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  • Title: Extracutaneous transformation into a high-grade lymphoma: a potential pitfall in the management of patients with Sézary syndrome.
    Author: Michaelis S, Kazakov DV, Burg G, Dummer R, Kempf W.
    Journal: Int J Dermatol; 2006 Mar; 45(3):277-9. PubMed ID: 16533228.
    Abstract:
    BACKGROUND: Transformation into a high-grade lymphoma in cutaneous T-cell lymphoma (CTCL) occurs in approximately 25% of cases and is associated with an aggressive clinical course. METHODS: We identified four cases of transformation of Sézary syndrome (SS) into pleomorphic T-cell lymphoma. RESULTS: In all patients, transformation occurred first in the lymph nodes, an average of 43 months after the diagnosis of SS. These high-grade lymphomas were composed of CD30-positive (two patients) and CD30-negative (two patients) pleomorphic large cells. All patients died of lymphoma an average of 29 months after nodal transformation. CONCLUSION: Because of an apparently poorer prognosis, the early recognition of transformation, especially by lymph node biopsy, is important for adequate therapy.
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