Follicular lymphoma

Follicular lymphoma
Micrograph of a follicular lymphoma, showing the characteristically abnormal lymphoid follicles that gave the condition its name. H&E stain.
SpecialtyHematology and oncology

Follicular lymphoma (FL) is a cancer that involves certain types of white blood cells known as lymphocytes. The cancer originates from the uncontrolled division of specific types of B-cells known as centrocytes and centroblasts. These cells normally occupy the follicles (nodular swirls of various types of lymphocytes) in the germinal centers of lymphoid tissues such as lymph nodes. The cancerous cells in FL typically form follicular or follicle-like structures (see adjacent Figure) in the tissues they invade. These structures are usually the dominant histological feature of this cancer.[1]

There are several synonymous and obsolete terms for FL such as CB/CC lymphoma (centroblastic and centrocytic lymphoma), nodular lymphoma,[2] Brill-Symmers Disease, and the subtype designation, follicular large-cell lymphoma.[3] In the US and Europe, this disease is the second most common form of non-Hodgkin's lymphomas, exceeded only by diffuse large B-cell lymphoma.[4] FL accounts for 10–20% of non-Hodgkin's lymphomas with ~15,000 new cases of it being newly diagnosed each year in the US and Europe.[5] Recent studies indicate that FL is similarly prevalent in Japan.[6]

FL is a broad and extremely complex clinical entity with a wide range of manifestations[7] which have not yet been fully systematized.[8] It is commonly preceded by a benign precancerous disorder in which abnormal centrocytes and/or centroblasts accumulate in lymphoid tissue. They may then circulate in the blood to cause an asymptomatic condition termed in situ lymphoid neoplasia of the follicular lymphoma type (i.e. ISFL). A small percentage of these cases progress to FL.[9] Most commonly, however, FL presents as a swelling of lymph nodes in the neck, armpits, and/or groin. Less often, it presents as a gastrointestinal tract cancer, a cancer in children involving lymphoid tissues of the head and neck area (e.g., tonsils),[10] or one or more masses in non-lymphoid tissues such as the testes.[11]

FL typically has a slow disease course which persists essentially unchanged for years.[7] However, each year 2–3%[12] of FL cases progress to a highly aggressive form often termed stage 3B FL, to an aggressive diffuse large B-cell lymphoma, or to another type of aggressive B-cell cancer. These transformed follicular lymphomas (t-FL) are essentially incurable.[5] However, recent advancements in the treatment of t-FL (e.g., the addition to standard chemotherapy of agents such as rituximab) have improved overall survival times. These newer regimens may also delay the transformation of FL to t-FL.[5] Additional advances in understanding FL may lead to further improvements in treating the disease.[12][13]

  1. ^ Xerri L, Dirnhofer S, Quintanilla-Martinez L, Sander B, Chan JK, Campo E, et al. (February 2016). "The heterogeneity of follicular lymphomas: from early development to transformation". Virchows Archiv. 468 (2): 127–39. doi:10.1007/s00428-015-1864-y. PMID 26481245. S2CID 2978889.
  2. ^ "follicular lymphoma" at Dorland's Medical Dictionary
  3. ^ Large-Cell+Lymphoma,+Follicular at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
  4. ^ Dada R (June 2019). "Diagnosis and management of follicular lymphoma: A comprehensive review". European Journal of Haematology. 103 (3): 152–163. doi:10.1111/ejh.13271. PMID 31270855.
  5. ^ a b c Fischer T, Zing NP, Chiattone CS, Federico M, Luminari S (January 2018). "Transformed follicular lymphoma". Annals of Hematology. 97 (1): 17–29. doi:10.1007/s00277-017-3151-2. hdl:11380/1152780. PMID 29043381. S2CID 11524500.
  6. ^ Yoshino T, Takata K, Tanaka T, Sato Y, Tari A, Okada H (December 2018). "Recent progress in follicular lymphoma in Japan and characteristics of the duodenal type". Pathology International. 68 (12): 665–676. doi:10.1111/pin.12733. PMID 30456840. S2CID 53871784.
  7. ^ a b Lynch RC, Gratzinger D, Advani RH (July 2017). "Clinical Impact of the 2016 Update to the WHO Lymphoma Classification". Current Treatment Options in Oncology. 18 (7): 45. doi:10.1007/s11864-017-0483-z. PMID 28670664. S2CID 4415738.
  8. ^ Boughan KM, Caimi PF (May 2019). "Follicular Lymphoma: Diagnostic and Prognostic Considerations in Initial Treatment Approach". Current Oncology Reports. 21 (7): 63. doi:10.1007/s11912-019-0808-0. PMID 31119485. S2CID 162181232.
  9. ^ Oishi N, Montes-Moreno S, Feldman AL (January 2018). "In situ neoplasia in lymph node pathology". Seminars in Diagnostic Pathology. 35 (1): 76–83. doi:10.1053/j.semdp.2017.11.001. PMID 29129357.
  10. ^ Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, Advani R, Ghielmini M, Salles GA, Zelenetz AD, Jaffe ES (May 2016). "The 2016 revision of the World Health Organization classification of lymphoid neoplasms". Blood. 127 (20): 2375–90. doi:10.1182/blood-2016-01-643569. PMC 4874220. PMID 26980727.
  11. ^ Takata K, Miyata-Takata T, Sato Y, Yoshino T (2014). "Pathology of follicular lymphoma". Journal of Clinical and Experimental Hematopathology. 54 (1): 3–9. doi:10.3960/jslrt.54.3. PMID 24942941.
  12. ^ a b Link BK (March 2018). "Transformation of follicular lymphoma – Why does it happen and can it be prevented?". Best Practice & Research. Clinical Haematology. 31 (1): 49–56. doi:10.1016/j.beha.2017.10.005. PMID 29452666.
  13. ^ Sorigue M, Sancho JM (February 2018). "Current prognostic and predictive factors in follicular lymphoma". Annals of Hematology. 97 (2): 209–227. doi:10.1007/s00277-017-3154-z. PMID 29032510. S2CID 27602442.