Mycosis fungoides medical therapy

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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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