Mycosis fungoides natural history, complications and prognosis: Difference between revisions

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Staging for cutaneous T cell lymphoma(Mycosis fungoides (MF) and Sezary syndrome have same critera for staging) is provided in the following table:
Staging for cutaneous T cell lymphoma(Mycosis fungoides (MF) and Sezary syndrome have same critera for staging) is provided in the following table:
* The staging of sezazry syndrome is based on the TNMB:<ref name="TrautingerEder2017">{{cite journal|last1=Trautinger|first1=Franz|last2=Eder|first2=Johanna|last3=Assaf|first3=Chalid|last4=Bagot|first4=Martine|last5=Cozzio|first5=Antonio|last6=Dummer|first6=Reinhard|last7=Gniadecki|first7=Robert|last8=Klemke|first8=Claus-Detlev|last9=Ortiz-Romero|first9=Pablo L.|last10=Papadavid|first10=Evangelia|last11=Pimpinelli|first11=Nicola|last12=Quaglino|first12=Pietro|last13=Ranki|first13=Annamari|last14=Scarisbrick|first14=Julia|last15=Stadler|first15=Rudolf|last16=Väkevä|first16=Liisa|last17=Vermeer|first17=Maarten H.|last18=Whittaker|first18=Sean|last19=Willemze|first19=Rein|last20=Knobler|first20=Robert|title=European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome – Update 2017|journal=European Journal of Cancer|volume=77|year=2017|pages=57–74|issn=09598049|doi=10.1016/j.ejca.2017.02.027}}</ref>
* Sezary syndrome is defined by [[Enterobacteria phage T4|T4]] [[erythroderma]] of [[body surface area]] (BSA) more than of 80 percent, Sezary [[Cell (biology)|cell]] is more than 1000 cells/microL in B2 involvement of peripheral [[blood]] staged  of Sezary syndrome is based on the presence of [[Nodal marginal zone lymphoma|nodal]] and/or [[visceral]] involvement<ref name="pmid17540844">{{cite journal |vauthors=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 |title=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) |journal=Blood |volume=110 |issue=6 |pages=1713–22 |date=September 2007 |pmid=17540844 |doi=10.1182/blood-2007-03-055749 |url=}}</ref>
{| class="wikitable"
{| class="wikitable"
|+
|+
! colspan="2" |Staging for mycosis fungoides and Sezary syndrome  
!colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;"|Staging for mycosis fungoides and Sezary syndrome  
|-
|-
| colspan="2" |Skin(T)
!colspan="3" style="background: #B0C4DE; color: #FFFFFF; text-align: center;"|'''[[Skin]] (T)'''
|-
|-
|T1
| align="center" style="background:#ADD8E6;" |T1
|Limited patches, papules, and/or plaques covering <10% of the skin surface. May further stratify into T1a (patch only) versus T1b (plaque  patch)
|Limited patches, [[Papule|papules]], and/or [[Plaque|plaques]] covering <10% of the [[skin]] [[Surface area|surface]]. May further stratify into T1a ([[Patched|patch]] only) versus T1b ([[plaque]] [[Patched|patch]])
|-
|-
|T2
| align="center" style="background:#ADD8E6;"|T2
|Patches, papules, or plaques covering 10% of the skin surface. May further stratify into T2a (patch only) versus T2b (plaque  patch).
|Patches, [[Papule|papules]], [[or]] [[Plaque|plaques]] covering 10% of the [[skin]] [[Surface area|surface]]. May further stratify into T2a (patch only) versus T2b ([[plaque]] patch).
|-
|-
|T3
| align="center" style="background:#ADD8E6;"|T3
|One or more tumours (1-cm diameter)
|One or more [[Tumor|tumours]] (1-cm diameter)
|-
|-
|T4
| align="center" style="background:#ADD8E6;"|T4
|Confluence of erythema covering 80% body surface area
|Confluence of [[erythema]] covering 80% [[body surface area]]
|-
|-
| colspan="2" |Node(N)
!colspan="3" style="background: #B0C4DE; color: #FFFFFF; text-align: center;"|'''Node (N)'''
|-
|-
|N0
