Mycosis fungoides medical therapy: Difference between revisions

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__NOTOC__
__NOTOC__
{{Cutaneous T cell lymphoma}}
{{Mycosis fungoides}}
{{CMG}}; {{AE}} {{AS}}
 
{{CMG}}; {{AE}} {{S.G.}}, {{AS}}
==Overview==
==Overview==
The predominant therapy for cutaneous T cell lymphoma is [[PUVA]]. Adjunctive [[chemotherapy]], [[radiotherapy]], [[biological therapy]], retinoid therapy, and photophoresis may be required.<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>
The predominant therapy for [[cutaneous]] [[T-cell lymphoma|T cell lymphoma]] is [[PUVA]]. Adjunctive [[chemotherapy]], [[radiotherapy]], [[biological therapy]], [[retinoid]] [[therapy]], and photophoresis may be required.
==Medical Therapy==
==Medical Therapy==
* Patients with Mycosis fungoides are treated based on the stage and the previous treatment history.<ref name="pmid24421750" />
* Patients with Mycosis fungoides are treated based on the stage and the previous treatment history.<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><ref name="pmid24421750">{{cite journal |vauthors=Al Hothali GI |title=Review of the treatment of mycosis fungoides and Sézary syndrome: A stage-based approach |journal=Int J Health Sci (Qassim) |volume=7 |issue=2 |pages=220–39 |date=June 2013 |pmid=24421750 |pmc=3883611 |doi= |url=}}</ref><ref name="WillemzeHodak2018">{{cite journal|last1=Willemze|first1=R|last2=Hodak|first2=E|last3=Zinzani|first3=P L|last4=Specht|first4=L|last5=Ladetto|first5=M|title=Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up†|journal=Annals of Oncology|volume=29|issue=Supplement_4|year=2018|pages=iv30–iv40|issn=0923-7534|doi=10.1093/annonc/mdy133}}</ref><ref name="pmid9923777">{{cite journal |vauthors=Kim YH, Chow S, Varghese A, Hoppe RT |title=Clinical characteristics and long-term outcome of patients with generalized patch and/or plaque (T2) mycosis fungoides |journal=Arch Dermatol |volume=135 |issue=1 |pages=26–32 |date=January 1999 |pmid=9923777 |doi= |url=}}</ref><ref name="pmid244217502">{{cite journal |vauthors=Al Hothali GI |title=Review of the treatment of mycosis fungoides and Sézary syndrome: A stage-based approach |journal=Int J Health Sci (Qassim) |volume=7 |issue=2 |pages=220–39 |date=June 2013 |pmid=24421750 |pmc=3883611 |doi= |url=}}</ref><ref name="WillemzeHodak20182">{{cite journal|last1=Willemze|first1=R|last2=Hodak|first2=E|last3=Zinzani|first3=P L|last4=Specht|first4=L|last5=Ladetto|first5=M|title=Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up†|journal=Annals of Oncology|volume=29|issue=Supplement_4|year=2018|pages=iv30–iv40|issn=0923-7534|doi=10.1093/annonc/mdy133}}</ref>
* Mycosis fungoides treatment selection should be based on the stage and previous treatment history stage and previous treatment history
===Mycosis fungoides (Early stages)===
* Medical therapy for early stage MF is:
* Patients with mycosis fungoides in stage IA ( patches, plaques, may be papules involve <10 % of total skin surface) treat by skin-directed therapy (topical corticosteroids, nitrogen mustard) is recommended more than systemic therapy.<ref name="pmid24421750">{{cite journal |vauthors=Al Hothali GI |title=Review of the treatment of mycosis fungoides and Sézary syndrome: A stage-based approach |journal=Int J Health Sci (Qassim) |volume=7 |issue=2 |pages=220–39 |date=June 2013 |pmid=24421750 |pmc=3883611 |doi= |url=}}</ref>  
The predominant therapy for cutaneous T cell lymphoma is [[PUVA]]. Adjunctive [[chemotherapy]], [[radiotherapy]], [[biological therapy]], retinoid therapy, and photophoresis may be required. <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>
             
