Cutaneous T-Cell Lymphoma Treatment & Management

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Mar 9, 2011
 

Medical Care

Treatment of Sézary syndrome (SS) should be predicated on disease burden and rapidity of progression.[57] One should attempt to preserve immune response, use immunomodulatory therapy before chemotherapy unless the disease burden or therapeutic failure requires otherwise, and consider combination therapy, particularly systemic immunomodulatory plus skin-directed treatments, as better options than monotherapy. Because Staphylococcus infection may be associated with a disease flare, systemic antibiotics should be administered when in doubt to prevent life-threatening sepsis. Pruritus treatment is an important quality of life consideration and should not be neglected.

When mycosis fungoides (MF) is confined to the skin (cutaneous T-cell lymphoma [CTCL]), skin-targeted therapies such as topical corticosteroids, heliotherapy, photochemotherapy (eg, psoralen plus UV-A [PUVA]), topical application of nitrogen mustard (mechlorethamine) or carmustine (BCNU), or radiotherapy, including total skin electron beam irradiation, are often effective a controlling manifestations.[58] Bexarotene is also sometimes used for disease confined to the skin. Psoriatic doses of methotrexate have been used, but some concern exists relating to large cell transformation occasionally observed in these patients.

Treatment of stages T1 and T2 MF with total skin electron beam therapy is highly effective in early-stage MF without adjuvant therapy. Management of relapses with local radiotherapy or a second round of total skin electron beam irradiation is feasible, timesaving, and cost-effective.[59] In patients with limited patch-stage MF, topical steroids or bexarotene gel can be used.

Azarotene, long-term and low-dose oral etoposide, and imiquimod may be of value in the treatment of early CTCL.[60, 61] One small pilot study showed tazarotene 0.1% gel to be a well-tolerated and effective adjuvant topical therapy for the treatment of refractory MF.[62]

Biological agents such as interferon alfa and other cytokines (eg, interleukin 2 [IL-2]), traditional and new retinoids such as bexarotene, and receptor-targeted cytotoxic fusion proteins (eg, DAB389I-2; denileukin diftitox) are increasingly being used. Combination therapy with bexarotene and PUVA should be considered for patients with treatment-resistant CTCL refractory to monotherapy.[63] However, the precise use of these new treatments, either as single-agent therapy or in combination with other therapies (eg, PUVA) in the treatment of MF, remains to be established.

Multiagent chemotherapy is used with unequivocal lymph node or systemic involvement or in cases with widespread tumor-stage MF that is refractory to skin-targeted therapies and is not early patch/plaque-stage disease.

Perifollicular localization of the dermal infiltrates in FMF is often less responsive to skin-targeted therapies (eg, PUVA, topical nitrogen mustard) than classic plaque-stage MF, and total skin electron beam irradiation is superior.[64] However, because sustained complete remissions are rarely achieved with electron beam therapy, PUVA combined with retinoids or interferon alfa should be considered, although persistent tumors can be effectively treated with local radiotherapy. Follicular CTCL may benefit from isotretinoin for persistent cysts and comedones.[65] Many benefit from psoralen plus ultraviolet A (PUVA) therapy, although they may not respond as well as those with classic MF.[22] Early syringotropic CTCL may be treated with local radiotherapy, with excellent local control.[66]

Extracorporeal photopheresis, either alone or in combination with other treatment modalities (eg, interferon alfa), is an effective treatment for Sézary syndrome (SS) and erythrodermic MF, with overall response rates of 30-80% and complete response rates of 14-25%.[67] The alleged superiority of extracorporeal photopheresis over the traditional low-dose chemotherapy regimens has not been proven. Beneficial results have also been described with interferon alfa, either alone or in combination with PUVA therapy, prolonged treatment with a combination of low-dose chlorambucil (2-4 mg/d) and prednisone (10-20 mg/d), or with methotrexate (MTX) (5-25 mg/wk), but complete responses are uncommon. Skin-directed therapies such as PUVA or potent topical steroids are good adjuvant therapies. Beneficial effects of bexarotene and alemtuzumab (anti-CD52) have been noted, but the long-term effects of these therapies remain to be established.[68]

Unrelated cord blood transplantation is a possible therapeutic option.[69] In one patient, it was given following myeloablative conditioning, which brought a complete remission. Although a relapse occurred 3 months later, discontinuation of immune suppressant therapy led to MF regression and a second complete remission that continued for more than 23 months. Reduced-intensity umbilical cord blood transplantation is another option for advanced MF.[70]

Also see the clinical guideline summary, Improving outcomes for people with skin tumours including melanoma.

