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1684 Part XI: Malignant Lymphoid Diseases Chapter 103: Cutaneous T-Cell Lymphoma (Mycosis Fungoides and Sézary Syndrome) 1685

Therapies for Cutaneous T-Cell Lymphomas Figure 103–6. Cutaneous T-cell lym-
phoma treatment algorithm. NBUVB, narrow-
band ultraviolet B; PUVA, psoralen ultraviolet
la Ib lla llb III IVa IVb A; TSEB, total-skin electron beam; UVB, ultra-
violet B.
Skin-directed therapies
Topical corticosteroids
Topical chemotherapy
Topical retinoids
Phototherapy
PUVA, UVB, NBUVB

Radiation therapy
Localized and TSEB
Systemic treatments
Bexarotene
Interferon
Denileukin diftitox
Vorinostat
Alemtuzumab
Chemotherapy single agent

Photopheresis

Multiagent chemotherapy

Experimental

on induction of long-term remissions with the least toxic agents and in patients with early stages of the disease (stages IA and IB), but can be
regimens. A number of highly effective monotherapies were recently used as a part of combination therapy with systemic agents in advanced
shown to be safe and effective in patients with MF and SS in prospec- stages. Prior to FDA-approval of mechlorethamine hydrochloride 0.02
tive clinical trials, leading to their approval by the FDA in the United percent gel in 2013, it was only available through certified compound-
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States and abroad. Several novel agents are in clinical trials to test for ing pharmacies in forms of solution, cream or ointment of various con-
their efficacy as monotherapy for MF. In addition, there are number of centrations (0.01 to 0.04 percent). The major advantage of this topical
promising combination regimens being tested in clinical trials examin- therapy is its relatively low toxicity. Disadvantages include the incon-
ing systemic multiagent therapies in advanced MF or SS to guide ther- venience of daily application to large areas of skin, allergic reactions in
apeutic decisions. up to half of cases, the potential for development of skin cancer, and
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the inability to cure the disease. Therapy is usually continued for up to
12 months in responders. Frequency then is reduced to every other day
SKIN-DIRECTED THERAPIES for an additional 1 to 2 years. Therapy is discontinued after 3 years or
Topical Glucocorticoids when cutaneous lesions disappear completely. Mechlorethamine should
These agents are effective during early stages of MF. They are limited to not be used together with ultraviolet A (UVA) or ultraviolet B (UVB)
temporary short-term use because of suppression of collagen synthe- therapy because of cumulative carcinogenic effects on the skin and sig-
sis (skin atrophy), striae formation, skin fragility, and secondary infec- nificant increase in risk for skin cancer and melanoma.
tions. The class of topical preparation depends on the area and the site Topical Carmustine (Bis-Chloroethylnitrosourea [BCNU]) BCNU
of involvement. Ultrapotent topical glucocorticoids should not be used is not currently widely used for treatment of MF because of its severe
on the face, neck, and intertriginous areas. Topical steroids are rarely irritant reactions and its absorption from the skin that results in sys-
used as monotherapy, but may be effective as an additional modality for temic toxicity. The preparation ranges from 20 to 40 mg/dL in petro-
symptomatic relieve of pruritus. latum ointment. It is applied at night and washed off in the morning.
Topical Tacrolimus (Protopic) Topical tacrolimus has been Monitoring includes biweekly complete blood counts to identify mar-
approved for use in atopic dermatitis. It is as effective as mid- to row suppression. Carmustine causes irreversible skin thinning, telang-
low-potency glucocorticoids for use on facial skin and intertriginous iectasias, and hyperpigmentation. 87
areas in patients with MF. A major advantage of tacrolimus compared Topical Retinoids Bexarotene (Targretin) 1 percent gel, is a topi-
with steroids is that it does not suppress collagen synthesis and therefore cal retinoid (rexinoid) FDA-approved for treatment of MF patients who
does not cause skin atrophy. However, because the use of calcineurin are refractory to at least one other topical therapy. It is a small lipophilic
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inhibitors in CTCL is controversial, tacrolimus use should be limited molecule related to vitamin A. It readily crosses the cytoplasmic mem-
83
to short-term use on small areas. brane and binds to nuclear receptors (retinoid X receptors [RXRs]),
Topical Nitrogen Mustard (Mechlorethamine Hydrochloride) resulting in changes in gene expression mediated through specific intra-
Topical nitrogen mustard is a topical alkylating agent used predominantly cellular receptors, leading to maturating and apoptosis of malignant

Kaushansky_chapter 103_p1679-1692.indd 1685 9/21/15 12:51 PM