Zusammenfassung
Hautlymphome umfassen ein breites Spektrum an prognostisch unterschiedlichen Subtypen, wobei T-Zell-Lymphome die häufigste Untergruppe darstellen. Für die endgültige Diagnosestellung ist eine sorgfältige klinisch-pathologische Korrelation entscheidend. Das häufigste Hautlymphom stellt die Mycosis fungoides dar, welche in Abhängigkeit des Krankheitsstadiums durch Patches, Plaques, Tumoren oder eine universelle Rötung des Hautorgans (= Erythrodermie) gekennzeichnet ist. Während in frühen Stadien bei der Mycosis fungoides meist hautgerichtete Therapiemaßnahmen ausreichen, sind in fortgeschrittenen Stadien und beim Sézary-Syndrom Systemtherapien oder multimodale und interdisziplinäre Therapiekonzepte indiziert. Zu den kutanen T-Zell-Lymphomen zählen des Weiteren indolente Subtypen mit meist solitärer Hautmanifestation wie die CD4-positive Lymphoproliferation oder das akrale CD8-positive Lymphom, zum anderen jedoch auch seltene aggressive Entitäten wie das periphere T-Zell-Lymphom oder das aggressive epidermotrope T-Zell-Lymphom. Kutane B-Zell-Lymphome sind selten. Während die Lebenserwartung beim kutanen Marginalzonenlymphom und kutanen follikulären B-Zell-Lymphom – trotz häufig kutaner Rezidive im Krankheitsverlauf – nahezu unbeeinträchtigt ist, zeigt das kutane großzellige B-Zell-Lymphom ein aggressives biologisches Verhalten.
Abbreviations
- R-CHOP:
-
Rituximab, Cyclophosphamid, Doxorubicin, Vincristin, Predniso(lo)n
- WHO:
-
World Health Organization
- EORTC:
-
European Organization for Research and Treatment of Cancer
- CLA:
-
kutanes Lymphozytenantigen
- CCR4:
-
C-C-Chemokinrezeptor Typ 4
- NFkB:
-
nuclear factor kappa B
- JAK:
-
Januskinase
- STAT:
-
signal transducers and activators of transcription
- MAPK:
-
mitogen-activated protein kinase
Literatur
Agar NS, Wedgeworth E, Crichton S, Mitchell TJ, Cox M, Ferreira S, Robson A, Calonje E, Stefanato CM, Wain EM, Wilkins B, Fields PA, Dean A, Webb K, Scarisbrick J, Morris S, Whittaker SJ (2010) Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: validation of the revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer staging proposal. J Clin Oncol 28:4730–4739
Barasch NJK, Liu YC, Ho J, Bailey N, Aggarwal N, Cook JR, Swerdlow SH (2020) The molecular landscape and other distinctive features of primary cutaneous follicle center lymphoma. Hum Pathol 106:93–105
Campbell JJ, Clark RA, Watanabe R, Kupper TS (2010) Sezary syndrome and mycosis fungoides arise from distinct T-cell subsets: a biologic rationale for their distinct clinical behaviors. Blood 116:767–771
Choi J, Goh G, Walradt T, Hong BS, Bunick CG, Chen K, Bjornson RD, Maman Y, Wang T, Tordoff J, Carlson K, Overton JD, Liu KJ, Lewis JM, Devine L, Barbarotta L, Foss FM, Subtil A, Vonderheid EC, Edelson RL, Schatz DG, Boggon TJ, Girardi M, Lifton RP (2015) Genomic landscape of cutaneous T cell lymphoma. Nat Genet 47:1011–1019
Dippel E, Assaf C, Becker JC, von Bergwelt-Baildon M, Bernreiter S, Cozzio A, Eich HT, Elsayad K, Follmann M, Grabbe S, Hillen U, Klapper W, Klemke CD, Loquai C, Meiss F, Mitteldorf C, Wehkamp U, Nashan D, Nicolay JP, Oschlies I, Schlaak M, Stranzenbach R, Moritz R, Stoll C, Vag T, Weichenthal M, Wobser M, Stadler R (2022) S2k-Guidelines – Cutaneous lymphomas (ICD10 C82 - C86): Update 2021. J Dtsch Dermatol Ges 20:537–554
Dummer R, Vermeer MH, Scarisbrick JJ, Kim YH, Stonesifer C, Tensen CP, Geskin LJ, Quaglino P, Ramelyte E (2021) Cutaneous T cell lymphoma. Nat Rev Dis Primers 7:61
