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Mycosis fungoides

MedGen UID:
7771
Concept ID:
C0026948
Neoplastic Process
Synonym: Granuloma fungoides
SNOMED CT: MF - Mycosis fungoides (118618005); Mycosis fungoides (90120004); Mycosis fungoides (clinical) (118618005); Mycosis fungoides (118618005)
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:0009691
OMIM®: 254400
Orphanet: ORPHA2584

Definition

Mycosis fungoides is a malignant T-cell lymphoma of the skin, first reported (and named) by Alibert (1835). Sezary syndrome is a leukemic variant of mycosis fungoides defined by erythroderma with greater than 80% of the skin showing redness, adenopathy and greater than 1,000 circulating Sezary cells/microliter with a CD4+CD26- or CD4+CD7- phenotype. Sezary cells have a type 2 helper T cell cytokine profile. Sezary syndrome has a median overall survival time of only 2.4 years in patients with Sezary cells at a density of greater than 10,000 cells/microliter or 5.4 years in patients with 1,000-10,000 Sezary cells/microliter. Mycosis fungoides and Sezary syndrome are the most common cutaneous T-cell lymphomas. Sezary syndrome can arise de novo or can appear following years of chronic mycosis fungoides. Both are thought to arise from clonal expansion of CD4+ helper T cells responding to chronic antigen stimulation (summary by Wang et al., 2015). [from OMIM]

Clinical features

From HPO
Lymphoma
MedGen UID:
44223
Concept ID:
C0024299
Neoplastic Process
A cancer originating in lymphocytes and presenting as a solid tumor of lymhpoid cells.
Neoplasm of the skin
MedGen UID:
19993
Concept ID:
C0037286
Neoplastic Process
A tumor (abnormal growth of tissue) of the skin.
Eczematoid dermatitis
MedGen UID:
3968
Concept ID:
C0013595
Disease or Syndrome
Eczema is a form of dermatitis that is characterized by scaly, pruritic, erythematous lesions located on flexural surfaces.
Psoriasiform dermatitis
MedGen UID:
75508
Concept ID:
C0262985
Disease or Syndrome
A skin abnormality characterized by redness and irritation, with thick, red skin that displays flaky, silver-white patches (scales).
Lymphadenopathy
MedGen UID:
96929
Concept ID:
C0497156
Disease or Syndrome
Enlargment (swelling) of a lymph node.
Pruritus
MedGen UID:
19534
Concept ID:
C0033774
Sign or Symptom
Pruritus is an itch or a sensation that makes a person want to scratch. This term refers to an abnormally increased disposition to experience pruritus.
Erythema
MedGen UID:
11999
Concept ID:
C0041834
Disease or Syndrome
Redness of the skin, caused by hyperemia of the capillaries in the lower layers of the skin.
Skin plaque
MedGen UID:
69134
Concept ID:
C0241148
Finding
A plaque is a solid, raised, plateau-like (flat-topped) lesion greater than 1 cm in diameter.

Professional guidelines

PubMed

Latzka J, Assaf C, Bagot M, Cozzio A, Dummer R, Guenova E, Gniadecki R, Hodak E, Jonak C, Klemke CD, Knobler R, Morrris S, Nicolay JP, Ortiz-Romero PL, Papadavid E, Pimpinelli N, Quaglino P, Ranki A, Scarisbrick J, Stadler R, Väkevä L, Vermeer MH, Wehkamp U, Whittaker S, Willemze R, Trautinger F
Eur J Cancer 2023 Dec;195:113343. Epub 2023 Sep 18 doi: 10.1016/j.ejca.2023.113343. PMID: 37890355
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Jan;98(1):193-209. Epub 2022 Oct 20 doi: 10.1002/ajh.26760. PMID: 36226409Free PMC Article
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2019 Sep;94(9):1027-1041. Epub 2019 Jul 31 doi: 10.1002/ajh.25577. PMID: 31313347

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Primary Cutaneous Lymphomas, 2024

