Follicular lymphomas
Section snippets
Definition, incidence and risk factors
Follicular lymphomas are defined as a group of malignancies composed of follicle center cells, usually a mixture of centrocytes (cleaved cells) and centroblasts (large non-cleaved cells). Diffuse areas may be present and, in fact, may even predominate, but follicles exist. This group of lymphomas includes many cases formerly recognized as centroblastic/centrocytic follicular or follicular centroblastic according to the Kiel classification [1], [2] and follicular, small cleaved, mixed or large
Morphology
Follicular lymphomas are characterized by at least partially follicular growth pattern, but diffuse areas may be present [2]. Sclerosis is common in diffuse areas. Centrocytes typically predominate; centroblasts are usually in the minority, but by definition are always present. Rare lymphomas with a follicular pattern consist almost entirely of centroblasts; because the follicular pattern implies a germinal center origin, these cases have been included in the category of follicular center cells
Clinical presentation and natural history
Although not uncommon in the third and fourth decades, follicular lymphomas occur most commonly in middle-aged patients and elderly [20], [21]. Every anatomic district may be involved, but the most common presentation is multiple lymphadenopathy, with or without abdominal or mediastinal masses, splenomegaly, hepatomegaly, and marrow involvement. Leukemic phase is less common than for other indolent lymphomas, and extranodal sites are usually involved with e lower frequency respect to diffuse
Staging procedures
Complete staging work-up for follicular lymphomas is the same that routinely used for other NHL. It includes an accurate physical examination, complete hematological and biochemical exams, total-body computerized tomography, and bone marrow aspirate and biopsy. Bone marrow assessment in follicular lymphoma should follow the general statements for all NHL. Abdominal staging, with evaluation of potential hepatic or splenic involvement in follicular lymphomas should follow the general statements
Prognostic factors
Several groups have described prognostic factors for patients with advanced follicular lymphoma [36], [37], [38]. In the Groupe d’Etude des Lymphomes Folliculaires schema, patients with any one of the following features are considered to have a high tumor burden: systemic symptoms, elevated LDH serum levels, bulky lesion >7 cm, effusion, three or more Ann Arbor sites each 3 cm or greater, circulating lymphoma cells, cytopenias, and splenomegaly [39], [40]. The International Prognostic Index for
“Watch and wait” policy
Treatment can be safely deferred without disadvantage on survival for patients with follicular lymphoma in stages III–IV disease [24], provided that none of the following features occurs: systemic symptoms, high tumor burden, extranodal disease, cytopenia due to marrow involvement, spleen involvement, leukemic phase, serous effusion, high LDH levels. This “watch and wait” policy was evaluated by three trials that randomized to either chemotherapy or watchful waiting (WW) strategy patients with
Umberto Vitolo is chief of the Chemoimmunotherapy Lymphoma Section at the Department of Onco-hematology, S. Giovanni Battista Hospital, Torino Italy.
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Cited by (39)
Follicular lymphoma grade 3: Review and updates
2014, Clinical Lymphoma, Myeloma and LeukemiaCitation Excerpt :It presents as advanced disease in 80% to 85% of FL Grade 1; 70% to 75% in Grade 2; and 65% to 70% of Grade 3. Systemic symptoms are observed 30% of Grade 3 FL compared with 20% in Grade 1 and 2 FL.2 The Follicular Lymphoma International Prognostic Index (FLIPI) is a prognostic scoring system based on age, Ann Arbor stage, number of nodal sites involved, hemoglobin levels, and serum lactate dehydrogenase levels.36
Oral follicular lymphomas. A short report of 8 cases with assessment of the IGH/BCL2 gene fusion with fluorescence in situ hybridization
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2011, Clinical Lymphoma, Myeloma and LeukemiaCitation Excerpt :They are characterized by a slow proliferation, but they are almost incurable, with frequent relapses. A careful analysis of biological and clinical prognostic features is mandatory and it is helpful for a better management of the disease.3 As for the treatment of Waldenstrom macroglobulinemia, many therapeutic approaches are available for low-grade lymphomas: watchful waiting, chemotherapy, monoclonal antibodies alone or in association with chemotherapy with or without maintenance treatment, high-dose chemotherapy, and autologous stem cell transplantation.4
A rare case of primary conjunctival Follicular Lymphoma grade 3B
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Umberto Vitolo is chief of the Chemoimmunotherapy Lymphoma Section at the Department of Onco-hematology, S. Giovanni Battista Hospital, Torino Italy.
Andrés J.M. Ferreri is coordinator of the Lymphoma Unit and vice director of the Medical Oncology Unit, San Raffaele Scientific Institute, Milan, Italy.
Silvia Montoto, trained as a haematologist at the Haematology Department of Hospital Clinic in Barcelona, Spain, is currently a senior lecturer in medical oncology at the Medical Oncology Department, St. Bartholomew's Hospital, London.