Faculty Collaboration Database

Transformation of mycosis fungoides/Sezary syndrome: clinical characteristics and prognosis. Blood 1998 Aug 15;92(4):1150-9

Date

08/08/1998

Pubmed ID

9694702

DOI

10.1182/blood.v92.4.1150.416k04_1150_1159

Scopus ID

2-s2.0-0032529675 (requires institutional sign-in at Scopus site)   289 Citations

Abstract

The occurrence of large cell transformation has been well documented in a subgroup of patients with mycosis fungoides/Sezary syndrome (MF/SS). However, because of the rarity of MF/SS, little is known about the influence of clinicopathologic features in predicting large cell transformation and about outcome in the transformed cases. We evaluated all patients with MF/SS who were registered in our clinic during the study period and for whom pathologic slides for review were available or could be obtained. Disease was classified as transformed if biopsy showed large cells (>/=4 times the size of a small lymphocyte) in more than 25% of the infiltrate or if they formed microscopic nodules. Twenty-six patients with transformation were identified from a total of 115 evaluable cases with a diagnosis of MF/SS. The actuarial cumulative probability of transformation reached 39% in 12 years. The median time from diagnosis of MF/SS to transformation was 12 months (range, 0 to 128 months). Thirty-one percent of all patients with stage IIB-IV disease at presentation eventually transformed versus 14% of those with stage I-IIA (P = . 03), with transformation being especially common in patients with tumors (T3), 46% of whom transformed. Combining elevated beta2 microglobulin and lactic dehydrogenase (neither elevated v one or both elevated) was also predictive for transformation (P = .009). The median survival from initial diagnosis of MF/SS for the transformed patients was 37 months versus 163 months for the untransformed group (P = .0029). The median survival from transformation was 19.4 months (range, 2+ to 138 months). The following characteristics were associated with an inferior survival in transformed patients: (1) early transformation (<2 years from the diagnosis v >/=2 years; P = .011) and (2) advanced stage (IIB-IV v I-IIA; 2-year survival, 23% v 86%; P = .0035). We conclude that MF/SS patients with stages IIB-IV disease and, in particular, those with tumors have a high incidence of large-cell transformation. Patients with transformation have a relatively poor survival, especially if transformation occurs early (within 2 years) in the course of disease or if they are staged as IIB or higher.

Author List

Diamandidou E, Colome-Grimmer M, Fayad L, Duvic M, Kurzrock R

Author

Razelle Kurzrock MD Center Associate Director, Professor in the Medicine department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Actuarial Analysis
Adult
Aged
Aged, 80 and over
Biomarkers, Tumor
Cell Transformation, Neoplastic
Combined Modality Therapy
Disease Progression
Female
Humans
L-Lactate Dehydrogenase
Male
Middle Aged
Mycosis Fungoides
Neoplastic Stem Cells
Prognosis
Sezary Syndrome
Skin Neoplasms
Survival Analysis
Treatment Outcome
beta 2-Microglobulin