The clinical practice guideline for primary cutaneous T/NK-cell lymphomas other than mycosis fungoides and Sezary syndrome

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In this report, the clinical practice guideline for primary cutaneous T/NK-cell lymphomas other than mycosis fungoides and Sezary syndrome is described. These lymphomas can be classified into two clinical subgroups based on clinical features and prognosis. The indolent group consists of cutaneous CD30+ lymphoproliferative disorders, subcutaneous panniculitis-like T-cell lymphoma and cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma. Because these three categories show unique clinical features and slightly different prognosis, clinical questions for these entities were set up separately. The aggressive group consists of cutaneous aggressive epidermotropic CD8+ T-cell lymphoma, cutaneous gamma/delta T-cell lymphoma and cutaneous peripheral T-cell lymphoma, unspecified. Since these lymphomas show similar cutaneous manifestations and very poor prognosis, we made clinical questions for these lymphomas in one group. In the algorithm described in this guideline, we adopted the recently proposed TNM classification system because it is necessary to standardize the clinical descriptions of patients with cutaneous lymphomas for the revision of this guideline in the near future. [Skin Cancer (Japan) 2008; 23: 294-301]
日本皮膚悪性腫瘍学会の論文