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Mantle cell lymphoma

MedGen UID:
1668377
Concept ID:
C4721414
Neoplastic Process
Synonyms: Centrocytic Small-Cell Lymphoma; Centrocytic Small-Cell Lymphomas; Diffuse Lymphocytic Lymphoma, Poorly Differentiated; Diffuse Lymphocytic Lymphoma, Poorly-Differentiated; Lymphocytic Lymphoma, Diffuse, Poorly Differentiated; Lymphocytic Lymphoma, Diffuse, Poorly-Differentiated; Lymphoma, Centrocytic Small Cell; Lymphoma, Centrocytic Small-Cell; Lymphoma, Lymphocytic, Diffuse, Intermediate Differentiated; Lymphoma, Lymphocytic, Diffuse, Poorly-Differentiated; Lymphoma, Mantle Cell; Lymphoma, Mantle-Cell; Lymphoma, Mantle-Zone; Lymphoma, Small-Cell, Centrocytic; Lymphomas, Centrocytic Small-Cell; Lymphomas, Mantle-Cell; Lymphomas, Mantle-Zone; Mantle Cell Lymphoma; Mantle Zone Lymphoma; Mantle-Cell Lymphoma; Mantle-Cell Lymphomas; Mantle-Zone Lymphoma; Mantle-Zone Lymphomas; Small-Cell Lymphoma, Centrocytic; Small-Cell Lymphomas, Centrocytic
SNOMED CT: Mantle cell lymphoma (74654000); Mantle cell lymphoma (443487006)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018876
OMIM®: 607585
Orphanet: ORPHA52416

Definition

Mantle cell lymphoma is a rare form of malignant non-Hodgkin lymphoma (see this term) affecting B lymphocytes in the lymph nodes in a region called the ``mantle zone''. [from ORDO]

Professional guidelines

PubMed

Ryan CE, Armand P, LaCasce AS
Blood 2025 Feb 13;145(7):663-672. doi: 10.1182/blood.2023022352. PMID: 38498174
Iwamuro M, Tanaka T, Okada H
World J Gastroenterol 2023 Mar 28;29(12):1852-1862. doi: 10.3748/wjg.v29.i12.1852. PMID: 37032723Free PMC Article
Hayden PJ, Roddie C, Bader P, Basak GW, Bonig H, Bonini C, Chabannon C, Ciceri F, Corbacioglu S, Ellard R, Sanchez-Guijo F, Jäger U, Hildebrandt M, Hudecek M, Kersten MJ, Köhl U, Kuball J, Mielke S, Mohty M, Murray J, Nagler A, Rees J, Rioufol C, Saccardi R, Snowden JA, Styczynski J, Subklewe M, Thieblemont C, Topp M, Ispizua ÁU, Chen D, Vrhovac R, Gribben JG, Kröger N, Einsele H, Yakoub-Agha I
Ann Oncol 2022 Mar;33(3):259-275. Epub 2021 Dec 16 doi: 10.1016/j.annonc.2021.12.003. PMID: 34923107

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) B-Cell Lymphomas, 2024

Recent clinical studies

Etiology

Dreyling M, Doorduijn J, Giné E, Jerkeman M, Walewski J, Hutchings M, Mey U, Riise J, Trneny M, Vergote V, Shpilberg O, Gomes da Silva M, Leppä S, Jiang L, Stilgenbauer S, Kerkhoff A, Jachimowicz RD, Celli M, Hess G, Arcaini L, Visco C, van Meerten T, Wirths S, Zinzani PL, Novak U, Herhaus P, Benedetti F, Sonnevi K, Hanoun C, Hänel M, Dierlamm J, Pott C, Klapper W, Gözel D, Schmidt C, Unterhalt M, Ladetto M, Hoster E
Lancet 2024 May 25;403(10441):2293-2306. Epub 2024 May 2 doi: 10.1016/S0140-6736(24)00184-3. PMID: 38705160
Wang M, Siddiqi T, Gordon LI, Kamdar M, Lunning M, Hirayama AV, Abramson JS, Arnason J, Ghosh N, Mehta A, Andreadis C, Solomon SR, Kostic A, Dehner C, Espinola R, Peng L, Ogasawara K, Chattin A, Eliason L, Palomba ML
J Clin Oncol 2024 Apr 1;42(10):1146-1157. Epub 2023 Dec 10 doi: 10.1200/JCO.23.02214. PMID: 38072625Free PMC Article
Silkenstedt E, Dreyling M
Hematol Oncol 2023 Jun;41 Suppl 1:36-42. doi: 10.1002/hon.3149. PMID: 37294961
Jain P, Wang ML
Am J Hematol 2022 May;97(5):638-656. doi: 10.1002/ajh.26523. PMID: 35266562
Wang M, Rule S, Zinzani PL, Goy A, Casasnovas O, Smith SD, Damaj G, Doorduijn J, Lamy T, Morschhauser F, Panizo C, Shah B, Davies A, Eek R, Dupuis J, Jacobsen E, Kater AP, Le Gouill S, Oberic L, Robak T, Covey T, Dua R, Hamdy A, Huang X, Izumi R, Patel P, Rothbaum W, Slatter JG, Jurczak W
Lancet 2018 Feb 17;391(10121):659-667. Epub 2017 Dec 11 doi: 10.1016/S0140-6736(17)33108-2. PMID: 29241979Free PMC Article