===Mycosis fungoides===


* '''1 Stage 1 -Mycosis fungoides'''
* '''Stage IA'''
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)''' 
** [[Patient|Patients]] with mycosis fungoides in stage IA ( [[Patched|patches]], [[Plaque|plaques]], may be [[Papule|papules]] involve <10 % of total [[skin]] [[Superficial anatomy|surface]]) treat by [[Skin|skin directed therapy]] [<nowiki/>[[topical]] [[Corticosteroid|corticosteroids]], [[nitrogen mustard]] (meclorethamin, NH2)].
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
** [[Skin]] direct [[therapy]] is recommended more than [[systemic]] [[therapy]] ([[chemotherapy]]+ [[skin]] direct [[therapy]]) in this stage.
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
** [[Systemic]] [[Therapy|therapies]] +/- [[topical]] [[therapy]] are recommended for [[Patient|patients]] who intolerant of [[topical]] [[treatments]]  
**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days  
**
**** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
**** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
*** 1.1.2 '''Pediatric'''
**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose) 
***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
****1.1.2.2 (Specific population e.g. '<nowiki/>'''''children < 8 years of age'''''')
***** Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose) 
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
** 1.2 '''Specific Organ system involved 2'''
*** 1.2.1 '''Adult'''
**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
*** 1.2.2  '''Pediatric'''
**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)


* 2 '''Stage 2 - Name of stage'''
* '''Stage IB-IIA'''
** 2.1 '''Specific Organ system involved 1 '''
** Generalized [[skin]] directed [[therapy]] with or without combination other [[skin]] direct [[therapy]]
**: '''Note (1):'''
***In majority of [[Patient|patients]] in this stages, [[skin]] directed [[therapy]] ([[topical]] [[corticosteroid]], HN2, [[ultraviolet]] B (UVB) [[therapy]]) recommended use more than [[systemic]] [[therapy]].
**: '''Note (2)''':
*The predominant [[therapy]] for [[cutaneous]] T cell lymphoma is [[PUVA therapy|PUVA]]. Adjunctive [[chemotherapy]], [[radiotherapy]], [[biological]] [[therapy]], [[retinoid]] [[therapy]], and photophoresis may be required.
**: '''Note (3):'''
* '''Stage IIB-IV'''
*** 2.1.1 '''Adult'''
**[[BV]] is used for the treatment of advanced stage
**** Parenteral regimen
**PCLBCL-LT
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
**The [[efficacy]] of [[BTK]] [[Inhibitor|inhibitors]]
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
 
