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Follicular Lymphoma is an indolent, neoplastic disorder of germinal center-derived B lymphocytes that afflicts approximately 14,000 people in the United States each year. It typically presents as a disseminated disorder with painless, diffuse lymphadenopathy and marrow infiltration, often with associated hepatosplenomegaly and circulating lymphoma cells in the blood. A characteristic translocation, t(14;18), is found in the cells of 85 percent of patients, which deregulates BCL2 protein expression and inhibits apoptosis of affected B cells. The cells typically express monoclonal surface immunoglobulin, CD10, CD19, CD20, CD22, CD45, and CD79a on their cell surface, but not CD5 or CD23. Patients are often asymptomatic at the time of presentation, and may live for many years in good health without therapy. On the other hand, most patients eventually develop progressive lymphadenopathy, causing symptoms mandating intervention. Many treatment regimens are effective at inducing remissions, including single-agent rituximab or chlorambucil; or several multidrug programs, such as rituximab, cyclophosphamide, vincristine, and prednisone (R-CVP); rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP); rituximab, fludarabine, mitoxantrone, and dexamethasone (R-FND); and radiolabeled monoclonal antibodies. None of these therapies, however, is considered curative and most patients eventually relapse with recurrent disease. The role of autologous and allogeneic hematopoietic cell transplantation is controversial. Histologic transformation to aggressive lymphoma occurs in 30 to 40 percent of patients, usually leading to death within 1 to 2 years of transformation.

Acronyms and Abbreviations

Abbreviations and acronyms that appear in this chapter include: ADCC, antibody-dependent cellular cytotoxicity; AML, acute myelocytic leukemia; CDC, complement-dependent cytotoxicity; CHOP, cyclophosphamide, doxorubicin, vincristine, prednisone; CR, complete remission; CVP, cyclophosphamide, vincristine, prednisone; FCM, fludarabine, cyclophosphamide, mitoxantrone; FDG, fluoro-2-deoxy-glucose; FL, follicular lymphoma; FND, fludarabine, mitoxantrone (Novantrone), dexamethasone; GELF, Groupe d’Etudes des Lymphomes Folliculaires; GM-CSF, granulocyte-macrophage colony-stimulating factor; Gy, gray; HLA, histocompatibility locus antigen; IFN, interferon; Ig, immunoglobulin; IPI, international prognostic index; KLH, keyhole limpet hemocyanin; LDH, lactate dehydrogenase; NHL, non-Hodgkin lymphoma; ORR, overall response rate; OS, overall survival; PACE, cisplatin, doxorubicin, cyclophosphamide, etoposide; PCR, polymerase chain reaction; PET, positron emission tomography; PFS, progression-free survival; PR, partial remission; ProMACE/MOPP, prednisone, methotrexate, doxorubicin, cyclophosphamide, etoposide, mechlorethamine, vincristine, procarbazine, prednisone; R-CHOP, rituximab plus CHOP; R-CVP, rituximab plus CVP; REAL, revised European-American lymphoma; RIT, radioimmunotherapy; WHO, World Health Organization.

Follicular lymphoma (FL) is an indolent lymphoid neoplasm that is derived from mutated germinal center B cells and exhibits a nodular or follicular histologic pattern. It is typically composed of a mixture of small, cleaved follicle center cells (centrocytes) and large noncleaved follicle center cells (centroblasts). The disease has masqueraded under multiple previous monikers, including “nodular lymphoma” in the Rappaport classification, and “follicle center cell lymphoma” in the Working Formulation.1 The current World Health Organization (WHO) classification proposes the terms follicular lymphoma, grades 1, 2, and 3, to differentiate cases based on the numbers of centroblasts per high-power microscopic field (see “Lymph Node Morphology and Lymphocyte Immunophenotype” below).1

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