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Follicular Helper T-Cell Lymphoma

MedGen UID:
1622985
•Concept ID:
C4528203
•
Neoplastic Process
Synonyms: Nodal Follicular Helper T-Cell Lymphoma; Nodal Peripheral T-Cell Lymphoma of T Follicular Helper Cell Origin; Nodal Peripheral T-Cell Lymphoma of TFH Cell Origin; TFH Lymphoma

Definition

A group of node-based peripheral T-cell lymphomas with phenotypic features of T follicular helper (TFH) cells. This category includes the following: follicular helper T-cell lymphoma, angioimmunoblastic-type; follicular helper T-cell lymphoma, follicular-type; and follicular helper T-cell lymphoma, not otherwise specified. [from NCI]

Professional guidelines

PubMed

Frontline Management of Nodal Peripheral T-Cell Lymphomas.
Ngu HS, Savage KJ
Am Soc Clin Oncol Educ Book 2023 May;43:e390334. doi: 10.1200/EDBK_390334. PMID: 37262395
Hodgkin Lymphoma and Its Differential Diagnosis: New Twists on an Old Challenge.
Sohani AR
Surg Pathol Clin 2023 Jun;16(2):287-346. Epub 2023 Mar 30 doi: 10.1016/j.path.2023.02.002. PMID: 37149361

Recent clinical studies

Etiology

Oral azacitidine compared with standard therapy in patients with relapsed or refractory follicular helper T-cell lymphoma (ORACLE): an open-label randomised, phase 3 study.
Dupuis J, Bachy E, Morschhauser F, Cartron G, Fukuhara N, Daguindau N, Casasnovas RO, Snauwaert S, Gressin R, Fox CP, d'Amore FA, Staber PB, Tournilhac O, Bouabdallah K, Thieblemont C, André M, Rai S, Ennishi D, Gkasiamis A, Nishio M, Fornecker LM, Delfau-Larue MH, Sako N, Mule S, de Leval L, Gaulard P, Tsukasaki K, Lemonnier F
Lancet Haematol 2024 Jun;11(6):e406-e414. doi: 10.1016/S2352-3026(24)00102-9. PMID: 38796193
What is new in the classification of peripheral T cell lymphomas?
de Leval L, Bisig B
Pathologie (Heidelb) 2023 Dec;44(Suppl 3):128-135. Epub 2023 Dec 4 doi: 10.1007/s00292-023-01260-y. PMID: 38047948Free PMC Article
Classification and diagnostic evaluation of nodal T- and NK-cell lymphomas.
Feldman AL, Laurent C, Narbaitz M, Nakamura S, Chan WC, de Leval L, Gaulard P
Virchows Arch 2023 Jan;482(1):265-279. Epub 2022 Oct 10 doi: 10.1007/s00428-022-03412-6. PMID: 36210383
Comprehensive comparison of international prognostic indexes for follicular helper T-cell lymphoma.
Lee YP, Yoon SE, Cho J, Ko YH, Jo H, Kim SJ, Kim WS
Ann Hematol 2022 Jul;101(7):1535-1543. Epub 2022 May 31 doi: 10.1007/s00277-022-04805-y. PMID: 35639152
Primary Cutaneous Follicular Helper T-Cell Lymphoma: A Case Series and Review of the Literature.
Wang JY, Nguyen GH, Ruan J, Magro CM
Am J Dermatopathol 2017 May;39(5):374-383. doi: 10.1097/DAD.0000000000000695. PMID: 28375859

Diagnosis

What is new in the classification of peripheral T cell lymphomas?
de Leval L, Bisig B
Pathologie (Heidelb) 2023 Dec;44(Suppl 3):128-135. Epub 2023 Dec 4 doi: 10.1007/s00292-023-01260-y. PMID: 38047948Free PMC Article
Classification and diagnostic evaluation of nodal T- and NK-cell lymphomas.
Feldman AL, Laurent C, Narbaitz M, Nakamura S, Chan WC, de Leval L, Gaulard P
Virchows Arch 2023 Jan;482(1):265-279. Epub 2022 Oct 10 doi: 10.1007/s00428-022-03412-6. PMID: 36210383
Comprehensive comparison of international prognostic indexes for follicular helper T-cell lymphoma.
Lee YP, Yoon SE, Cho J, Ko YH, Jo H, Kim SJ, Kim WS
Ann Hematol 2022 Jul;101(7):1535-1543. Epub 2022 May 31 doi: 10.1007/s00277-022-04805-y. PMID: 35639152
Primary cutaneous follicular helper T-cell lymphoma.
Santonja C, Soto C, Manso R, Requena L, Piris MA, Rodríguez-Pinilla SM
J Cutan Pathol 2016 Feb;43(2):164-70. Epub 2015 Sep 18 doi: 10.1111/cup.12614. PMID: 26282465
Follicular helper T-cell lymphoma: a B-cell-rich variant of T-cell lymphoma.
Ruiz SJ, Cotta CV
Ann Diagn Pathol 2015 Aug;19(4):187-92. Epub 2015 Apr 6 doi: 10.1016/j.anndiagpath.2015.03.008. PMID: 25913305

