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Pathologic review and appropriate clinical and staging evaluations are necessary to distinguish primary cutaneous and systemic lymphomas.<\/jats:p><\/jats:sec><jats:sec><jats:title>Risk\u2010Stratification<\/jats:title><jats:p>Disease histopathology remains the most important prognostic determinant in primary cutaneous B\u2010cell lymphomas, whereas staging remains an important prognostic determinant in cutaneous T\u2010cell lymphomas.<\/jats:p><\/jats:sec><jats:sec><jats:title>Risk\u2010Adapted Therapy<\/jats:title><jats:p>PCFCL and PCMZL patients with solitary or relatively few skin lesions may be effectively managed with local radiation therapy. While single\u2010agent rituximab may be employed for patients with more widespread skin involvement, multiagent chemotherapy is rarely appropriate. In contrast, the management of patients with PCDLBCL, LT is comparable to the management of patients with systemic DLBCL. Clinical staging forms the basis for a \u201crisk\u2010adapted,\u201d multi\u2010disciplinary approach to CTCL treatment. For patients with disease limited to the skin, disease\u2010specific and overall survival is measured in years, if not decades, and skin\u2010directed therapies are preferred. In contrast, patients with advanced\u2010stage disease, including cutaneous tumors or significant nodal, visceral, or blood involvement, are approached with systemic therapies in an escalating fashion. In selected patients, allogeneic stem\u2010cell transplantation may be considered, as this is curative in some patients.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajh.27735","type":"journal-article","created":{"date-parts":[[2025,6,11]],"date-time":"2025-06-11T01:59:20Z","timestamp":1749607160000},"page":"1603-1628","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Mycosis Fungoides, S\u00e9zary Syndrome, and Cutaneous B\u2010Cell Lymphomas: 2025 Update on Diagnosis, Risk\u2010Stratification, and Management"],"prefix":"10.1002","volume":"100","author":[{"given":"Alexandra C.","family":"Hristov","sequence":"first","affiliation":[{"name":"Department of Pathology University of Michigan  Ann Arbor Michigan USA"},{"name":"Department of Dermatology University of Michigan  Ann Arbor Michigan 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