Table 2

Summary of treatment options for MF/SS

TherapyMF
Sézary syndrome/erythrodermic MFComments
Early-stage diseaseAdvanced-stage disease
Topical corticosteroids ++++ ++ +++ Symptomatic control 
PUVA ++++ +++ Availability may be restricted in nonmetropolitan areas 
UVB +++ ++ More readily accessible than PUVA 
Topical chemotherapy   If limited number of lesions 
Imiquimod   If small lesions and limited number of lesions 
Photodynamic therapy   If limited number of lesions; limited availability 
Retinoids Usually second line; less used since bexarotene became available 
Bexarotene ++ +++ +++ Usually second line; can be used in combination with PUVA or IFN-α 
Interferon-α ++ +++ ++++ Second line 
HDACi +++ ++++ Beyond second line 
Oral MTX +++ ++ Low dose weekly 
Localized radiotherapy +++  If localized or large/plaques and tumor nodules 
TSEB ++ For widespread disease 
Systemic chemotherapy  ++ ++ Beyond second line 
ECP  ++++  If circulating clone detectable 
Autologous transplantation  Very selected cases 
Allogeneic transplantation  Very selected cases 
Denileukin diftitox  ++ ++ Beyond second line 
Alemtuzamab  Beyond second line; immunosuppressive 
Proteasome inhibitors   Under investigation 
Immunomodulatory agents (lenalidomide)   Under investigation 
TherapyMF
Sézary syndrome/erythrodermic MFComments
Early-stage diseaseAdvanced-stage disease
Topical corticosteroids ++++ ++ +++ Symptomatic control 
PUVA ++++ +++ Availability may be restricted in nonmetropolitan areas 
UVB +++ ++ More readily accessible than PUVA 
Topical chemotherapy   If limited number of lesions 
Imiquimod   If small lesions and limited number of lesions 
Photodynamic therapy   If limited number of lesions; limited availability 
Retinoids Usually second line; less used since bexarotene became available 
Bexarotene ++ +++ +++ Usually second line; can be used in combination with PUVA or IFN-α 
Interferon-α ++ +++ ++++ Second line 
HDACi +++ ++++ Beyond second line 
Oral MTX +++ ++ Low dose weekly 
Localized radiotherapy +++  If localized or large/plaques and tumor nodules 
TSEB ++ For widespread disease 
Systemic chemotherapy  ++ ++ Beyond second line 
ECP  ++++  If circulating clone detectable 
Autologous transplantation  Very selected cases 
Allogeneic transplantation  Very selected cases 
Denileukin diftitox  ++ ++ Beyond second line 
Alemtuzamab  Beyond second line; immunosuppressive 
Proteasome inhibitors   Under investigation 
Immunomodulatory agents (lenalidomide)   Under investigation 

MF indicates mycosis fungoides; SS, Sézary syndrome; PUVA, psoralan ultraviolet A; UVB, ultraviolet B; ECP, extracorporeal photopheresis; HDACi, histone deacetylase inhibitors; and TSEB, total skin electron beam.

Crosses indicate frequency of use: ++++, almost always; +++, very frequently; ++, moderately frequently; and +, occasionally.

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