Grange F, Joly P, Barbe C, Bagot M, Dalle S, Ingen-Housz-Oro S, Maubec E, D'Incan M, Ram-Wolff C, Dalac S, Templier I, Esteve E, Quereux G, Machet L, Leduc M, Dereure O, Laroche L, Saiag P, Vergier B, Beylot-Barry M (2014) Improvement of survival in patients with primary cutaneous diffuse large B-cell lymphoma, leg type, in France. JAMA Dermatol 150:535–541
Horna P, Moscinski LC, Sokol L, Shao H (2019) Naïve/memory T-cell phenotypes in leukemic cutaneous T-cell lymphoma: putative cell of origin overlaps disease classification. Cytometry B Clin Cytom 96:234–241
Hristov AC, Tejasvi T, Wilcox RA (2019) Mycosis fungoides and Sézary syndrome: 2019 update on diagnosis, risk-stratification, and management. Am J Hematol 94(9):1027–1041. https://doi.org/10.1002/ajh.25577
Hughes CF, Khot A, McCormack C, Lade S, Westerman DA, Twigger R, Buelens O, Newland K, Tam C, Dickinson M, Ryan G, Ritchie D, Wood C, Prince HM (2015) Lack of durable disease control with chemotherapy for mycosis fungoides and Sézary syndrome: a comparative study of systemic therapy. Blood 125:71–81
Humme D, Nast A, Erdmann R, Vandersee S, Beyer M (2014) Systematic review of combination therapies for mycosis fungoides. Cancer Treat Rev 40:927–933
Jonak C, Tittes J, Brunner PM, Guenova E (2021) Mycosis fungoides and Sézary syndrome. J Dtsch Dermatol Ges 19:1307–1334
Kempf W, Pfaltz K, Vermeer MH, Cozzio A, Ortiz-Romero PL, Bagot M, Olsen E, Kim YH, Dummer R, Pimpinelli N, Whittaker S, Hodak E, Cerroni L, Berti E, Horwitz S, Prince HM, Guitart J, Estrach T, Sanches JA, Duvic M, Ranki A, Dreno B, Ostheeren-Michaelis S, Knobler R, Wood G, Willemze R (2011) EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Blood 118:4024–4035
Kempf W, Kerl K, Mitteldorf C (2018) Cutaneous CD30-positive T-cell lymphoproliferative disorders-clinical and histopathologic features, differential diagnosis, and treatment. Semin Cutan Med Surg 37:24–29
Kempf W, Mitteldorf C, Battistella M, Willemze R, Cerroni L, Santucci M, Geissinger E, Jansen P, Vermeer MH, Marschalko M, Papadavid E, Piris MA, Ortiz-Romero PL, Novelli M, Paulli M, Quaglino P, Ranki A, Rodríguez Peralto JL, Wobser M, Auschra B, Robson A (2021) Primary cutaneous peripheral T-cell lymphoma, not otherwise specified: results of a multicentre European Organization for Research and Treatment of Cancer (EORTC) cutaneous lymphoma taskforce study on the clinico-pathological and prognostic features. J Eur Acad Dermatol Venereol 35:658–668
Kim YH, Willemze R, Pimpinelli N, Whittaker S, Olsen EA, Ranki A, Dummer R, Hoppe RT (2007) TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC). Blood 110:479–484
Kim YH, Bagot M, Pinter-Brown L, Rook AH, Porcu P, Horwitz SM, Whittaker S, Tokura Y, Vermeer M, Zinzani PL, Sokol L, Morris S, Kim EJ, Ortiz-Romero PL, Eradat H, Scarisbrick J, Tsianakas A, Elmets C, Dalle S, Fisher DC, Halwani A, Poligone B, Greer J, Fierro MT, Khot A, Moskowitz AJ, Musiek A, Shustov A, Pro B, Geskin LJ, Dwyer K, Moriya J, Leoni M, Humphrey JS, Hudgens S, Grebennik DO, Tobinai K, Duvic M (2018) Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial. Lancet Oncol 19:1192–1204
Krenitsky A, Klager S, Hatch L, Sarriera-Lazaro C, Chen PL, Seminario-Vidal L (2022) Update in diagnosis and management of primary cutaneous B-Cell lymphomas. Am J Clin Dermatol 23:689–706
