Mycosis fungoides medical therapy

Cutaneous T cell lymphoma Microchapters
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Patient Information
Overview
Classification Mycosis fungoides Sezary syndrome
Pathophysiology

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.

Mycosis fungoides (Early stages)

• Stage IA
  • 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 (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
  • Generalized skin directed therapy with or without combination other skin direct therapy
    • In majority of patients in this stages, skin directed therapy (topical corticosteroid, HN2, ultraviolet B (UVB) therapy) recommended use more than systemic therapy.
• 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

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]

• 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.
• For patients with more extensive infiltrated plaques and tumours or patients refractory to skin-directed therapies, a combination of PUVA and IFNa or PUVA and retinoids (including bexarotene), a combination of IFNa and retinoids or TSEBT can be considered [III, B]
• TSEBT has been given to total doses of 30–36 Gy, but recently lower doses (10–12 Gy) have been employed with the advantages of shorter duration of the treatment period, fewer side effects and opportunity for re-treatment [III, A]
• In patients with advanced and refractory disease, gemcitabine or liposomal doxorubicin may be considered, but responses are generally short-lived [II, B]
• Multi-agent ChT is only indicated in MF patients with effaced lymph nodes or visceral involvement (stage IV), or in patients with widespread tumour stage MF, which cannot be controlled with skin-targeted and immunomodulating therapies or who failed single-agent ChT
• Local palliation of cutaneous and as well as extracutaneous lesions may be achieved with local RT to doses 8 Gy [III, A]
• In relatively young patients with refractory, progressive MF alloSCT should be considered. The optimal conditioning regimen and timing for an allogeneic transplant are currently unknown [IV, C]

Medical therapy for cutaneous T cell lymphoma

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 By itself Or with topical chemotherapy	May be offered	---------
Stage II	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	May be given By itself Or with topical chemotherapy	May be offered	---------
Stage III	May be given By itself Or with interferon alfa Or systemic chemotherapy	May be offered	May be combined with other skin-focussed therapies	Total skin electron beam therapy As palliative therapy to reduce the size of tumours or relieve symptoms	May be given By itself Or with topical chemotherapy	May be offered	May be offered
Stage IV	May be given By itself Or with interferon alfa Or systemic chemotherapy	May be offered	May be offered	Total skin electron beam therapy (TSEB) As palliative therapy to reduce the size of tumours or relieve symptoms	May be given By itself Or with topical chemotherapy	May be offered	May be given By itself Or with total skin electron beam therapy
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

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 Methotrexate Chlorambucil Etoposide Intravenous chemotherapy drugs Fludarabine Cladribine Pentostatin Pegylated liposomal doxorubicin Gemcitabine
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.

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