Diagnosis

Eyre TA, Bishton MJ, McCulloch R, O'Reilly M, Sanderson R, Menon G, Iyengar S, Lewis D, Lambert J, Linton KM, McKay P; Haemato-Oncology Task Force of the British Society for Haematology
Br J Haematol 2024 Jan;204(1):108-126. Epub 2023 Oct 25 doi: 10.1111/bjh.19131. PMID: 37880821
Silkenstedt E, Dreyling M
Hematol Oncol 2023 Jun;41 Suppl 1:36-42. doi: 10.1002/hon.3149. PMID: 37294961
Navarro A, Beà S, Jares P, Campo E
Hematol Oncol Clin North Am 2020 Oct;34(5):795-807. Epub 2020 Jul 22 doi: 10.1016/j.hoc.2020.05.002. PMID: 32861278Free PMC Article
Hoster E, Rosenwald A, Berger F, Bernd HW, Hartmann S, Loddenkemper C, Barth TF, Brousse N, Pileri S, Rymkiewicz G, Kodet R, Stilgenbauer S, Forstpointner R, Thieblemont C, Hallek M, Coiffier B, Vehling-Kaiser U, Bouabdallah R, Kanz L, Pfreundschuh M, Schmidt C, Ribrag V, Hiddemann W, Unterhalt M, Kluin-Nelemans JC, Hermine O, Dreyling MH, Klapper W
J Clin Oncol 2016 Apr 20;34(12):1386-94. Epub 2016 Feb 29 doi: 10.1200/JCO.2015.63.8387. PMID: 26926679
Birgitta S
Semin Cancer Biol 2011 Nov;21(5):291-2. Epub 2011 Oct 28 doi: 10.1016/j.semcancer.2011.10.006. PMID: 22082693

