Follicular lymphoma classification

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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

Follicular lymphoma may be classified according to WHO criteria into 3 groups: low grade follicular lymphoma, high grade follicular lymphoma and diffuse large B cell 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.

There are three variants of follicular lymphoma:

1.Pediatric follicular lymphoma

• Involves cervical lymph nodes, other peripheral lymph nodes, or Waldeyer ring
• Typically have early stage disease
• Lacks BCL-2 protein expression and t(14;18)

2.Primary intestinal follicular lymphoma

• Occurs most commonly in the small intestine, and frequently involves the duodenum

• Duodenal follicular lymphoma

• Found in the second portion of the duodenum
• Present as multiple polyps
• Diagnosis is most often an incidental finding
• Most patients have localized disease
• Prognosis is excellent even without treatment

3.Other extranodal follicular lymphomas

• Usually have localized extranodal disease
• Systemic relapses are rare
• Testicular follicular lymphoma are reported with increased frequency in children, but also are reported in adults.