Mareschal S, Pham-Ledard A, Viailly PJ, Dubois S, Bertrand P, Maingonnat C, Fontanilles M, Bohers E, Ruminy P, Tournier I, Courville P, Lenormand B, Duval AB, Andrieu E, Verneuil L, Vergier B, Tilly H, Joly P, Frebourg T, Beylot-Barry M, Merlio JP, Jardin F (2017) Identification of somatic mutations in primary cutaneous diffuse large B-Cell lymphoma, leg type by massive parallel sequencing. J Invest Dermatol 137:1984–1994
Maurus K, Appenzeller S, Roth S, Kuper J, Rost S, Meierjohann S, Arampatzi P, Goebeler M, Rosenwald A, Geissinger E, Wobser M (2018) Panel sequencing shows recurrent genetic FAS alterations in primary cutaneous marginal zone lymphoma. J Invest Dermatol 138:1573–1581
McGirt LY, Jia P, Baerenwald DA, Duszynski RJ, Dahlman KB, Zic JA, Zwerner JP, Hucks D, Dave U, Zhao Z, Eischen CM (2015) Whole-genome sequencing reveals oncogenic mutations in mycosis fungoides. Blood 126:508–519
Mehta-Shah N, Horwitz SM, Ansell S, Ai WZ, Barnes J, Barta SK, Clemens MW, Dogan A, Fisher K, Goodman AM, Goyal G, Guitart J, Halwani A, Haverkos BM, Hoppe RT, Jacobsen E, Jagadeesh D, Lunning MA, Mehta A, Olsen EA, Pro B, Rajguru SA, Shanbhag S, Shaver A, Shustov A, Sokol L, Torka P, Torres-Cabala C, Wilcox R, William BM, Zain J, Dwyer MA, Sundar H, Kim YH (2020) NCCN guidelines insights: Primary cutaneous lymphomas, version 2.2020. J Natl Compr Cancer Netw 18:522–536
Melchers RC, Willemze R, Bekkenk MW, de Haas ERM, Horvath B, van Rossum MM, Sanders CJG, Jcjm Veraart H, Putter PM, Jansen MH, Vermeer, K. D. Quint. (2020) Frequency and prognosis of associated malignancies in 504 patients with lymphomatoid papulosis. J Eur Acad Dermatol Venereol 34:260–266
Molloy K, Jonak C, Woei AJF, Guenova E, Busschots AM, Bervoets A, Hauben E, Knobler R, Porkert S, Fassnacht C, Cowan R, Papadavid E, Beylot-Barry M (2020) Characteristics associated with significantly worse quality of life in mycosis fungoides/Sézary syndrome from the Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study. Br J Dermatol 182:770–779
Nicolay JP, Wobser M (2016) Cutaneous B-cell lymphomas – pathogenesis, diagnostic workup, and therapy. J Dtsch Dermatol Ges 14:1207–1224
Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, Zackheim H, Duvic M, Estrach T, Lamberg S, Wood G, Dummer R, Ranki A, Burg G, Heald P, Pittelkow M, Bernengo MG, Sterry W, Laroche L, Trautinger F, Whittaker S (2007) Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood 110:1713–1722
Pham-Ledard A, Beylot-Barry M, Barbe C, Leduc M, Petrella T, Vergier B, Martinez F, Cappellen D, Merlio JP, Grange F (2014) High frequency and clinical prognostic value of MYD88 L265P mutation in primary cutaneous diffuse large B-cell lymphoma, leg-type. JAMA Dermatol 150:1173–1179
Plumptre IR, Said JT, Sun T, Larocca C, Virgen CA, Kupper TS, Fisher DC, Devlin PM, Elco CP, Song JS, LeBoeuf NR (2022) Clinical features and treatment outcomes for primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder: a retrospective cohort study from the Dana-Farber Cancer Institute and updated literature review. Leuk Lymphoma 63:1–15
Prince HM, Kim YH, Horwitz SM, Dummer R, Scarisbrick J, Quaglino P, Zinzani PL, Wolter P, Sanches JA, Ortiz-Romero PL, Akilov OE, Geskin L, Trotman J, Taylor K, Dalle S, Weichenthal M, Walewski J, Fisher D, Dréno B, Stadler R, Feldman T, Kuzel TM, Wang Y, Palanca-Wessels MC, Zagadailov E, Trepicchio WL, Zhang W, Lin HM, Liu Y, Huebner D, Little M, Whittaker S, Duvic M (2017) Brentuximab vedotin or physician’s choice in CD30-positive cutaneous T-cell lymphoma (ALCANZA): an international, open-label, randomised, phase 3, multicentre trial. Lancet 390:555–566