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
{{Family tree/start}}
**** Oral regimen
{{familytree | | | | | | | | | | A01 | | | | | | | | | | | | | | | | | | | | | A01=Mycosis fungoides }}
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
{{familytree | | | | | | | | | | |!| | | | | | | | | | | | | | | }}
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
{{familytree | | |,|-|-|-|-|v|-|-|^|-|v|-|-|-|-|.| | | | | | | | | }}
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
{{familytree | | | B01 | | B02 | | | B03 | | | B04 | | | | |B01=Stage IA-IIA |B02=Stage IIA |B03=Stage III |B04= Stage IV | | |}}
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
{{familytree | | | |!| | | |!| | | | |!| | | | |!| | | | | }}
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
{{familytree | | | C01 | | C02 | | | C03 | | | C04 | | | | |C01=<br>• Expectane policy <br>• Topical steroides [IV-A] <br>• nb-UVB[III,A] <br>• PUVA [III-A] <br>• Topical mechlorethamine [II,B] <br>• Local RT [IV,A] |C02=<br>• Skin direct therapy(SDT) + local radiotherapy <br>• ST[III+A] <br>• (SDT+) retiods[III,B] <br>• (SDT+) IFN a {III,B] <br>• TSEBT [III,A] |C03=<br>• (SDT+) retinoides <br>• (SDT+) IFNa <br>• ECPI INFa +/- rtinoides <br>• Low dose MTX <br>• [IV-B] |C04=<br>• Gemcitabine <br>• Liposomal doxorubicin <br>• Brentuximab vedotin[II,B] }}
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
{{familytree | | | |!| | | |!| | | | |!| | | | |!| | | | | }}T
*** 2.1.2 '''Pediatric'''
{{familytree | | | D01 | | D02 | | | D03 | | | D04 | | |D01=<br>• (SDT+) retinoides [III,B] <br>• (SDT+) IFNa [III,B] <br>• Retinoides +IFN a [II,B] <br>• TSEBT [IV,A] |D02=<br>• Gemcitabin [IV,B] <br>• Liposomal doxorubicin [IV,B] <br>• Brentuximabvedotin [II,B] <br>• Combinatio Cht [Iv,B] <br>• AlloSCT[V,C] |D03=TSEBT[LV,B] |D04=<br>• Combination Cht [IV,B] <br>• AlloSCT [V,C] | | |}}
**** Parenteral regimen
{{Family tree/end}}
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
*Skin-directed therapies such as [[Steroid|steroids]], [[PUVA]], nb-[[UVB]], or [[mechlorethamine]] should be used as [[treatments]] for early-stage MF [[Patient|patients]] (stage IA-IIA)
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
*[[PUVA]] is the preferred method for [[Patient|patients]] with thicker [[Plaque|plaques]] [III, A], while [[Patient|patients]] with [[Patched|patches]] or very thin [[Plaque|plaques]] can be treated with nb-[[UVB]]
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '<nowiki/>'''''(Contraindications/specific instructions)''''''
*Adding low-[[dose]] [[local]] RT [III, A] is sufficient for [[Patient|patients]] with one or few infiltrated [[Tumor|tumors]] or [[Plaque|plaques]] (stage IIB).
**** Oral regimen
*In [[Patient|patients]] with unilesional MF and [[pagetoid]] reticulosis [IV, A], [[local]] RT eith dose 20–24 Gy [IV, A]  is recommended.
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
*In [[Patient|patients]] with more extensive infiltrated [[Plaque|plaques]] and [[Tumor|tumours]] or [[Patient|patients]] [[refractory]] to [[skin]]-directed therapies, a combination of [[PUVA]] and IFNα or [[PUVA]] and [[Retinoid|retinoids]] (including [[bexarotene]]), a combination of IFNα nd [[Retinoid|retinoids]] or TSEBT can be considered [III, B].
***** Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
*Even though total [[Dose|doses]] of TSEBT have been used in the range of 30 – 36 Gy, lower [[Dose|doses]] of 10 – 12 Gy are possible. This could benefit from shortertreatment periods, fewer side effects, and retreatment chances [III, A].
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
*[[Gemcitabine]] or [[Liposome|liposomal]] [[doxorubicin]] can be applied for [[Patient|patients]] with advanced and [[refractory]] [[disease]]. However, responses are short-lived in general [II, B].
***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
*Mycosis fungoides [[Patient|patients]] with distorted [[lymph]] nodes or [[Viscus|visceral]] involvement (stage IV), or [[Patient|patients]] with widespread tumor stage MF, show signs of multi-stage ChT. Controlling a multi-stage ChT with therapies that are [[skin]]-targeted and immunomodulating or single-agent ChT is not feasible.
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
*[[Local]] RT to [[Dose|doses]] 8 Gy [III, A] can be employed for local [[palliation]] of [[Skin|cutaneous]] and extracutaneous [[Lesion|lesions]].
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
*Young [[Patient|patients]] with [[refractory]] and progressive MF may be treated with alloSCT.  Though timing and optimal conditioning regimen are yet to be determined for an allogenic [[transplant]].
** 2.2  '<nowiki/>'''''Other Organ system involved 2''''''
*Mogamulizumab, a new [[drug]], is currently being examined in clinical trials.
**: '''Note (1):'''
**: '''Note (2)''':
**: '''Note (3):'''
*** 2.2.1 '''Adult'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
*** 2.2.2 '''Pediatric'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
**** Oral regimen
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per d


