
Health Dictionary
Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Mycosis fungoides medical therapy
Introduction
Overview
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
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)
- Stage IA
- Patients with mycosis fungoides in stage IA ( patches, plaques, may be papules involve <10Â % of total skin surface) treat by skin directed therapy [topical corticosteroids, nitrogen mustard (meclorethamin, NH2)].
- Skin direct therapy is recommended more than systemic therapy (chemotherapy+ skin direct therapy) in this stage.
- Systemic therapies +/- topical therapy are recommended for patients who intolerant of topical treatments
- Stage IB-IIA
- The predominant therapy for cutaneous T cell lymphoma is PUVA. Adjunctive chemotherapy, radiotherapy, biological therapy, retinoid therapy, and photophoresis may be required.
- Stage IIB-IV
- BV is used for the treatment of advanced stage
- PCLBCL-LT
- The efficacy of BTK inhibitors
 |  |  |  |  |  |  |  |  | Mycosis fungoides |  |  |  |  |  |  |  |  |  |  |  |  |  |  |  |  |  |  |  |  | ||||||||||||||||||||||||||||||||||
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 |  | Stage IA-IIA |  | Stage IIA |  |  | Stage III |  |  | Stage IV |  |  |  |  |  |  | |||||||||||||||||||||||||||||||||||||||||||||||
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 |  | • 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] |  |  |  |  | |||||||||||||||||||||||||||||||||||||||||||||||||
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 |  | • (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] |  |  |  |  | |||||||||||||||||||||||||||||||||||||||||||||||||
- Skin-directed therapies such as steroids, PUVA, nb-UVB, or mechlorethamine should be used as treatments for early-stage MF patients (stage IA-IIA)
- PUVA is the preferred method for patients with thicker plaques [III, A], while patients with patches or very thin plaques can be treated with nb-UVB
- Adding low-dose local RT [III, A] is sufficient for patients with one or few infiltrated tumors or plaques (stage IIB).
- In patients with unilesional MF and pagetoid reticulosis [IV, A], local RT eith dose 20–24 Gy [IV, A] is recommended.
- In patients with more extensive infiltrated plaques and tumours or patients refractory to skin-directed therapies, a combination of PUVA and IFNα or PUVA and retinoids (including bexarotene), a combination of IFNα nd retinoids or TSEBT can be considered [III, B].
- Even though total doses of TSEBT have been used in the range of 30 – 36 Gy, lower doses of 10 – 12 Gy are possible. This could benefit from shortertreatment periods, fewer side effects, and retreatment chances [III, A].
- Gemcitabine or liposomal doxorubicin can be applied for patients with advanced and refractory disease. However, responses are short-lived in general [II, B].
- Mycosis fungoides patients with distorted lymph nodes or visceral involvement (stage IV), or 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.
- Local RT to doses 8 Gy [III, A] can be employed for local palliation of cutaneous and extracutaneous lesions.
- Young 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.
- Mogamulizumab, a new drug, is currently being examined in clinical trials.
| Stage | PUVA | Topical chemotherapy | Systemic chemotherapy | Radiotherapy | Biological therapy | Retinoid therapy | Photopheresis |
|---|---|---|---|---|---|---|---|
| Stage I |
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| Stage II |
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| Stage III |
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| Stage IV |
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| Recurrent cutaneous T cell lymphoma |
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| Treatment | Description | |
|---|---|---|
| Phototherapy or Ultraviolet light therapy | ||
| PUVA (psoralen and ultraviolet A light therapy) |
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| Ultraviolet B (UVB) light |
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| Chemotherapy | ||
| Topical chemotherapy |
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| Systemic chemotherapy |
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| Radiation therapy | ||
| Local external beam radiation therapy |
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| Total skin electron beam (TSEB) therapy |
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| Biological therapy | ||
| Interferon alfa |
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| Denileukin diftitox |
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| Retinoid therapy | ||
| Retinoids |
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| Photopheresis | ||
| Photopheresis |
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- 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
References
- ↑ 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
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
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]