Therapy

Wang M, Salek D, Belada D, Song Y, Jurczak W, Kahl BS, Paludo J, Chu MP, Kryachok I, Fogliatto L, Cheah CY, Morawska M, Sancho JM, Li Y, Patti C, Forsyth C, Zhang J, Lesley R, Ramadan S, Rule S, Dreyling M; ECHO investigators; ECHO Investigators
J Clin Oncol 2025 Jul 10;43(20):2276-2284. Epub 2025 May 1 doi: 10.1200/JCO-25-00690. PMID: 40311141Free PMC Article
Dreyling M, Doorduijn J, Giné E, Jerkeman M, Walewski J, Hutchings M, Mey U, Riise J, Trneny M, Vergote V, Shpilberg O, Gomes da Silva M, Leppä S, Jiang L, Stilgenbauer S, Kerkhoff A, Jachimowicz RD, Celli M, Hess G, Arcaini L, Visco C, van Meerten T, Wirths S, Zinzani PL, Novak U, Herhaus P, Benedetti F, Sonnevi K, Hanoun C, Hänel M, Dierlamm J, Pott C, Klapper W, Gözel D, Schmidt C, Unterhalt M, Ladetto M, Hoster E
Lancet 2024 May 25;403(10441):2293-2306. Epub 2024 May 2 doi: 10.1016/S0140-6736(24)00184-3. PMID: 38705160
Park JH, Nath K, Devlin SM, Sauter CS, Palomba ML, Shah G, Dahi P, Lin RJ, Scordo M, Perales MA, Shouval R, Tomas AA, Cathcart E, Mead E, Santomasso B, Holodny A, Brentjens RJ, Riviere I, Sadelain M
Nat Med 2023 Jul;29(7):1710-1717. Epub 2023 Jul 3 doi: 10.1038/s41591-023-02404-6. PMID: 37400640Free PMC Article
Wang M, Munoz J, Goy A, Locke FL, Jacobson CA, Hill BT, Timmerman JM, Holmes H, Jaglowski S, Flinn IW, McSweeney PA, Miklos DB, Pagel JM, Kersten MJ, Milpied N, Fung H, Topp MS, Houot R, Beitinjaneh A, Peng W, Zheng L, Rossi JM, Jain RK, Rao AV, Reagan PM
N Engl J Med 2020 Apr 2;382(14):1331-1342. doi: 10.1056/NEJMoa1914347. PMID: 32242358Free PMC Article
Wang M, Rule S, Zinzani PL, Goy A, Casasnovas O, Smith SD, Damaj G, Doorduijn J, Lamy T, Morschhauser F, Panizo C, Shah B, Davies A, Eek R, Dupuis J, Jacobsen E, Kater AP, Le Gouill S, Oberic L, Robak T, Covey T, Dua R, Hamdy A, Huang X, Izumi R, Patel P, Rothbaum W, Slatter JG, Jurczak W
Lancet 2018 Feb 17;391(10121):659-667. Epub 2017 Dec 11 doi: 10.1016/S0140-6736(17)33108-2. PMID: 29241979Free PMC Article

Prognosis

GBD 2021 Diseases and Injuries Collaborators
Lancet 2024 May 18;403(10440):2133-2161. Epub 2024 Apr 17 doi: 10.1016/S0140-6736(24)00757-8. PMID: 38642570Free PMC Article
GBD 2021 Causes of Death Collaborators
Lancet 2024 May 18;403(10440):2100-2132. Epub 2024 Apr 3 doi: 10.1016/S0140-6736(24)00367-2. PMID: 38582094Free PMC Article
Wang M, Munoz J, Goy A, Locke FL, Jacobson CA, Hill BT, Timmerman JM, Holmes H, Jaglowski S, Flinn IW, McSweeney PA, Miklos DB, Pagel JM, Kersten MJ, Bouabdallah K, Khanal R, Topp MS, Houot R, Beitinjaneh A, Peng W, Fang X, Shen RR, Siddiqi R, Kloos I, Reagan PM
J Clin Oncol 2023 Jan 20;41(3):555-567. Epub 2022 Jun 4 doi: 10.1200/JCO.21.02370. PMID: 35658525Free PMC Article
Jain P, Wang M
Am J Hematol 2019 Jun;94(6):710-725. Epub 2019 Apr 19 doi: 10.1002/ajh.25487. PMID: 30963600
Flinn IW, van der Jagt R, Kahl B, Wood P, Hawkins T, MacDonald D, Simpson D, Kolibaba K, Issa S, Chang J, Trotman J, Hallman D, Chen L, Burke JM
J Clin Oncol 2019 Apr 20;37(12):984-991. Epub 2019 Feb 27 doi: 10.1200/JCO.18.00605. PMID: 30811293Free PMC Article

