Follicular lymphoma, grade 2
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Most patients present with widespread disease, including peripheral and central (abdominal and thoracic) lymphadenopathy and splenomegaly. The BM is involved in 40-70%.
Only 1/3 of patients present with stage I or II at the time of diagnosis.
FL 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 (DLBCL) is present in a FL, the disease should be reported as diffuse large B-cell lymphoma (9680/3) (See PH rules).
There are three variants of FL:
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 FL typically have early stage disease. Pediatric FL 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 FL are reported with increased frequency in children, but also are reported in adults.