{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
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* May be given  
* May be given  
:* By itself  
:* By itself  
:* Or with topical chemotherapy
:* Or with [[topical]] [[chemotherapy]]
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* May be offered
* May be offered
Line 131: Line 81:
* May be given  
* May be given  
:* By itself  
:* By itself  
:* Or with interferon alfa
:* Or with [[Interferon-alpha|interferon alpha]]
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* May be offered
* May be offered
Line 137: Line 87:
* May be offered
* May be offered
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* To 1 or 2 skin lesions (local radiation therapy)  
* To 1 or 2 skin lesions (local [[radiation therapy]])  
* Total skin electron beam therapy
* Total [[skin]] [[electron]] beam [[therapy]]
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* May be given  
* May be given  
:* By itself  
:* By itself  
:* Or with topical chemotherapy
:* Or with [[topical]] [[chemotherapy]]
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* May be offered
* May be offered
Line 151: Line 101:
* May be given  
* May be given  
:* By itself  
:* By itself  
:* Or with interferon alfa
:* Or with [[interferon alpha]]
:* Or systemic chemotherapy
:* Or [[systemic]] [[chemotherapy]]
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* May be offered
* May be offered
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* May be combined with other skin-focussed therapies
* May be combined with other [[skin]]-focussed therapies
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* Total skin electron beam therapy
* Total [[skin]] [[electron]] beam [[therapy]]
* As palliative therapy to reduce the size of tumours or relieve symptoms
* As [[palliative]] therapy to reduce the size of [[Tumor|tumours]] or relieve [[Symptom|symptoms]]
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* May be given  
* May be given  
:* By itself
:* By itself
:* Or with topical chemotherapy
:* Or with [[topical]] [[chemotherapy]]
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* May be offered
* May be offered
Line 173: Line 123:
* May be given  
* May be given  
:* By itself  
:* By itself  
:* Or with interferon alfa
:* Or with [[interferon alpha]]
:* Or systemic chemotherapy
:* Or [[systemic]] [[chemotherapy]]
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* May be offered
* May be offered
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* May be offered
* May be offered
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* Total skin electron beam therapy (TSEB)
* Total [[skin]] [[electron]] beam [[therapy]] (TSEB)
* As palliative therapy to reduce the size of tumours or relieve symptoms
* As [[palliative]] [[therapy]] to reduce the size of [[Tumor|tumours]] or relieve [[Symptom|symptoms]]
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* May be given  
* May be given  
:* By itself  
:* By itself  
:* Or with topical chemotherapy
:* Or with [[topical]] [[chemotherapy]]
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* May be offered
* May be offered
Line 191: Line 141:
* May be given  
* May be given  
:*By itself  
:*By itself  
:* Or with total skin electron beam therapy
:* Or with total [[skin]] [[electron]] beam [[therapy]]
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | Recurrent cutaneous T cell lymphoma
| style="padding: 5px 5px; background: #F5F5F5;" | Recurrent cutaneous T cell lymphoma
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* Some authors have also documented liver toxicities associated with administration of retinodis, and liver function tests (LFTs) should also be monitored in these patients.
* Some authors have also documented liver toxicities associated with administration of retinodis, and liver function tests (LFTs) should also be monitored in these patients.


 
==References==
 
{{reflist|2}}
{{reflist|2}}


Latest revision as of 15:44, 12 February 2019

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

Overview

The predominant therapy for cutaneous T cell lymphoma is PUVA. Adjunctive chemotherapy, radiotherapy, biological therapy, retinoid therapy, and photophoresis may be required.

