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Follicular lymphoma

July 25, 2023 — published by Pyramidale Communication

At Pyramidale Communication Agency, we leverage our expertise in numerous blood disorders, particularly in onco-hematology. One of these is follicular lymphoma (FL).

Key numbers for follicular lymphoma

Follicular lymphoma is the most common type of indolent lymphoma accounting for 30% of non-Hodgkin’s lymphoma (NHL) cases in France. ^1-3
Its prevalence is approximately 1 in 3,000, person, with an estimated 3,000 new cases per year in France. ¹
The median age at diagnosis is 60–65 years with a slight male predominance. The disease is extremely rare in children. ^1,3

What is follicular lymphoma?

Follicular lymphoma is a form of non-Hodgkin lymphoma characterized by the uncontrolled proliferation of B cells. Its nodular structure with follicular architecture is typically preserved. ¹ In 85% of cases, FL is associated with the t(14;18) (q32;q21) translocation, which leads to overproduction of the BCL2 protein, a key regulator of apoptosis. ^1,3 Other genetic events may also contribute to disease development. ⁴

In a significant number of patients, the disease is completely indolent and asymptomatic. ³ Symptoms appear at an advanced stage of the disease,¹ with general signs such as prolonged fever, profuse night sweats, significant weight loss (-10% of body weight), or symptoms suggestive of possible compression by the tumor syndrome. ^3,5

Diagnostic criteria and management

The diagnosis of follicular lymphoma primarily relies on a histology from a biopsy of a lymph node a complete blood count and LDH levels. ^1,3 The presence of the t(14;18) translocation can support the diagnosis but is not always required, as it is less frequent in grade 3A and 3B follicular lymphoma. ³
Other cytogenetic and molecular abnormalities may also be evaluated. ³ Differential diagnoses, such as chronic B-cell lymphocytic leukemia or diffuse large B-cell lymphoma, must be excluded.

Although overall survival of patients with follicular lymphoma has greatly improved with advances in treatment, the disease remains incurable. ² The selection of a therapeutic strategy should be discussed in a multidisciplinary meeting and tailored to each individual situation. ³

In general radiotherapy with or without immunochemotherapy is used in cases of stage I localized follicular lymphoma. ³ In disseminated forms with low tumor mass, watchful waiting is generally recommended. In cases of high tumor burden chemotherapy combined with anti-CD20 antibody is preferred. ^2,3

The therapeutic challenge is particularly significant in patients who relapse within 24 months of initiating first-line treatment (20% of cases). In these patients, overall survival is nearly twice as low compared with patients who do not relapse during this period (5-year OS: 50% versus 90%). ²

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GELF: follicular lymphoma study group; LDH: lactate dehydrogenase; FL: follicular lymphoma; NHL: non-Hodgkin lymphoma.

References:

Orphanet. Lymphome folliculaire. Disponible en ligne : https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=545
Rossi C and Bastie JN. Actualités thérapeutiques dans les lymphomes non hodgkiniens et le lymphome de Hodgkin. La Revue de médecine interne. 2019;40(4):246-254.
Sujobert P, Salles G. Actualités dans le lymphome folliculaire. Presse Med. 2019;48(7-8)part 1:850-858.
Freedman A, Jacobsen E. Follicular lymphoma: 2020 update on diagnosis and management. Am J Hematol. 2020 Mar;95(3):316-327.
ESMO et Fonds Anticancer. Qu’est-ce que lymphoma folliculaire? Laissez-nous vous expliquer. Disponible sur le lien : https://www.esmo.org/content/download/52237/963503/1/FR-Lymphome-Folliculaire-Guide-pour-les-Patients.pdf

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