Extracutaneous transformation into a high-grade lymphoma: a potential pitfall in the management of patients with Sézary syndrome

International Journal of Dermatology
Sonja MichaelisW Kempf

Abstract

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. We identified four cases of transformation of Sézary syndrome (SS) into pleomorphic T-cell lymphoma. 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. Because of an apparently poorer prognosis, the early recognition of transformation, especially by lymph node biopsy, is important for adequate therapy.

References

Oct 28, 1998·Annals of Oncology : Official Journal of the European Society for Medical Oncology·M G BernengoA Appino
Jan 5, 2002·Journal of the American Academy of Dermatology·Eric C VonderheidUNKNOWN ISCL
Feb 5, 2005·Blood·Rein WillemzeChris J L M Meijer

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Citations

Dec 31, 2014·The American Journal of Dermatopathology·Mariela Judith NevetReuven Bergman

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