Medical Therapy

  • Patients with Mycosis fungoides are treated based on the stage and the previous treatment history.[1][2][3][4][5][6]

Mycosis fungoides (Early stages)

T
 
 
 
 
 
 
 
 
 
Mycosis fungoides
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Stage IA-IIA
 
Stage IIA
 
 
Stage III
 
 
Stage IV
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

• Expectane policy
• Topical steroides [IV-A]
• nb-UVB[III,A]
• PUVA [III-A]
• Topical mechlorethamine [II,B]
• Local RT [IV,A]
 

• Skin direct therapy(SDT) + local radiotherapy
• ST[III+A]
• (SDT+) retiods[III,B]
• (SDT+) IFN a {III,B]
• TSEBT [III,A]
 
 

• (SDT+) retinoides
• (SDT+) IFNa
• ECPI INFa +/- rtinoides
• Low dose MTX
• [IV-B]
 
 

• Gemcitabine
• Liposomal doxorubicin
• Brentuximab vedotin[II,B]
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

• (SDT+) retinoides [III,B]
• (SDT+) IFNa [III,B]
• Retinoides +IFN a [II,B]
• TSEBT [IV,A]
 

• Gemcitabin [IV,B]
• Liposomal doxorubicin [IV,B]
• Brentuximabvedotin [II,B]
• Combinatio Cht [Iv,B]
• AlloSCT[V,C]
 
 
TSEBT[LV,B]
 
 

• Combination Cht [IV,B]
• AlloSCT [V,C]
 
 
 
