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Cutaneous T-cell lymphomas (CTCL) are a heterogenous group of non-Hodgkin lymphomas arising in the skin. Mycosis fungoides (MF), the most common variant, is characterised by clonal proliferation of skin residing malignant T-cells. Initially appearing with erythematous patches and plaques it follows a chronic course with progression to cutaneous tumours and extracutaneous involvement in some patients. Phototherapy with ultraviolet A radiation combined with 8-methoxypsoralen (PUVA) and with narrow-band ultraviolet B radiation (NB-UVB) are among the first line options for the treatment of MF and can induce remission in most patients. Sézary syndrome (SS) is a rare and more aggressive CTCL variant with generalized skin involvement. Patients with SS and with erythroderma from MF can benefit from treatment with extracorporeal photochemotherapy (ECP) where peripheral blood is exposed to PUVA. Phototherapy can be safely combined with systemic agents, most notably interferon-alpha and retinoids. Another photoresponsive CTCL variant is lymphomatoid papulosis (LP), a CD30+ lymphoproliferative disease characterised by chronically recurring papules. The disease responds favourably to PUVA but low dose methotrexate might be preferred for long term disease control. Recently updated treatment guidelines have been published to provide evidence-based algorithms for the stage-oriented treatment of MF, SS and LP. Areas of uncertainty are treatment schedules that are currently not optimised for CTCL, the use of phototherapy for maintenance, and the value of ultraviolet A1 radiation, excimer lasers, and photodynamic therapy.
Authors : Trautinger Franz ,
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WP1011: T Cell Receptor Signaling Pathway
WP1025: B Cell Receptor Signaling Pathway
WP1043: Calcium Regulation in the Cardiac Cell
WP1069: Integrin-mediated cell adhesion
WP1078: G1 to S cell cycle control
WP1083: Cell cycle
WP1130: T Cell Receptor Signaling Pathway
WP1144: B Cell Receptor Signaling Pathway
WP1159: Calcium Regulation in the Cardiac Cell
WP1185: Integrin-mediated cell adhesion
WP1195: G1 to S cell cycle control
WP1200: Cell cycle
WP1345: T Cell Receptor Signaling Pathway
WP1354: B Cell Receptor Signaling Pathway
WP1365: Calcium Regulation in the Cardiac Cell
WP1386: Integrin-mediated cell adhesion
WP1393: Cell cycle
WP1411: Cell Division: First embryonic mitosis
WP1775: Cell Cycle Checkpoints
WP1782: APC/C-mediated degradation of cell cycle proteins
WP179: Cell cycle
WP1792: Cell Cycle, Mitotic
WP1793: Cell junction organization
WP1794: Cell surface interactions at the vascular wall
WP1829: Immunoregulatory interactions between a Lymphoid and a non-Lymphoid cell
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 From Early Immunomodulatory Triggers to Immunosuppressive Outcome: Therapeutic Implications of the Complex Interplay Between the Wavebands of Sunlight and the Skin.
 A Perspective on the Use of NB-UVB Phototherapy vs. PUVA Photochemotherapy.
 Folliculotropic mycosis fungoides in a pediatric patient with response to psoralen-ultraviolet A phototherapy.
 Perplexing Rash: Challenges to Diagnosis and Management of Mycosis Fungoides.
 Poikilodermatous Mycosis Fungoides - Rare Entity, Different Treatment Modalities.
 The First Year of the AEVD Primary Cutaneous Lymphoma Registry.
 The efficacy and safety of phototherapy in geriatric patients: a retrospective study.
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