Clinical prediction guides

Hermine O, Jiang L, Walewski J, Bosly A, Thieblemont C, Szymczyk M, Pott C, Salles G, Feugier P, Hübel K, Haioun C, Casasnovas RO, Schmidt C, Bouabdallah K, Ribrag V, Kanz L, Dürig J, Metzner B, Sibon D, Cheminant M, Burroni B, Klapper W, Hiddemann W, Unterhalt M, Hoster E, Dreyling M; European Mantle Cell Lymphoma Network
J Clin Oncol 2023 Jan 20;41(3):479-484. Epub 2022 Dec 5 doi: 10.1200/JCO.22.01780. PMID: 36469833
Eskelund CW, Kolstad A, Jerkeman M, Räty R, Laurell A, Eloranta S, Smedby KE, Husby S, Pedersen LB, Andersen NS, Eriksson M, Kimby E, Bentzen H, Kuittinen O, Lauritzsen GF, Nilsson-Ehle H, Ralfkiaer E, Ehinger M, Sundström C, Delabie J, Karjalainen-Lindsberg ML, Workman CT, Garde C, Elonen E, Brown P, Grønbaek K, Geisler CH
Br J Haematol 2016 Nov;175(3):410-418. Epub 2016 Jul 5 doi: 10.1111/bjh.14241. PMID: 27378674
Cheson BD, Fisher RI, Barrington SF, Cavalli F, Schwartz LH, Zucca E, Lister TA; Alliance, Australasian Leukaemia and Lymphoma Group; Eastern Cooperative Oncology Group; European Mantle Cell Lymphoma Consortium; Italian Lymphoma Foundation; European Organisation for Research; Treatment of Cancer/Dutch Hemato-Oncology Group; Grupo Español de Médula Ósea; German High-Grade Lymphoma Study Group; German Hodgkin's Study Group; Japanese Lymphorra Study Group; Lymphoma Study Association; NCIC Clinical Trials Group; Nordic Lymphoma Study Group; Southwest Oncology Group; United Kingdom National Cancer Research Institute
J Clin Oncol 2014 Sep 20;32(27):3059-68. doi: 10.1200/JCO.2013.54.8800. PMID: 25113753Free PMC Article
Wang ML, Rule S, Martin P, Goy A, Auer R, Kahl BS, Jurczak W, Advani RH, Romaguera JE, Williams ME, Barrientos JC, Chmielowska E, Radford J, Stilgenbauer S, Dreyling M, Jedrzejczak WW, Johnson P, Spurgeon SE, Li L, Zhang L, Newberry K, Ou Z, Cheng N, Fang B, McGreivy J, Clow F, Buggy JJ, Chang BY, Beaupre DM, Kunkel LA, Blum KA
N Engl J Med 2013 Aug 8;369(6):507-16. Epub 2013 Jun 19 doi: 10.1056/NEJMoa1306220. PMID: 23782157Free PMC Article
Hoster E, Dreyling M, Klapper W, Gisselbrecht C, van Hoof A, Kluin-Nelemans HC, Pfreundschuh M, Reiser M, Metzner B, Einsele H, Peter N, Jung W, Wörmann B, Ludwig WD, Dührsen U, Eimermacher H, Wandt H, Hasford J, Hiddemann W, Unterhalt M; German Low Grade Lymphoma Study Group (GLSG); European Mantle Cell Lymphoma Network
Blood 2008 Jan 15;111(2):558-65. Epub 2007 Oct 25 doi: 10.1182/blood-2007-06-095331. PMID: 17962512

Recent systematic reviews

Cordas Dos Santos DM, Tix T, Shouval R, Gafter-Gvili A, Alberge JB, Cliff ERS, Theurich S, von Bergwelt-Baildon M, Ghobrial IM, Subklewe M, Perales MA, Rejeski K
Nat Med 2024 Sep;30(9):2667-2678. Epub 2024 Jul 8 doi: 10.1038/s41591-024-03084-6. PMID: 38977912Free PMC Article
Wu JJ, Wade SW, Itani T, Castaigne JG, Kloos I, Peng W, Kanters S, Zoratti MJ, Dreyling M, Shah B, Wang M
Leuk Lymphoma 2024 Nov;65(11):1609-1622. Epub 2024 Jul 8 doi: 10.1080/10428194.2024.2369653. PMID: 38975903
Roufarshbaf M, Javeri M, Akbari V, Matin PH, Farrokhi P, Sadeghi E, Heidari Z, Moghaddas A
Daru 2022 Dec;30(2):367-378. Epub 2022 Sep 3 doi: 10.1007/s40199-022-00444-w. PMID: 36057010Free PMC Article
Hilal T, Wang Z, Almader-Douglas D, Rosenthal A, Reeder CB, Jain T
Am J Hematol 2018 Oct;93(10):1220-1226. Epub 2018 Sep 3 doi: 10.1002/ajh.25226. PMID: 30033656
Parrott M, Rule S, Kelleher M, Wilson J
Clin Lymphoma Myeloma Leuk 2018 Jan;18(1):13-25.e6. Epub 2017 Oct 13 doi: 10.1016/j.clml.2017.10.004. PMID: 29122536

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) B-Cell Lymphomas, 2024

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