Follicular Large Cell Lymphoma: An Aggressive Lymphoma That Often Presents With Favorable Prognostic Features

Rodríguez, José A.; McLaughlin, Peter; Hagemeister, Fredrick B.; Fayad, Luis; Rodriguez, Maria Alma; Santiago, Marta; Hess, Mark A.; Romaguera, Jorge; Cabanillas, Fernando

doi:10.1182/blood.v93.7.2202

Cited by 59 publications

(25 citation statements)

References 25 publications

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“…Since many studies likely include both grade IIIa and IIIb, this heterogeneity may affect interpretation of the outcomes . Although the follicular architecture is intact, the clinical presentation, behavior, and outcome with treatment in many patients with FL grade IIIb more closely approximates that of diffuse large B‐cell lymphoma (DLBCL) . In contrast to DLBCL, the relapse rate of FL grade 3b is in some series is higher, but survival is longer …”

Section: Prognosismentioning

confidence: 99%

Follicular lymphoma: 2020 update on diagnosis and management

2019

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Disease overview: Follicular lymphoma (FL) is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells. Follicular lymphoma is characterized by diffuse lymphadenopathy, bone marrow involvement, and splenomegaly. Extranodal involvement is less common. Cytopenias are relatively common but constitutional symptoms of fever, night sweats, and weight loss are uncommon in the absence of transformation to diffuse large B cell lymphoma. Diagnosis:The diagnosis is based on histology from a biopsy of a lymph node or other affected tissue. Incisional biopsy is preferred over needle biopsies in order to give adequate tissue to assign grade and assess for transformation. Immunohistochemical staining is positive in virtually all cases for cell surface CD19, CD20, CD10 and monoclonal immunoglobulin, as well as cytoplasmic expression of bcl-2 protein.The overwhelming majority of cases have the characteristic t(14;18) translocation involving the IgH/bcl-2 genes.Risk stratification: The Follicular Lymphoma International Prognostic Index (FLIPI) uses five independent predictors of inferior survival: age > 60 years, hemoglobin <12 g/dL, serum LDH > normal, Ann Arbor stage III/IV, number of involved nodal areas >4. The presence of 0-1, 2, and ≥ 3 adverse factors defines low, intermediate, and high-risk disease. There are other clinical prognostic models but the FLIPI remains the most common. Other factors such as time to relapse of less than 2 years from chemoimmunotherapy and specific gene mutations may also be useful for prognosis.Regardless of the prognostic model used, modern therapies have demonstrably improved prognosis.Risk-adapted therapy: Observation continues to be appropriate for asymptomatic patients with low bulk disease and no cytopenias. There is no overall survival advantage for early treatment with either chemotherapy or single agent rituximab. For patients needing therapy, most patients are treated with chemoimmunotherapy, which has improved response rates, duration of response and overall survival (OS).Randomized studies have shown additional benefit for maintenance rituximab.Lenalidomide was non-inferior to chemoimmunotherapy in a randomized front-line study and, when combined with rituximab, was superior to rituximab alone in relapsed FL. Kinase inhibitors, other immunotherapies, and stem cell transplantation (SCT) are also considered for recurrent disease.

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Section: Prognosismentioning

confidence: 99%

Follicular lymphoma: 2020 update on diagnosis and management

2019

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“…Since many studies likely include both Grade IIIa and IIIb, this heterogeneity may affect interpretation of the outcomes. Although the follicular architecture is intact, the clinical presentation, behavior, and outcome with treatment in many patients with FL Grade IIIb more closely approximates that of diffuse large B‐cell lymphoma (DLBCL) In contrast to DLBCL, the relapse rate of FL Grade 3b is in some series is higher, but survival is longer . A recent series suggested similar outcome of Grades IIIa and IIIb cases, and no benefit for the inclusion of anthracyclines in the treatment regimen .…”

