Mycosis fungoides classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Overview

There are 3 classification methods used to classify cutaneous T cell lymphoma into several subtypes. Cutaneous T cell lymphoma may be classified into several subtypes based on WHO-EORTC classification.

Classification

According to world Health Organization (WHO) and European Organization for Research and Treatment of Cancer (EORTC) classification, mycosis fungoides may be classified into the following types:[1][2]


Cutaneous T cell lymphoma classification[3]
Name Description
Mycosis fungoides (MF)
  • Most common form of cutaneous T cell lymphoma
  • Starts in the skin in areas of the body not usually exposed to the sun
  • May appear as a scaly, pink patches on the skin
  • Signs can progress to the development of skin tumors in more advanced cases

Staging

  • The staging of cutaneous T cell lymphoma is based on skin and lymph node involvement.[3]
  • Staging for cutaneous T cell lymphoma(Mycosis fungoides (MF) and Sezary syndrome have same critera for staging) is provided in the following table:[4][5]
Staging for mycosis fungoides and Sezary syndrome
Skin (T)
T1 Limited patches, papules, and/or plaques covering <10% of the skin surface. May further stratify into T1a (patch only) versus T1b (plaque patch)
T2 Patches, papules, or plaques covering 10% of the skin surface. May further stratify into T2a (patch only) versus T2b (plaque patch).
T3 One or more tumours (1-cm diameter)
T4 Confluence of erythema covering 80% body surface area
Node (N)
N0 No clinically abnormal peripheral lymph nodes; biopsy not required
N1 Clinically abnormal lymph nodes; histopathology Dutch grade 1 or NCI LN0-2
N1a Clone negative
N1b Clone posetive
N2 Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3
N2a Clone negatove
N2b Clone posetive
N3 Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3e4 or NCI LN4; clone positive or negative
NX Clinically abnormal peripheral lymph nodes; no histologic confirmation
Visceral (M)
M0 No visceral organ involvement
M1 Visceral involvement (must have pathology confirmation and organ involved should be specified)
Blood (B)
B0 0 Absence of significant blood involvement: 5% of peripheral blood lymphocytes are atypical (Sezary) cells B0a Clone negative B0b Clone positive
B1 Low blood tumour burden: >5% of peripheral blood lymphocytes are atypical (Sezary) cells but does not meet the criteria of B2 B1a Clone negative B1b Clone positive
B2 High blood tumour burden: 1000/mL Sezary cells with positive clone

The staging of Sezary syndrome is based on the clinical stages:[4][6]

clinical stages
Stage T N M B DDS
IA 1 0 0 0/1 98
IB 2 0 0 0/1 89
IIA 1.2 1.2 0 0/1 89
IIB 3 0-2 0 0/1 56
IIIA 4 0-2 0 0 54
IIIB 4 0-2 0 1 48
IVA1 1-4 0-2 0 2 41
IVA2 1-4 3 0 0-2 23
IVB 1-4 0-3 1 0-2 18

References

  1. Matutes, E. (2018). "The 2017 WHO update on mature T- and natural killer (NK) cell neoplasms". International Journal of Laboratory Hematology. 40: 97–103. doi:10.1111/ijlh.12817. ISSN 1751-5521.
  2. Sundram, Uma (2018). "Cutaneous Lymphoproliferative Disorders". Advances In Anatomic Pathology: 1. doi:10.1097/PAP.0000000000000208. ISSN 1072-4109.
  3. 3.0 3.1 Cutaneous T cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/cutaneous-t-cell-lymphoma/?region=on Accessed on January 19, 2016
  4. 4.0 4.1 Trautinger, Franz; Eder, Johanna; Assaf, Chalid; Bagot, Martine; Cozzio, Antonio; Dummer, Reinhard; Gniadecki, Robert; Klemke, Claus-Detlev; Ortiz-Romero, Pablo L.; Papadavid, Evangelia; Pimpinelli, Nicola; Quaglino, Pietro; Ranki, Annamari; Scarisbrick, Julia; Stadler, Rudolf; Väkevä, Liisa; Vermeer, Maarten H.; Whittaker, Sean; Willemze, Rein; Knobler, Robert (2017). "European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome – Update 2017". European Journal of Cancer. 77: 57–74. doi:10.1016/j.ejca.2017.02.027. ISSN 0959-8049.
  5. 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 (September 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 (6): 1713–22. doi:10.1182/blood-2007-03-055749. PMID 17540844.
  6. Jawed, Sarah I.; Myskowski, Patricia L.; Horwitz, Steven; Moskowitz, Alison; Querfeld, Christiane (2014). "Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome)". Journal of the American Academy of Dermatology. 70 (2): 205.e1–205.e16. doi:10.1016/j.jaad.2013.07.049. ISSN 0190-9622.

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