Therapy

Oral azacitidine compared with standard therapy in patients with relapsed or refractory follicular helper T-cell lymphoma (ORACLE): an open-label randomised, phase 3 study.
Dupuis J, Bachy E, Morschhauser F, Cartron G, Fukuhara N, Daguindau N, Casasnovas RO, Snauwaert S, Gressin R, Fox CP, d'Amore FA, Staber PB, Tournilhac O, Bouabdallah K, Thieblemont C, André M, Rai S, Ennishi D, Gkasiamis A, Nishio M, Fornecker LM, Delfau-Larue MH, Sako N, Mule S, de Leval L, Gaulard P, Tsukasaki K, Lemonnier F
Lancet Haematol 2024 Jun;11(6):e406-e414. doi: 10.1016/S2352-3026(24)00102-9. PMID: 38796193
Romidepsin Plus Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone Versus Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone in Patients With Previously Untreated Peripheral T-Cell Lymphoma: Final Analysis of the Ro-CHOP Trial.
Camus V, Thieblemont C, Gaulard P, Cheminant M, Casasnovas RO, Ysebaert L, Damaj GL, Guidez S, Pica GM, Kim WS, Lim ST, Andre M, Gutiérrez N, Penarrubia MJ, Staber PB, Trotman J, Hüttmann A, Stefoni V, Tucci A, Fogarty P, Farhat H, Abraham J, Abarah W, Belmecheri F, Ribrag V, Delfau-Larue MH, Cottereau AS, Itti E, Li J, Delarue R, de Leval L, Morschhauser F, Bachy E
J Clin Oncol 2024 May 10;42(14):1612-1618. Epub 2024 Feb 16 doi: 10.1200/JCO.23.01687. PMID: 38364196
Primary Cutaneous Follicular Helper T-Cell Lymphoma: A Case Series and Review of the Literature.
Wang JY, Nguyen GH, Ruan J, Magro CM
Am J Dermatopathol 2017 May;39(5):374-383. doi: 10.1097/DAD.0000000000000695. PMID: 28375859
Primary cutaneous follicular helper T-cell lymphoma: a new subtype of cutaneous T-cell lymphoma reported in a series of 5 cases.
Battistella M, Beylot-Barry M, Bachelez H, Rivet J, Vergier B, Bagot M
Arch Dermatol 2012 Jul;148(7):832-9. doi: 10.1001/archdermatol.2011.3269. PMID: 22508770
Nodal follicular helper T-cell lymphoma may present with different patterns. A case report.
Qubaja M, Audouin J, Moulin JC, Molina TJ, Le Tourneau A, Gaulard P, Straub P, Audhuy B, Diebold J
Hum Pathol 2009 Feb;40(2):264-9. Epub 2008 Aug 28 doi: 10.1016/j.humpath.2008.05.018. PMID: 18760445