 
Medical therapy for cutaneous T cell lymphoma[1]
Stage PUVA Topical chemotherapy Systemic chemotherapy Radiotherapy Biological therapy Retinoid therapy Photopheresis
Stage I
  • May be given
  • By itself
  • Or with interferon alfa
  • May be offered
  • May be offered
  • To 1 or 2 skin lesions (local radiation therapy)
  • Total skin electron beam therapy (TSEB)
  • May be given
  • May be offered
 ---------
Stage II
  • May be given
  • May be offered
  • May be offered
  • May be given
  • May be offered
 ---------
Stage III
  • May be given
  • May be offered
  • May be combined with other skin-focussed therapies
  • May be given
  • May be offered
  • May be offered
Stage IV
  • May be given
  • May be offered
  • May be offered
  • May be given
  • May be offered
  • May be given
Recurrent cutaneous T cell lymphoma
  • May be offered
  • May be offered
  • May be offered
  • Total skin electron beam therapy
  • Radiation therapy to bulky tumours or lymph nodes
  • May be offered
 --------- ---------
Treatment for cutaneous T cell lymphoma[1]
Treatment Description
Phototherapy or Ultraviolet light therapy
PUVA (psoralen and ultraviolet A light therapy)
  • Treatment consists of giving a drug called psoralen and then a certain amount of ultraviolet A light is used on the skin
  • Psoralen makes the skin very sensitive to the effects of UVA light, which helps destroy the lymphoma cells
  • Psoralen is taken as a pill, usually about 2 hours before the skin is treated with the UVA light
  • PUVA is effective for treating thick patches and plaques
  • PUVA treatments are given much the same as a tanning session under a sunlamp
  • Treatments are given several times (often 3 times) a week at first
  • When the person responds, then the number of treatments is usually decreased
  • Treatments may need to be continued on a regular basis for several months (maintenance therapy)
  • PUVA treatment is sometimes called photochemotherapy
Ultraviolet B (UVB) light
  • UVB therapy is effective in treating skin patches or thin plaques
  • Psoralen is not used with UVB treatment
  • Treatment with UVB phototherapy may also be given several times a week
Chemotherapy
Topical chemotherapy
  • Is usually used to treat limited disease or early stage cutaneous T cell lymphoma because it is a local therapy
  • Mechlorethamine
  • Carmustine
Systemic chemotherapy
  • Is used to treat cutaneous T cell lymphoma that is more advanced, that has relapsed, or that no longer seems to be responding to other treatments
  • Most common chemotherapy pills
  • Intravenous chemotherapy drugs
Radiation therapy
Local external beam radiation therapy
  • May be used if only 1 or 2 small areas of skin are affected
  • It may also be used to treat patches that remain after PUVA treatment
Total skin electron beam (TSEB) therapy
  • May be used to treat larger areas of skin
  • Usually given only once to treat a person with cutaneous T cell lymphoma
  • But can sometimes be repeated using reduced doses if cutaneous T cell lymphoma recurs
  • Can cause a sunburn-like reaction and people may lose their finger nails, toe nails and hair
  • Requires special equipment and may not be available in all treatment centres
Biological therapy
Interferon alfa
  • Interferon alfa is injected under the skin into the fatty tissue (subcutaneously) to help boost the immune response
  • It may be used alone or in combination with other treatments, such as PUVA
Denileukin diftitox
  • Is a newer drug that is a combination of the biological therapy drug interleukin-2 and the diphtheria toxin
  • The interleukin finds the cutaneous T cell lymphoma cells and the diphtheria toxin kills the cells
Retinoid therapy
Retinoids
  • Retinoids are drugs that are similar to vitamin A and interfere with cell growth
  • Retinoids may be applied to the skin or may be taken by mouth (orally)
  • Bexarotene is one retinoid drug that may be used
  • Bexarotene comes in a gel form that can be put on the skin
  • It is used for early stage cutaneous T cell lymphoma with limited skin involvement
  • It can also be taken as a pill and is used for people with extensive skin involvement or who relapse
Photopheresis
Photopheresis
  • Involves running a person's blood from a vein in their arm through a machine that exposes it to ultraviolet A light
  • Similar to PUVA treatment, psoralen is used to make the cancerous white blood cells in the blood more sensitive to the effects of UVA light
  • The treated blood is then returned (reinfused) back into the body
  • This treatment is used for sezary syndrome or for progressing cutaneous T cell lymphoma
  • Often need to be repeated several times
  • May also be called extracorporeal photochemotherapy (ECP)
  • During treatment with systemic retinoids, lipid panel and thyroid function tests should be closely monitored
  • Gemfibrozil should be avoided because of the known side effects of the combined therapy; fish oil tablets can be used instead
  • Some authors have also documented liver toxicities associated with administration of retinodis, and liver function tests (LFTs) should also be monitored in these patients.

References

  1. ↑ 1.0 1.1 1.2 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
  2. ↑ Al Hothali GI (June 2013). "Review of the treatment of mycosis fungoides and Sézary syndrome: A stage-based approach". Int J Health Sci (Qassim). 7 (2): 220–39. PMC 3883611. PMID 24421750.
  3. ↑ Willemze, R; Hodak, E; Zinzani, P L; Specht, L; Ladetto, M (2018). "Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up†". Annals of Oncology. 29 (Supplement_4): iv30–iv40. doi:10.1093/annonc/mdy133. ISSN 0923-7534.
  4. ↑ Kim YH, Chow S, Varghese A, Hoppe RT (January 1999). "Clinical characteristics and long-term outcome of patients with generalized patch and/or plaque (T2) mycosis fungoides". Arch Dermatol. 135 (1): 26–32. PMID 9923777.
  5. ↑ Al Hothali GI (June 2013). "Review of the treatment of mycosis fungoides and Sézary syndrome: A stage-based approach". Int J Health Sci (Qassim). 7 (2): 220–39. PMC 3883611. PMID 24421750.
  6. ↑ Willemze, R; Hodak, E; Zinzani, P L; Specht, L; Ladetto, M (2018). "Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up†". Annals of Oncology. 29 (Supplement_4): iv30–iv40. doi:10.1093/annonc/mdy133. ISSN 0923-7534.


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