Follicular lymphoma classification

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

Overview

Classification

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

  • 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)

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.

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 (9680/3) (See PH rules).[2]

There are three variants of Follicular Lymphoma: 1. Pediatric follicular lymphoma (mostly grade 3) involves cervical lymph nodes, other peripheral lymph nodes, or Waldeyer ring. Other extranodal involvement may occur. Children with follicular lymphoma typically have early stage disease. Pediatric follicular lymphoma typically lacks BCL2 protein expression and t(14;18).

2. Primary intestinal follicular lymphoma in the GI tract occurs most commonly in the small intestine, and frequently involves the duodenum.

3. Duodenal follicular lymphoma is predominantly found in the second portion of the duodenum, presenting as multiple polyps. The diagnosis is most often an incidental finding. Most patients have localized disease and prognosis is excellent even without treatment.

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.

References

  1. "Follicular Lymphomas". Retrieved 2008-07-26.
  2. National Cancer Institute. Surveillance, Epidemiology, and End Results Program 2015. http://seer.cancer.gov