Prognosis

Immunophenotypic classification regarding prognosis in peripheral T cell lymphoma, NOS, and nodal T follicular helper T cell lymphoma, angioimmunoblastic-type.
Choi S, Jo JC, Lee YJ, Chae SW, Cha HJ
Ann Hematol 2024 Jul;103(7):2429-2443. Epub 2024 May 30 doi: 10.1007/s00277-024-05817-6. PMID: 38814447
Romidepsin Plus Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone Versus Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone in Patients With Previously Untreated Peripheral T-Cell Lymphoma: Final Analysis of the Ro-CHOP Trial.
Camus V, Thieblemont C, Gaulard P, Cheminant M, Casasnovas RO, Ysebaert L, Damaj GL, Guidez S, Pica GM, Kim WS, Lim ST, Andre M, Gutiérrez N, Penarrubia MJ, Staber PB, Trotman J, Hüttmann A, Stefoni V, Tucci A, Fogarty P, Farhat H, Abraham J, Abarah W, Belmecheri F, Ribrag V, Delfau-Larue MH, Cottereau AS, Itti E, Li J, Delarue R, de Leval L, Morschhauser F, Bachy E
J Clin Oncol 2024 May 10;42(14):1612-1618. Epub 2024 Feb 16 doi: 10.1200/JCO.23.01687. PMID: 38364196
Comprehensive comparison of international prognostic indexes for follicular helper T-cell lymphoma.
Lee YP, Yoon SE, Cho J, Ko YH, Jo H, Kim SJ, Kim WS
Ann Hematol 2022 Jul;101(7):1535-1543. Epub 2022 May 31 doi: 10.1007/s00277-022-04805-y. PMID: 35639152
Monoclonal plasma cell infiltrates in the setting of cutaneous follicular helper T cell lymphoproliferative disorders.
Magro CM, Ruan J, Grossman M, Hedayat AA
Ann Diagn Pathol 2019 Jun;40:94-104. Epub 2019 Apr 26 doi: 10.1016/j.anndiagpath.2019.04.013. PMID: 31077877
Primary Cutaneous Follicular Helper T-Cell Lymphoma: A Case Series and Review of the Literature.
Wang JY, Nguyen GH, Ruan J, Magro CM
Am J Dermatopathol 2017 May;39(5):374-383. doi: 10.1097/DAD.0000000000000695. PMID: 28375859

Clinical prediction guides

Immunophenotypic classification regarding prognosis in peripheral T cell lymphoma, NOS, and nodal T follicular helper T cell lymphoma, angioimmunoblastic-type.
Choi S, Jo JC, Lee YJ, Chae SW, Cha HJ
Ann Hematol 2024 Jul;103(7):2429-2443. Epub 2024 May 30 doi: 10.1007/s00277-024-05817-6. PMID: 38814447
Oral azacitidine compared with standard therapy in patients with relapsed or refractory follicular helper T-cell lymphoma (ORACLE): an open-label randomised, phase 3 study.
Dupuis J, Bachy E, Morschhauser F, Cartron G, Fukuhara N, Daguindau N, Casasnovas RO, Snauwaert S, Gressin R, Fox CP, d'Amore FA, Staber PB, Tournilhac O, Bouabdallah K, Thieblemont C, André M, Rai S, Ennishi D, Gkasiamis A, Nishio M, Fornecker LM, Delfau-Larue MH, Sako N, Mule S, de Leval L, Gaulard P, Tsukasaki K, Lemonnier F
Lancet Haematol 2024 Jun;11(6):e406-e414. doi: 10.1016/S2352-3026(24)00102-9. PMID: 38796193
Romidepsin Plus Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone Versus Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone in Patients With Previously Untreated Peripheral T-Cell Lymphoma: Final Analysis of the Ro-CHOP Trial.
Camus V, Thieblemont C, Gaulard P, Cheminant M, Casasnovas RO, Ysebaert L, Damaj GL, Guidez S, Pica GM, Kim WS, Lim ST, Andre M, Gutiérrez N, Penarrubia MJ, Staber PB, Trotman J, Hüttmann A, Stefoni V, Tucci A, Fogarty P, Farhat H, Abraham J, Abarah W, Belmecheri F, Ribrag V, Delfau-Larue MH, Cottereau AS, Itti E, Li J, Delarue R, de Leval L, Morschhauser F, Bachy E
J Clin Oncol 2024 May 10;42(14):1612-1618. Epub 2024 Feb 16 doi: 10.1200/JCO.23.01687. PMID: 38364196
Comprehensive comparison of international prognostic indexes for follicular helper T-cell lymphoma.
Lee YP, Yoon SE, Cho J, Ko YH, Jo H, Kim SJ, Kim WS
Ann Hematol 2022 Jul;101(7):1535-1543. Epub 2022 May 31 doi: 10.1007/s00277-022-04805-y. PMID: 35639152
Primary cutaneous follicular helper T-cell lymphoma: a new subtype of cutaneous T-cell lymphoma reported in a series of 5 cases.
Battistella M, Beylot-Barry M, Bachelez H, Rivet J, Vergier B, Bagot M
Arch Dermatol 2012 Jul;148(7):832-9. doi: 10.1001/archdermatol.2011.3269. PMID: 22508770

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