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


CONCLUSIONS
Because of an apparently poorer prognosis, the early recognition of transformation, especially by lymph node biopsy, is important for adequate therapy.
Department of Dermatology, University Hospital, Zurich, Switzerland.
Sonja
S
Michaelis
Dmitry V
DV
Kazakov
Günter
G
Burg
Reinhard
R
Dummer
Werner
W
Kempf
D000328:Adult; D002471:Cell Transformation, Neoplastic / Q000473:pathology*; D005260:Female; D006801:Humans; D017730:Ki-1 Antigen; D016399:Lymphoma, T-Cell / Q000276:immunology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D012751:Sezary Syndrome / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D012878:Skin Neoplasms / Q000276:immunology / Q000503:physiopathology*
D017730:Ki-1 Antigen