Section: Risk Stratificationmentioning

confidence: 99%

Follicular lymphoma: 2014 update on diagnosis and management

Freedman

2014

American J Hematol

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Disease overview Follicular lymphoma is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells. Follicular lymphoma (FL) is characterized by diffuse lymphoadenopathy, bone marrow involvement, splenomegaly, and less commonly other extranodal sites of involvement. In general cytopenias can occur but constitutional symptoms of fever, nightsweats, and weight loss are uncommon. Diagnosis Diagnosis is based on histology of preferably a biopsy of a lymph node. Immunohistochemical staining is positive in virtually all cases for cell surface CD19, CD20, CD10, and monoclonal immunoglobulin, as well as cytoplasmic expression of bcl‐2 protein. The overwhelming majority of cases have the characteristic t(14;18) translocation involving the IgH/bcl‐2 genes. Risk stratification The Follicular Lymphoma International Prognostic Index prognostic model for FL uses five independent predictors of inferior survival: age >60 years, hemoglobin <12 g/dL, serum LDH > normal, Ann Arbor stage III/IV, number of involved nodal areas > 4. The presence of 0, 1, 2, and ≥ 3 adverse factors defines low, intermediate, and high‐risk disease. With the use of more modern therapies, outcomes have improved. Risk‐adapted therapy Observation continues to be adequate for asymptomatic patients with low bulk disease and no cytopenias. For patients needing therapy, most patients are treated with chemotherapy plus rituximab, which has improved response rates, duration of response and overall survival. Randomized studies have shown additional benefit for maintenance rituximab both following chemotherapy‐rituximab and single agent rituximab. Experimental therapies as well as stem cell transplantation (SCT) are considered for recurrent disease. Am. J. Hematol. 89:430–436, 2014. © 2014 Wiley Periodicals, Inc.

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“…Since many studies likely include both grade IIIa and IIIb, this heterogeneity may affect interpretation of the outcomes. Although the follicular architecture is intact, the clinical presentation, behavior, and outcome with treatment in many patients with FL grade IIIb more closely approximates that of diffuse large B‐cell lymphoma (DLBCL) . In contrast to DLBCL, the relapse rate of FL grade 3b is in some series is higher, but survival is longer .…”

Section: Prognosismentioning

confidence: 99%

Follicular lymphoma: 2015 update on diagnosis and management

Freedman

2015

American J Hematol

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Disease overview: Follicular lymphoma is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells. Follicular lymphoma (FL) is characterized by diffuse lymphoadenopathy, bone marrow involvement, splenomegaly, and less commonly other extranodal sites of involvement. In general, cytopenias can occur but constitutional symptoms of fever, night sweats, and weight loss are uncommon. Diagnosis: Diagnosis is based on histology of preferably a biopsy of a lymph node. Immunohistochemical staining is positive in virtually all cases for cell surface CD19, CD20, CD10, and monoclonal immunoglobulin, as well as cytoplasmic expression of bcl‐2 protein. The overwhelming majority of cases have the characteristic t(14;18) translocation involving the IgH/bcl‐2 genes. Risk stratification: The Follicular Lymphoma International Prognostic Index prognostic model for FL uses five independent predictors of inferior survival: age >60 years, hemoglobin <12 g/dL, serum LDH > normal, Ann Arbor stage III/IV, number of involved nodal areas > 4. The presence of 0, 1, 2, and ≥ 3 adverse factors defines low, intermediate, and high‐risk disease. With the use of more modern therapies, outcomes have improved. Risk‐adapted therapy: Observation continues to be adequate for asymptomatic patients with low bulk disease and no cytopenias, with no survival advantage for early treatment with either chemotherapy or rituximab alone. For patients needing therapy, most patients are treated with chemotherapy plus rituximab, which has improved response rates, duration of response and overall survival. Randomized studies have shown additional benefit for maintenance rituximab both following chemotherapy‐rituximab and single agent rituximab. Experimental therapies as well as stem cell transplantation (SCT) are considered for recurrent disease. Am. J. Hematol. 90:1172–1178, 2015. © 2015 Wiley Periodicals, Inc.

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Follicular Large Cell Lymphoma: An Aggressive Lymphoma That Often Presents With Favorable Prognostic Features

Cited by 59 publications

References 25 publications

Follicular lymphoma: 2020 update on diagnosis and management

Follicular lymphoma: 2020 update on diagnosis and management

Follicular lymphoma: 2014 update on diagnosis and management

Follicular lymphoma: 2015 update on diagnosis and management

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