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| <blockquote class='blockedit'>{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Follicular Lymphoma]]. | | <blockquote class="blockedit">{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Follicular Lymphoma]]. |
| Other relevent pages include: [[HAEM4:Testicular Follicular Lymphoma]] | | Other relevent pages include: [[HAEM4:Testicular Follicular Lymphoma]] |
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| |Follicular lymphoma | | |Follicular lymphoma |
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| ==Definition / Description of Disease==
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| *Follicular Lymphoma (FL) arises from germinal center B cells of the secondary lymphatic system (lymph nodes, spleen)
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| *WHO 5th edition classifies FL into four variants: ''in situ'' FL, duodenal-type FL, pediatric FL, and Follicular Lymphoma<ref name=":0">{{Cite journal|last=Alaggio|first=Rita|last2=Amador|first2=Catalina|last3=Anagnostopoulos|first3=Ioannis|last4=Attygalle|first4=Ayoma D.|last5=Araujo|first5=Iguaracyra Barreto de Oliveira|last6=Berti|first6=Emilio|last7=Bhagat|first7=Govind|last8=Borges|first8=Anita Maria|last9=Boyer|first9=Daniel|date=2022|title=The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9214472/|journal=Leukemia|volume=36|issue=7|pages=1720–1748|doi=10.1038/s41375-022-01620-2|issn=0887-6924|pmc=9214472|pmid=35732829}}</ref>.
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| **FL is further classified as Classic FL (cFL), characterized by t(14;18), follicular large B-cell lymphoma (FLBL), and FL with uncommon features (uFL)<ref name=":0" />
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| **There is emerging evidence that FL may arise from ''in situ'' FL or as a primary <ref>{{Cite journal|last=Carbone|first=Antonino|last2=Gloghini|first2=Annunziata|last3=Santoro|first3=Armando|date=2012-03|title=In situ follicular lymphoma: pathologic characteristics and diagnostic features: In situ follicular lymphoma|url=https://onlinelibrary.wiley.com/doi/10.1002/hon.993|journal=Hematological Oncology|language=en|volume=30|issue=1|pages=1–7|doi=10.1002/hon.993}}</ref>
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| *For most patients, FL is a chronic, incurable disease with survival often measured in decades.
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| *Histologic transformation to more aggressive disease with potentially fatal outcome may occur
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| ==Synonyms / Terminology==
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| Follicular Center Lymphoma; Follicle-related B-cell Lymphoma
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| ==Epidemiology / Prevalence==
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| Slight male predominance (male-to-female ratio of 1.2:1)
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| Median age at diagnosis is 60-65 years;
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| Progressive increase in incidence between ages 35-70
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| FL is extremely rare in children; considered a separate entity from adult FL
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| ~5% of all hematological neoplasms are FL
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| ~20% of all non-Hodgkin lymphomas are FL<ref name=":1">{{Cite journal|last=Ferry|first=Judith A.|date=2010-12-01|title=Recent Advances in Follicular Lymphoma: Pediatric, Extranodal, and Follicular Lymphoma in Situ|url=https://www.sciencedirect.com/science/article/pii/S1875918110001133|journal=Surgical Pathology Clinics|series=Current Concepts in Hematopathology|volume=3|issue=4|pages=877–906|doi=10.1016/j.path.2010.08.002|issn=1875-9181}}</ref>
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| Highest incidence in developed/high income countries
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| Second most common lymphoma in the USA and western Europe
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| Most common lymphoma among non-Hispanic white population [2]
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| Environmental exposures to pesticides and herbicides as risk factors are disputed; Hair dye use prior to 1980 is associated with increased risk (meta RR = 1.66) [3, 4].
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| Genetic risk factors may include SNPs within HLA class I or II genes [8] or homozygosity of HLA class II genes [9]; these features have been identified within a few familial cases of FL.
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| ----
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| ==Clinical Features==
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| Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table. Do not delete table.'') </span>
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| *FL commonly presents as painless lymphadenopathy<ref name=":12" />
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| *May wax and wane over years before diagnosis
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| *Majority of cases have widespread involvement at diagnosis<ref name=":12" />
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| *Bone marrow involvement in 40-70% of cases at diagnosis
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| *May not require treatment depending staging and other parameters.
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| <blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote>[1, 2, 3, 4]<blockquote class="blockedit">
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| ==Sites of Involvement==
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| Lymph Nodes / Lymphadenopathy; Spleen; Bone Marrow
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| <blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote>[1, 2, 3, 4]<blockquote class="blockedit">
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| <center><span style="color:Maroon">'''End of V4 Section'''</span>
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| </blockquote>
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| ==Morphologic Features==
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| Appearance of predominantly follicular pattern
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| Consists of both centrocytes and centroblasts, with the relative proportions of these cells informing grading
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| Grade 1: 0-5 centroblasts/high power field (hpf)
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| Grade 2: 6-15 centroblasts/hpf
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| Grade III: >15 centroblasts/hpf
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| Grade IIIa: centrocytes present
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| Grade IIIb: sheets of centroblasts
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| ==Immunophenotype==
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| Typically, FL is CD10+, BL2+. Atypical FL subgroups CD10- and/or BCL2 - all FL are STMN+ - useful differentiator between atypical FL and MZL [9]
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| {| class="wikitable sortable"
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| !Finding!!Marker
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| |Positive (universal)||monotypic surface Ig (sIg)+, BCL2, CD10, CD19, CD20, CD79a, STMN+
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| |Positive (subset)||atypical FL [CD10+/- and/or BCL2 +/-]
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| |Negative (universal)||CD5-, CD23-, CD43-
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| |Negative (subset)||
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| |}
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| ==WHO Essential and Desirable Genetic Diagnostic Criteria== | | ==WHO Essential and Desirable Genetic Diagnostic Criteria== |
| <span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span> | | <span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span> |
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| <blockquote class= 'blockedit '>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote>[1, 2, 5, 7, 9, 11]<blockquote class="blockedit"> | | <blockquote class= "blockedit ">{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote>[1, 2, 5, 7, 9, 11]<blockquote class="blockedit"> |
| <center><span style="color:Maroon">'''End of V4 Section'''</span> | | <center><span style="color:Maroon">'''End of V4 Section'''</span> |
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| <blockquote class='blockedit'>{{Box-round|title=v4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).|Please incorporate this section into the relevant tables found in: | | <blockquote class="blockedit">{{Box-round|title=v4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).|Please incorporate this section into the relevant tables found in: |
| * Chromosomal Rearrangements (Gene Fusions) | | * Chromosomal Rearrangements (Gene Fusions) |
| * Individual Region Genomic Gain/Loss/LOH | | * Individual Region Genomic Gain/Loss/LOH |
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| |} | | |} |
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| deletions in 1p36, 6q, 10q, 13p, 17p; gains of 1q, 2p, 7, 8, 12q, 18q | | deletions in 1p36, 6q, 10q, 13p, 17p; gains of 1q, 2p, 7, 8, 12q, 18q |
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| <blockquote class= 'blockedit '>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote>[11]<blockquote class="blockedit"> | | <blockquote class= "blockedit ">{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote>[11]<blockquote class="blockedit"> |
| <center><span style="color:Maroon">'''End of V4 Section'''</span> | | <center><span style="color:Maroon">'''End of V4 Section'''</span> |
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| |}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content. | | |}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content. |
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| ''BCR-NFκB'', ''JAK/STAT''; ''mTORC'' signaling | | ''BCR-NFκB'', ''JAK/STAT''; ''mTORC'' signaling |