Quaglino P, Prince HM, Cowan R, Vermeer M, Papadavid E, Bagot M, Servitjie O, Berti E, Guenova E, Stadler R, Querfeld C, Busschots AM, Hodak E, Patsatsi A, Sanches J, Maule M, Yoo J, Kevin M, Fava P, Ribero S, Zocchi L, Rubatto M, Fierro MT, Wehkamp U, Marshalko M, Mitteldorf C, Akilov O, Ortiz-Romero P, Estrach T, Vakeva L, Enz PA, Wobser M, Bayne M, Jonak C, Rubeta M, Forbes A, Bates A, Battistella M, Amel-Kashipaz R, Vydianath B, Combalia A, Georgiou E, Hauben E, Hong EK, Jost M, Knobler R, Amitay-Laish I, Miyashiro D, Cury-Martins J, Martinez X, Muniesa C, Prag-Naveh H, Stratigos A, Nikolaou V, Quint K, Ram-Wolff C, Rieger K, Stranzenbach R, Szepesi Á, Alberti-Violetti S, Felicity E, Cerroni L, Kempf W, Whittaker S, Willemze R, Kim Y, Scarisbrick JJ (2021) Treatment of early-stage mycosis fungoides: results from the PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study. Br J Dermatol 184:722–730
Roelens M, Delord M, Ram-Wolff C, Marie-Cardine A, Alberdi A, Maki G, Homyrda L, Bensussan A, Bagot M, Toubert A, Moins-Teisserenc H (2017) Circulating and skin-derived Sézary cells: clonal but with phenotypic plasticity. Blood 130:1468–1471
Scarisbrick JJ (2017) Prognostic factors in mycosis fungoides: international advances in the validation of prognostic indices. Br J Dermatol 176:1129–1130
Scarisbrick JJ, Prince HM, Vermeer MH, Quaglino P, Horwitz S, Porcu P, Stadler R, Wood GS, Beylot-Barry M, Pham-Ledard A, Foss F, Girardi M, Bagot M, Michel L, Battistella M, Guitart J, Kuzel TM, Martinez-Escala ME, Estrach T, Papadavid E, Antoniou C, Rigopoulos D, Nikolaou V, Sugaya M, Miyagaki T, Gniadecki R, Sanches JA, Cury-Martins J, Miyashiro D, Servitje O, Muniesa C, Berti E, Onida F, Corti L, Hodak E, Amitay-Laish I, Ortiz-Romero PL, Rodríguez-Peralto JL, Knobler R, Porkert S, Bauer W, Pimpinelli N, Grandi V, Cowan R, Rook A, Kim E, Pileri A, Patrizi A, Pujol RM, Wong H, Tyler K, Stranzenbach R, Querfeld C, Fava P, Maule M, Willemze R, Evison F, Morris S, Twigger R, Talpur R, Kim J, Ognibene G, Li S, Tavallaee M, Hoppe RT, Duvic M, Whittaker SJ, Kim YH (2015) Cutaneous lymphoma international consortium study of outcome in advanced stages of mycosis fungoides and sézary syndrome: effect of specific prognostic markers on survival and development of a prognostic model. J Clin Oncol 33:3766–3773
Scarisbrick JJ, Quaglino P, Prince HM, Papadavid E, Hodak E, Bagot M, Servitje O, Berti E, Ortiz-Romero P, Stadler R (2019) The PROCLIPI international registry of early-stage mycosis fungoides identifies substantial diagnostic delay in most patients. Br J Dermatol 181:350–357
Senff NJ, Hoefnagel JJ, Jansen PM, Vermeer MH, van Baarlen J, Blokx WA, Canninga-van Dijk MR, Geerts ML, Hebeda KM, Kluin PM, Lam KH, Meijer CJ, Willemze R (2007) Reclassification of 300 primary cutaneous B-Cell lymphomas according to the new WHO-EORTC classification for cutaneous lymphomas: comparison with previous classifications and identification of prognostic markers. J Clin Oncol 25:1581–1587
Trautinger F, Eder J, Assaf C, Bagot M, Cozzio A, Dummer R, Gniadecki R, Klemke CD, Ortiz-Romero PL, Papadavid E, Pimpinelli N, Quaglino P, Ranki A, Scarisbrick J, Stadler R, Väkevä L, Vermeer MH, Whittaker S, Willemze R, Knobler R (2017) European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome – Update 2017. Eur J Cancer 77:57–74