| align="center" style="background:#ADD8E6;"|N0
|No clinically abnormal peripheral lymph nodes; biopsy not required
|No [[Clinical|clinically]] [[abnormal]] [[T-cell lymphoma classification|peripheral]] [[Lymph node|lymph nodes]]; [[biopsy]] not required
|-
|-
|N1
| align="center" style="background:#ADD8E6;"|N1
|Clinically abnormal lymph nodes; histopathology Dutch grade 1 or NCI LN0-2
|Clinically [[abnormal]] [[Lymph node|lymph nodes]]; [[histopathology]] Dutch grade 1 or [[NCI]] LN0-2
|-
|-
|N1a
| align="center" style="background:#ADD8E6;"|N1a
|Clone negative
|[[Clone]] negative
|-
|-
|N1b
| align="center" style="background:#ADD8E6;"|N1b
|Clone posetive
|[[Clone]] posetive
|-
|-
|N2
| align="center" style="background:#ADD8E6;"|N2
|Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3
|[[Clinical|Clinically]] [[abnormal]] [[T-cell lymphoma classification|peripheral]] [[Lymph node|lymph nodes]]; [[histopathology]] Dutch grade 2 or [[NCI]] LN3
|-
|-
|N2a
| align="center" style="background:#ADD8E6;"|N2a
|Clone negatove
|[[Clone]] negatove
|-
|-
|N2b
| align="center" style="background:#ADD8E6;"|N2b
|Clone posetive
|[[Clone]] posetive
|-
|-
|N3
| align="center" style="background:#ADD8E6;"|N3
|Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3e4 or NCI LN4; clone positive or negative
|[[Clinical|Clinically]] [[abnormal]] [[T-cell lymphoma classification|peripheral]] [[Lymph node|lymph nodes]]; [[histopathology]] Dutch grades 3e4 or [[NCI]] LN4; [[clone]] positive or negative
|-
|-
|NX
| align="center" style="background:#ADD8E6;"|NX
|Clinically abnormal peripheral lymph nodes; no histologic confirmation
|[[Clinical|Clinically]] [[abnormal]] [[T-cell lymphoma classification|peripheral]] [[Lymph node|lymph nodes]]; no [[histologic]] confirmation
|-
|-
| colspan="2" |Visceral(M)
!colspan="3" style="background: #B0C4DE; color: #FFFFFF; text-align: center;"|[[Visceral|'''Visceral''']] ('''M''')
|-
|-
|M0
| align="center" style="background:#ADD8E6;"|M0
|No visceral organ involvement
|No [[visceral]] [[Organ (anatomy)|organ]] involvement
|-
|-
|M1
| align="center" style="background:#ADD8E6;"|M1
|Visceral involvement (must have pathology confirmation and organ involved should be specified)
|[[Visceral]] involvement (must have [[pathology]] confirmation and [[Organ (anatomy)|organ]] involved should be specified)
|-
|-
| colspan="2" |Blood
!colspan="3" style="background: #B0C4DE; color: #FFFFFF; text-align: center;"|[[Blood|'''Blood''']] '''(B)'''
|-
|-
|B0
| align="center" style="background:#ADD8E6;"|B0
|0 Absence of significant blood involvement: �5% of peripheral blood lymphocytes are atypical (Se´zary) cells B0a Clone negative B0b Clone positive
|0 Absence of significant [[blood]] involvement: 5% of [[T-cell lymphoma classification|peripheral]] [[blood]] [[Lymphocyte|lymphocytes]] are atypical (Sezary) [[Cell (biology)|cell]]<nowiki/>s B0a [[Clone]] negative B0b [[Clone (cell biology)|Clone]] positive
|-
|-
|B1
| align="center" style="background:#ADD8E6;"|B1
|Low blood tumour burden: >5% of peripheral blood lymphocytes are atypical (Se´zary) cells but does not meet the criteria of B2 B1a Clone negative B1b Clone positive
|Low [[blood]] [[Tumor|tumour]] burden: >5% of [[T-cell lymphoma classification|peripheral]] [[blood]] [[Lymphocyte|lymphocytes]] are atypical (Sezary) cells but does not meet the [[criteria]] of B2 B1a Clone negative B1b [[Clone (cell biology)|Clone]] positive
|-
|-
|B2
| align="center" style="background:#ADD8E6;"|B2
|High blood tumour burden: 1000/mL Se´zary cells with positive clone
|High [[blood]] tumour burden: 1000/mL Sezary [[Cell (biology)|cells]] with positive clone
|}
|}


