), infiltration of extranodal sites, the development of systemic symptoms (eg, fever, weight loss, drenching night sweats), or an elevated serum lactate dehydrogenase.

¥ Bendamustine plus rituximab (BR) and lenalidomide plus rituximab (R²) appear to have similar efficacy but differ in their toxicities, administration burdens, and implications for subsequent therapy. Single-agent rituximab is an acceptable initial treatment for patients with comorbid conditions that make them poor candidates for chemoimmunotherapy and for those with a low tumor burden and/or disease progressing slowly over years.

‡ Biopsy should aim to sample a lymph node with the highest activity on FDG PET.

† For younger, fit patients, we prefer R-CHOP or O-CHOP. For older, frail patients or those with cardiac disease, we prefer O-CVP.