Follicular lymphoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Follicular lymphoma may be classified according to WHO criteria into low grade follicular lymphoma, high grade follicular lymphoma and diffuse large B cell lymphoma. Pediatric-type follicular lymphoma, primary intestinal follicular lymphoma, other extranodal follicular lymphomas and follicular lymphoma “in situ” (FLIS) are the other variants that are included under follicular lymphoma.

Classification

1. According to the WHO criteria, the disease is morphologically graded into:

• Grade 1 (<5 centroblasts per high-power field (hpf))
• Grade 2 (6–15 centroblasts/hpf)
• Grade 3 (>15 centroblasts/hpf)

• Grade 3A (centrocytes still present)
• Grade 3B (the follicles consist almost entirely of centroblasts)

2-The WHO 2008 update classifies

• Grades 1 and 2 now as low grade follicular lymphoma
• Grade 3A as high grade follicular lymphoma
• Grade 3B as diffuse large B cell lymphoma.

A-Pediatric-type follicular lymphoma

• Rare variant of follicular lymphoma
• Characterized by lack of BCL2 rearrangement and t(14,18)
• Better prognosis than adult follicular lymphoma
• Often cured with minimal therapy.

B-Primary intestinal follicular lymphoma

• Common in the small intestine with the vast majority of cases occurring in the duodenum.
• The morphology, immunophenotype, and genetic features are similar to those of nodal follicular lymphoma.
• Most patients have clinically indolent and localized disease.
• Survival appears to be excellent even without treatment.

C-Other extranodal follicular lymphoma

• The morphology, immunophenotype, and genetic features are similar to those of nodal follicular lymphoma.
• Patients usually have localized disease and systemic relapses are rare.

D-Follicular Lymphoma “in situ”

• The occurrence in the general population appears to be rare,reported in patients with prior follicular lymphoma or concurrent follicular lymphoma (at other sites), as well as in individuals with no known history of follicular lymphoma.
• Preservation of the lymph node architecture
• Incidental finding of focal strongly positive staining for BCL2 (restricted to germinal centers) and CD10 in the involved follicles
• The detection of t(14;18) by FISH.

Other extranodal follicular lymphomas occur in almost any extranodal site. Patients usually have localized extranodal disease and systemic relapses are rare. Testicular follicular lymphoma are reported with increased frequency in children, but also are reported in adults.