Follicular lymphoma natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Prognosis of follicular lymphoma is generally poor. The median survival is around 10 years.

Prognosis

• Follicular lymphoma is regarded as incurable (although allogenic stem cell transplantation may be curative, the mortality from the procedure is too high to be a first line option). The median survival is around 10 years, but the range is wide, from less than one year, to more than 20 years. Some patients may never need treatment. The overall survival rate at 5 years is 72-77%.
• Follicular lymphoma is graded according to the proportion of large cells (centroblasts). Studies suggest this histologic grading predicts clinical outcome, with more large cells behaving more aggressively and having a higher likelihood of transformation to diffuse large cell lymphoma. When any area of diffuse large-B-cell lymphoma is present in a follicular lymphoma the disease should be reported as diffuse large B-cell lymphoma..
• There was no difference in survival outcomes between patients with Grade 3A and 3B follicular lymphoma, whereas patients with FL3 with more than 50% diffuse component have an inferior survival similar to the survival of those with diffuse large cell lymphoma.
• FL3B with cytogenetic abnormalities of BCL6 (at 3q27) are thought to be genetically more akin to germinal center type diffuse large B-cell lymphoma than FL1-3A, and is associated with a more aggressive clinical course.
• Patients with FL3B with BCL2 translocation appear to have a clinical course similar to patients with FL1-3A. Since FL3B is rare, the clinical behavior of FL3 in most studies is based mainly on FL3A cases.

1. The Follicular Lymphoma International Prognostic Index (FLIPI) includes

• Age
• Ann Arbor stage
• Number of nodal sites involved
• Hemoglobin levels
• Serum LDH levels

2. The Follicular Lymphoma International Prognostic Index (FLIPI-2) includes

• Age
• Hemoglobin levels
• Longest diameter of largest involved lymph node
• Beta-2 microglobulin levels
• Bone marrow involvement

Both the FLIPI-1 and FLIPI-2 predict for prognosis, but not yet useful for selecting treatment options.

3. Most recently,a simpler prognostic index includes

• baseline serum beta-2 microglobulin
• LDH levels