Recent clinical studies

Etiology

Maguire A, Puelles J, Raboisson P, Chavda R, Gabriel S, Thornton S
Acta Derm Venereol 2020 Jan 7;100(1):adv00013. doi: 10.2340/00015555-3367. PMID: 31663598Free PMC Article
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2019 Sep;94(9):1027-1041. Epub 2019 Jul 31 doi: 10.1002/ajh.25577. PMID: 31313347
Larocca CA, LeBoeuf NR
Hematol Oncol Clin North Am 2019 Aug;33(4):669-686. Epub 2019 May 23 doi: 10.1016/j.hoc.2019.04.004. PMID: 31229162
Mitteldorf C, Kempf W
Surg Pathol Clin 2017 Jun;10(2):455-476. Epub 2017 Mar 22 doi: 10.1016/j.path.2017.01.002. PMID: 28477891
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; ISCL/EORTC
Blood 2007 Sep 15;110(6):1713-22. Epub 2007 May 31 doi: 10.1182/blood-2007-03-055749. PMID: 17540844

Diagnosis

Shou Y, Jing J
QJM 2023 Dec 27;116(12):1035-1036. doi: 10.1093/qjmed/hcad185. PMID: 37498541
Yu Y, Feng L
J Cutan Med Surg 2022 Jul-Aug;26(4):440. Epub 2021 Dec 7 doi: 10.1177/12034754211064311. PMID: 34875891
Hodak E, Amitay-Laish I
Clin Dermatol 2019 May-Jun;37(3):255-267. Epub 2019 Jan 11 doi: 10.1016/j.clindermatol.2019.01.004. PMID: 31178107
Mitteldorf C, Stadler R, Sander CA, Kempf W
J Dtsch Dermatol Ges 2018 May;16(5):543-557. Epub 2018 May 4 doi: 10.1111/ddg.13514. PMID: 29726638
Muñoz-González H, Molina-Ruiz AM, Requena L
Actas Dermosifiliogr 2017 Apr;108(3):192-208. Epub 2016 Nov 18 doi: 10.1016/j.ad.2016.08.009. PMID: 27871620

Therapy

Olsen EA, Whittaker S, Willemze R, Pinter-Brown L, Foss F, Geskin L, Schwartz L, Horwitz S, Guitart J, Zic J, Kim YH, Wood GS, Duvic M, Ai W, Girardi M, Gru A, Guenova E, Hodak E, Hoppe R, Kempf W, Kim E, Lechowicz MJ, Ortiz-Romero P, Papadavid E, Quaglino P, Pittelkow M, Prince HM, Sanches JA, Sugaya M, Vermeer M, Zain J, Knobler R, Stadler R, Bagot M, Scarisbrick J
Blood 2022 Aug 4;140(5):419-437. doi: 10.1182/blood.2021012057. PMID: 34758074Free PMC Article
Phillips D, Matusiak M, Gutierrez BR, Bhate SS, Barlow GL, Jiang S, Demeter J, Smythe KS, Pierce RH, Fling SP, Ramchurren N, Cheever MA, Goltsev Y, West RB, Khodadoust MS, Kim YH, Schürch CM, Nolan GP
Nat Commun 2021 Nov 18;12(1):6726. doi: 10.1038/s41467-021-26974-6. PMID: 34795254Free PMC Article
Kamijo H, Miyagaki T
Curr Treat Options Oncol 2021 Jan 7;22(2):10. doi: 10.1007/s11864-020-00809-w. PMID: 33415447
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; MAVORIC Investigators
Lancet Oncol 2018 Sep;19(9):1192-1204. Epub 2018 Aug 9 doi: 10.1016/S1470-2045(18)30379-6. PMID: 30100375
Olsen EA, Whittaker S, Kim YH, Duvic M, Prince HM, Lessin SR, Wood GS, Willemze R, Demierre MF, Pimpinelli N, Bernengo MG, Ortiz-Romero PL, Bagot M, Estrach T, Guitart J, Knobler R, Sanches JA, Iwatsuki K, Sugaya M, Dummer R, Pittelkow M, Hoppe R, Parker S, Geskin L, Pinter-Brown L, Girardi M, Burg G, Ranki A, Vermeer M, Horwitz S, Heald P, Rosen S, Cerroni L, Dreno B, Vonderheid EC; International Society for Cutaneous Lymphomas; United States Cutaneous Lymphoma Consortium; Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer
J Clin Oncol 2011 Jun 20;29(18):2598-607. Epub 2011 May 16 doi: 10.1200/JCO.2010.32.0630. PMID: 21576639Free PMC Article