The staging of Sezary syndrome is based on the clinical stages:<ref name="TrautingerEder2017" /><ref name="JawedMyskowski2014">{{cite journal|last1=Jawed|first1=Sarah I.|last2=Myskowski|first2=Patricia L.|last3=Horwitz|first3=Steven|last4=Moskowitz|first4=Alison|last5=Querfeld|first5=Christiane|title=Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome)|journal=Journal of the American Academy of Dermatology|volume=70|issue=2|year=2014|pages=205.e1–205.e16|issn=01909622|doi=10.1016/j.jaad.2013.07.049}}</ref>
The staging of [[Sezary syndrome]] is based on the [[clinical]] stages:<ref name="TrautingerEder2017" /><ref name="JawedMyskowski2014">{{cite journal|last1=Jawed|first1=Sarah I.|last2=Myskowski|first2=Patricia L.|last3=Horwitz|first3=Steven|last4=Moskowitz|first4=Alison|last5=Querfeld|first5=Christiane|title=Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome)|journal=Journal of the American Academy of Dermatology|volume=70|issue=2|year=2014|pages=205.e1–205.e16|issn=01909622|doi=10.1016/j.jaad.2013.07.049}}</ref>


{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
{| class="wikitable"
|+ '''Staging of cutaneous T cell lymphoma <ref name="canadiancancer">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</ref>'''
|+
! style="background: #4479BA; color:#FFF;" | Stage
! colspan="6" !colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;"|clinical stages
! style="background: #4479BA; color:#FFF;" | Involvement
|-
|-
| colspan="3" style="padding: 5px 5px; background: #DCDCDC;" | '''stage I'''
| align="center" style="background:#6495ED;" |Stage
!colspan="1" style="background: #B0C4DE; color:#FFFFFF; text-align: center;"|T
!colspan="1" style="background: #B0C4DE; color:#FFFFFF; text-align: center;"|N
!colspan="1" style="background: #B0C4DE; color:#FFFFFF; text-align: center;"|M
!colspan="1" style="background: #B0C4DE; color:#FFFFFF; text-align: center;"|B
!colspan="1" style="background: #B0C4DE; color:#FFFFFF; text-align: center;"|DDS
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | I A
| align="center" style="background:#ADD8E6;" |IA
| style="padding: 5px 5px; background: #F5F5F5;" |  
|1
* Less than 10% of the skin is covered with patches or plaques
|0
|0
|0/1
|98
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | I B
| align="center" style="background:#ADD8E6;" |IB
| style="padding: 5px 5px; background: #F5F5F5;" |  
|2
* 10% or more of the skin is covered with patches or plaques
|0
|0
|0/1
|89
|-
|-
| colspan="3" style="padding: 5px 5px; background: #DCDCDC;" | '''stage II'''
| align="center" style="background:#ADD8E6;" |IIA
|1.2
|1.2
|0
|0/1
|89
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | II A
| align="center" style="background:#ADD8E6;" |IIB
| style="padding: 5px 5px; background: #F5F5F5;" |  
|3
* Any amount of the skin is covered in patches or plaques
|0-2
* Lymph nodes are enlarged, but do not contain cancer
|0
|0/1
|56
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | II B
| align="center" style="background:#ADD8E6;" |IIIA
| style="padding: 5px 5px; background: #F5F5F5;" |  
|4
* There is one or more raised tumors on the skin
|0-2
* Lymph nodes may or may not be enlarged and do not contain cancer
|0
|0
|54
|-
|-
| colspan="3" style="padding: 5px 5px; background: #DCDCDC;" | '''stage III'''
| align="center" style="background:#ADD8E6;" |IIIB
|4
|0-2
|0
|1
|48
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | III
| align="center" style="background:#ADD8E6;" |IVA1
| style="padding: 5px 5px; background: #F5F5F5;" |  
|1-4
* Almost all of the skin is reddened
|0-2
* There may or may not be patches, plaques or skin tumors
|0
* Lymph nodes may or may not be enlarged and do not contain cancer
|2
|-
|41
| colspan="3" style="padding: 5px 5px; background: #DCDCDC;" | '''stage IV'''
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | IV A
| align="center" style="background:#ADD8E6;" |IVA2
| style="padding: 5px 5px; background: #F5F5F5;" |  
|1-4
* Cancer has spread to the lymph nodes, but not to other organs in the body
|3
|0
|0-2
|23
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | IV B
| align="center" style="background:#ADD8E6;" |IVB
| style="padding: 5px 5px; background: #F5F5F5;" |  
|1-4
|0-3
|1
|0-2
|18
|}
 