Vela V, Juskevicius D, Dirnhofer S, Menter T, Tzankov A (2021) Mutational landscape of marginal zone B-cell lymphomas of various origin: organotypic alterations and diagnostic potential for assignment of organ origin. Virchows Arch 480(2):403–413
Wang L, Ni X, Covington KR, Yang BY, Shiu J, Zhang X, ** L, Meng Q, Langridge T, Drummond J, Donehower LA, Doddapaneni H, Muzny DM, Gibbs RA, Wheeler DA, Duvic M (2015) Genomic profiling of Sezary syndrome identifies alterations of key T cell signaling and differentiation genes. Nat Genet 47:1426–1434
Willemze R, Hodak E, Zinzani PL, Specht L, Ladetto M (2018) Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 29:iv30-iv40
Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, Jaffe ES (2019) The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood 133:1703–1714
Wobser M, Goebeler M (2020) Cutaneous lymphomas: clinical presentation – diagnosis – treatment. Pathologe 41:79–94
Wobser M, Roth S, Reinartz T, Rosenwald A, Goebeler M, Geissinger E (2015) CD68 expression is a discriminative feature of indolent cutaneous CD8-positive lymphoid proliferation and distinguishes this lymphoma subtype from other CD8-positive cutaneous lymphomas. Br J Dermatol 172:1573–1580
Wobser M, Roth S, Appenzeller S, Kneitz H, Goebeler M, Geissinger E, Rosenwald A, Maurus K (2021) Oncogenic mutations and gene fusions in CD30-positive lymphoproliferations and clonally related mycosis fungoides occurring in the same patients. JID Innovations 1:100034
Woo DK, Jones CR, Vanoli-Storz MN, Kohler S, Reddy S, Advani R, Hoppe RT, Kim YH (2009) Prognostic factors in primary cutaneous anaplastic large cell lymphoma: characterization of clinical subset with worse outcome. Arch Dermatol 145:667–674
Woollard WJ, Pullabhatla V, Lorenc A, Patel VM, Butler RM, Bayega A, Begum N, Bakr F, Dedhia K, Fisher J, Aguilar-Duran S, Flanagan C, Ghasemi AA, Hoffmann RM, Castillo-Mosquera N, Nuttall EA, Paul A, Roberts CA, Solomonidis EG, Tarrant R, Yoxall A, Beyers CZ, Ferreira S, Tosi I, Simpson MA, de Rinaldis E, Mitchell TJ, Whittaker SJ (2016) Candidate driver genes involved in genome maintenance and DNA repair in Sézary syndrome. Blood 127:3387–3397
Zhang Y, LeWitt TM, Louissaint A Jr, Guitart J, Zhou XA, Choi J (2022) Disease-defining molecular features of primary cutaneous B-Cell lymphomas: implications for classification and treatment. J Invest Dermatol 143(2):189–196
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Section Editor information
Rights and permissions
Copyright information
© 2023 Springer-Verlag GmbH Deutschland, ein Teil von Springer Nature
About this entry
Cite this entry
Wobser, M. (2023). Kutane Lymphome. In: Lehnert, H., Märker-Hermann, E., Marx, N., Meyhöfer, S.M. (eds) DGIM Innere Medizin. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-54676-1_514-2
Download citation
DOI: https://doi.org/10.1007/978-3-642-54676-1_514-2
Received:
Accepted:
Published:
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-54676-1
Online ISBN: 978-3-642-54676-1
eBook Packages: Springer Referenz Medizin
Publish with us
Chapter history
-
Latest
Kutane Lymphome- Published:
- 19 April 2023
DOI: https://doi.org/10.1007/978-3-642-54676-1_514-2
-
Original
Primär kutane Lymphome- Published:
- 06 June 2015
DOI: https://doi.org/10.1007/978-3-642-54676-1_514-1