Prognosis

Bhabha FK, McCormack C, Wells J, Campbell BA, Newland K, Lade S, Buelens O, Joske D, Shortt J, Mapp S, Radeski D, Hertzberg M, Khot A, Van Der Weyden C, Khoo C, Hawkes E, Prince HM
Australas J Dermatol 2021 Feb;62(1):e8-e18. Epub 2020 Dec 26 doi: 10.1111/ajd.13467. PMID: 33368169
Vonderheid EC, Hamilton RG, Kadin ME
Clin Lymphoma Myeloma Leuk 2021 Apr;21(4):279-288.e7. Epub 2020 Nov 12 doi: 10.1016/j.clml.2020.11.007. PMID: 33342729
Larocca C, Kupper T
Hematol Oncol Clin North Am 2019 Feb;33(1):103-120. doi: 10.1016/j.hoc.2018.09.001. PMID: 30497668Free PMC Article
Cerroni L
Semin Cutan Med Surg 2018 Mar;37(1):2-10. doi: 10.12788/j.sder.2018.002. PMID: 29719014
Rodney IJ, Kindred C, Angra K, Qutub ON, Villanueva AR, Halder RM
J Eur Acad Dermatol Venereol 2017 May;31(5):808-814. Epub 2016 Aug 12 doi: 10.1111/jdv.13843. PMID: 27515575

Clinical prediction guides

Boesjes CM, van der Gang LF, Bakker DS, Ten Cate TA, Spekhorst LS, de Graaf M, van Dijk MR, de Bruin-Weller MS
JAMA Dermatol 2023 Nov 1;159(11):1240-1247. doi: 10.1001/jamadermatol.2023.3849. PMID: 37851456Free PMC Article
Nielsen PR, Eriksen JO, Sørensen MD, Wehkamp U, Lindahl LM, Bzorek M, Iversen L, Woetman A, Ødum N, Litman T, Gjerdrum LMR
Acta Derm Venereol 2021 Mar 11;101(3):adv00413. doi: 10.2340/00015555-3775. PMID: 33686443Free PMC Article
Chen Y, Xu J, Qiu L, Fu L, Liang Y, Wei L, Xiang X, Wang Z, Xu Z, Ma L
Am J Dermatopathol 2021 Apr 1;43(4):259-265. doi: 10.1097/DAD.0000000000001723. PMID: 33201017
Rubio Gonzalez B, Zain J, Rosen ST, Querfeld C
Curr Opin Oncol 2016 Jan;28(1):88-96. doi: 10.1097/CCO.0000000000000243. PMID: 26632770
Zinzani PL, Ferreri AJ, Cerroni L
Crit Rev Oncol Hematol 2008 Feb;65(2):172-82. Epub 2007 Oct 22 doi: 10.1016/j.critrevonc.2007.08.004. PMID: 17950613

Recent systematic reviews

Jfri A, Smith JS, Larocca C
J Am Acad Dermatol 2023 May;88(5):1164-1166. Epub 2022 Dec 5 doi: 10.1016/j.jaad.2022.12.001. PMID: 36481378
Ottevanger R, van Beugen S, Evers AWM, Willemze R, Vermeer MH, Quint KD
J Eur Acad Dermatol Venereol 2021 Dec;35(12):2377-2387. Epub 2021 Aug 14 doi: 10.1111/jdv.17570. PMID: 34331819Free PMC Article
Jung JM, Lim DJ, Won CH, Chang SE, Lee MW, Lee WJ
JAMA Dermatol 2021 Apr 1;157(4):431-438. doi: 10.1001/jamadermatol.2021.0083. PMID: 33656521
Chu S, Michelle L, Ekelem C, Sung CT, Rojek N, Mesinkovska NA
Arch Dermatol Res 2021 Aug;313(6):391-430. Epub 2020 Nov 5 doi: 10.1007/s00403-020-02152-4. PMID: 33151346
Humme D, Nast A, Erdmann R, Vandersee S, Beyer M
Cancer Treat Rev 2014 Sep;40(8):927-33. Epub 2014 Jun 19 doi: 10.1016/j.ctrv.2014.06.005. PMID: 24997678

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Primary Cutaneous Lymphomas, 2024

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