*[5-year [[disease]] free [[survival]] (DSS)]
* Cancer has spread to other organs in the body, including the blood and bone marrow
* Cancer has spread to other organs in the body, including the blood and bone marrow
* Lymph nodes may be enlarged and may contain cancer
* Lymph nodes may be enlarged and may contain cancer

Revision as of 18:16, 4 December 2018


Mycosis fungoides Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2], Sogand Goudarzi, MD [3]

Overview

If left untreated, cutaneous T cell lymphoma may progress to develop cutaneous patches, plaque, and tumors. Depending on the extent of the lymphoma at the time of diagnosis, the prognosis may vary.

Natural History

  • Mycosis fungoides is initially an indolent lymphoma that may later develop peripheral lymphadenopathy and can finally progress to widespread visceral involvement.[1]
  • Cutaneous T cell lymphoma is usually initially seen by dermatologists with patients presenting with skin lesions such as erythematous patches or plaque.
  • Patients often have a history of several years of eczematous or dermatitic skin lesions before the diagnosis is finally established.
  • The skin lesions then progress from the patch stage to the plaque stage to cutaneous tumors.

Complications

  • Common complications of Cutaneous T cell lymphoma include:
    • Mycosis Fungoides increased risk of secondary malignancies such as primary malignancy, especially Hodgkin lymphoma, chronic leukemia, and lung cancer.[2]
    • [Complication 2]
    • [Complication 3]

Prognosis

  • Cutaneous T cell lymphoma is usually a slow-growing (indolent) lymphoma.[3]
  • The prognosis for people with cutaneous T cell lymphoma is based on the extent of disease and how the person responds to treatment.
  • Although more advanced stages of cutaneous T cell lymphoma may not be cured, the lymphoma can still be controlled with treatment.

Favorable prognosis

  • Early stage disease
  • Lymphoma is confined to the skin

Unfavorable prognosis

  • More advanced disease
  • Lymphoma has spread to lymph nodes
  • Lymphoma has spread to other organs


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:

  • The staging of sezazry syndrome is based on the TNMB:[4]
  • Sezary syndrome is defined by T4 erythroderma of body surface area (BSA) more than of 80 percent, Sezary cell is more than 1000 cells/microL in B2 involvement of peripheral blood staged of Sezary syndrome is based on the presence of nodal and/or visceral involvement[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
  • [5-year disease free survival (DSS)]
  • Cancer has spread to other organs in the body, including the blood and bone marrow
  • Lymph nodes may be enlarged and may contain cancer

|}

References

  1. ↑ Mycosis fungoides. Radiopaedia.http://radiopaedia.org/articles/mycosis-fungoides Accessed on January 20, 2016
  2. ↑ Cengiz FP, Emiroğlu N, Onsun N (December 2017). "Frequency and Risk Factors for Secondary Malignancies in Patients with Mycosis Fungoides". Turk J Haematol. 34 (4): 378–379. doi:10.4274/tjh.2017.0234. PMC 5774354. PMID 28832009.
  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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