A selection of active and recruiting clinical trials are as follows:

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Surgical Care

Localized mycosis fungoides (MF) may benefit from a number of therapeutic modalities, including radiotherapy, intralesional steroids, or surgical excision. One option is to excise a patch or plaque with a 0.5-cm margin and then control subsequent disease with either irradiation or photochemotherapy with PUVA, photodynamic therapy, or carbon dioxide laser surgery.[71] Localized radiation or a surgical approach to localized MF can be used, with the latter having the advantage that a full dose of radiation can be delivered in the same location at a later time, thus preserving options for future therapy.

Also see the clinical guideline summary from the British Association of Dermatologists, Guidelines for topical photodynamic therapy: update.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

W Clark Lambert, MD, PhD  Professor and Head, Dermatopathology, Departments of Pathology and Dermatology, UMDNJ-New Jersey Medical School

W Clark Lambert, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Dermatological Association, American Society of Dermatopathology, International Academy of Pathology, Medical Society of New Jersey, Sigma Xi, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Günter Burg, MD  Professor and Chairman Emeritus, Department of Dermatology, University of Zürich School of Medicine; Delegate of The Foundation for Modern Teaching and Learning in Medicine Faculty of Medicine, University of Zürich, Switzerland

Günter Burg, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, International Society for Dermatologic Surgery, North American Clinical Dermatologic Society, and Pacific Dermatologic Association

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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Tumor-stage cutaneous T-cell lymphoma.
Patch-stage cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD.
Plaque-stage parapsoriasis.
Patch-stage mycosis fungoides progressing to plaque stage with cutaneous cigarette-paper appearance evident.
Erythematous partially confluent plaques in advancing mycosis fungoides.
Close-up view of advancing plaque-stage mycosis fungoides with partially confluent erythematous plaques.
Early patch-stage cutaneous T-cell lymphoma.
Hypopigmented cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD.
Hypopigmented cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD.
Nodular mycosis fungoides.
Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.
Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.
Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.
Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.
Mycosis fungoides with large atypical T lymphocytes in both epidermis and dermis.
Mycosis fungoides with epidermal nest of large atypical T cells displaying bizarre, convoluted nuclei surrounded by a clear space (Pautrier microabscess).
Table 1. Staging of CTCL
StageSkin LesionsLymphadenopathyErythrodermaHistological Lymphoma
Patches



< 10%



Plaques



³10%



TumorsLymph NodesViscera
IA+------
IB+ or -+-----
IIA+ or -+ or -+ or -+---
IIB+ or -+ or -++ or ----
III+ or -+ or -+ or -+ or -+--
IVA+ or -+ or -+ or -+ or -+ or -+-
IVB+ or -+ or -+ or -+ or -+ or -+ or -+
Table 2. TNMB Staging of CTCL
Stage ClassStageDefinition
T



(Tumor)



T1Patches/plaques involving < 10% of body surface
T2Patches/plaques involving ³10% of body surface
T3Tumor(s) present on skin
T4Erythroderma
N



(Nodes)



N0No enlarged lymph node present
N1Enlarged lymph nodes, histologically uninvolved
N2No enlarged lymph node; one or more nodes histologically involved*
N3Enlarged lymph nodes, histologically involved
M



(Metastasis to viscera)



M0No visceral lesion present
M1Visceral involvement
B



(Blood involvement)



B0Circulating atypical lymphocytes (Sézary cells) £5% of lymphocytes
B1Circulating atypical lymphocytes ³5% of lymphocytes (Sézary syndrome)
*Uncommon finding, usually not considered/investigated.
Table 3. Comparison of Staging Systems for CTCL
Clinical StageTNM (B) Stage
IAT1 N0 M0
IIBT2 N0 M0
IIAT1 N1 M0T2 N1 M0
IIBT3 N0 M0T3 N1 M0
IIIT4 N0 M0T4 N1 M0
IVAT1 N2 M0T2 N2 M0T3 N2 M0T4 N2 M0
T1 N3 M0T2 N3 M0T3 N3 M0T4 N3 M0
IVBT1 N0 M1T2 N0 M1T3 N0 M1T4 N0 M1
T1 N1 M1T2 N1 M1T3 N1 M1T4 N1 M1
T1 N2 M1T2 N2 M1T3 N2 M1T4 N2 M1
T1 N3 M1T2 N3 M1T3 N3 M1T4 N3 M1
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