|
|
 Previous Article | Table of Contents | Next Article 
Blood, Vol. 92 No. 4 (August 15), 1998:
pp. 1150-1159
Transformation of Mycosis Fungoides/Sezary Syndrome: Clinical
Characteristics and Prognosis
Eleni Diamandidou,
Maria Colome-Grimmer,
Luis Fayad,
Madeleine Duvic, and
Razelle Kurzrock
From the Department of Bioimmunotherapy, Pathology, and Medical
Specialties, University of Texas M.D. Anderson Cancer Center, Houston,
TX.
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  2
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.
© 1998 by The American Society of Hematology.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
H. M. Prince, S. Whittaker, and R. T. Hoppe
How I treat mycosis fungoides and Sezary syndrome
Blood,
November 12, 2009;
114(20):
4337 - 4353.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. H. Weenig, N. I. Comfere, L. E. Gibson, J. Alonso-Llamazares, M. D. P. Davis, M. R. Pittelkow, and R. A. el-Azhary
Fatal Cytotoxic Cutaneous Lymphoma Presenting as Ulcerative Psoriasis
Arch Dermatol,
July 1, 2009;
145(7):
801 - 808.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. Chandra, J. A. Plaza, Z. Zuo, A. H. Diwan, H. Koeppen, M. Duvic, L. J. Medeiros, and V. G. Prieto
Clusterin Expression Correlates With Stage and Presence of Large Cells in Mycosis Fungoides
Am J Clin Pathol,
April 1, 2009;
131(4):
511 - 515.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. O. Arulogun, H. M. Prince, J. Ng, S. Lade, G. F. Ryan, O. Blewitt, and C. McCormack
Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation
Blood,
October 15, 2008;
112(8):
3082 - 3087.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. Quereux, S. Marques, J.-M. Nguyen, C. Bedane, M. D'incan, O. Dereure, E. Puzenat, A. Claudy, L. Martin, P. Joly, et al.
Prospective Multicenter Study of Pegylated Liposomal Doxorubicin Treatment in Patients With Advanced or Refractory Mycosis Fungoides or Sezary Syndrome
Arch Dermatol,
June 1, 2008;
144(6):
727 - 733.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Wabulya, J. Imitola, S. Santagata, and S. Kesari
Mycosis Fungoides With Leptomeningeal Involvement
J. Clin. Oncol.,
December 10, 2007;
25(35):
5658 - 5661.
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. Shin, S. Monti, D. J. Aires, M. Duvic, T. Golub, D. A. Jones, and T. S. Kupper
Lesional gene expression profiling in cutaneous T-cell lymphoma reveals natural clusters associated with disease outcome
Blood,
October 15, 2007;
110(8):
3015 - 3027.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. Olsen, E. Vonderheid, N. Pimpinelli, R. Willemze, Y. Kim, R. Knobler, H. Zackheim, M. Duvic, T. Estrach, S. Lamberg, et al.
Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)
Blood,
September 15, 2007;
110(6):
1713 - 1722.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. Callet-Bauchu, G. Salles, S. Gazzo, S. Dalle, F. Berger, and S. Hayette
Identification of a novel e8/a4 BCR/ABL fusion transcript in a case of a transformed Sezary syndrome
Haematologica,
September 1, 2007;
92(9):
1277 - 1278.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. Natsuga, T. Shimizu, R. Abe, K. Kodama, and H. Shimizu
Mycosis fungoides bullosa.
Arch Dermatol,
June 1, 2006;
142(6):
793 - 795.
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. Willemze, E. S. Jaffe, G. Burg, L. Cerroni, E. Berti, S. H. Swerdlow, E. Ralfkiaer, S. Chimenti, J. L. Diaz-Perez, L. M. Duncan, et al.
WHO-EORTC classification for cutaneous lymphomas
Blood,
May 15, 2005;
105(10):
3768 - 3785.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
N. Franck, A. Carlotti, I. Gorin, M. Buffet, C. Mateus, N. Dupin, and for the French Study Group on Cutaneous Lymphomas
Mycosis Fungoides-Type Cutaneous T-Cell Lymphoma and Neutrophilic Dermatosis
Arch Dermatol,
March 1, 2005;
141(3):
353 - 356.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C Assaf, M Hummel, M Zemlin, M Steinhoff, C C Geilen, H Stein, and C E Orfanos
Transition of Sezary syndrome into mycosis fungoides after complete clinical and molecular remission under extracorporeal photophoresis
J. Clin. Pathol.,
December 1, 2004;
57(12):
1325 - 1328.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. Jones, S. Ibrahim, K. Patel, R. Luthra, M. Duvic, and L. J. Medeiros
Degree of CD25 Expression in T-Cell Lymphoma Is Dependent on Tissue Site: Implications for Targeted Therapy
Clin. Cancer Res.,
August 15, 2004;
10(16):
5587 - 5594.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Girardi, P. W. Heald, and L. D. Wilson
The Pathogenesis of Mycosis Fungoides
N. Engl. J. Med.,
May 6, 2004;
350(19):
1978 - 1988.
[Full Text]
[PDF]
|
|
|
|
|
|
|
Y. H. Kim, H. L. Liu, S. Mraz-Gernhard, A. Varghese, and R. T. Hoppe
Long-term Outcome of 525 Patients With Mycosis Fungoides and Sezary Syndrome: Clinical Prognostic Factors and Risk for Disease Progression
Arch Dermatol,
July 1, 2003;
139(7):
857 - 866.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. Lundin, H. Hagberg, R. Repp, E. Cavallin-Stahl, S. Freden, G. Juliusson, E. Rosenblad, G. Tjonnfjord, T. Wiklund, and A. Osterborg
Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome
Blood,
June 1, 2003;
101(11):
4267 - 4272.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. H. Sarris, A. Phan, M. Duvic, J. Romaguera, P. McLaughlin, O. Mesina, K. King, L. J. Medeiros, G. Z. Rassidakis, B. Samuels, et al.
Trimetrexate in Relapsed T-Cell Lymphoma With Skin Involvement
J. Clin. Oncol.,
June 15, 2002;
20(12):
2876 - 2880.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Urosevic, J. Willers, B. Mueller, W. Kempf, G. Burg, and R. Dummer
HLA-G protein up-regulation in primary cutaneous lymphomas is associated with interleukin-10 expression in large cell T-cell lymphomas and indolent B-cell lymphomas
Blood,
January 15, 2002;
99(2):
609 - 617.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. M. Connors, E. D. Hsi, and F. M. Foss
Lymphoma of the Skin
Hematology,
January 1, 2002;
2002(1):
263 - 282.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
M. Duvic, K. Hymes, P. Heald, D. Breneman, A. G. Martin, P. Myskowski, C. Crowley, and R. C. Yocum
Bexarotene Is Effective and Safe for Treatment of Refractory Advanced-Stage Cutaneous T-Cell Lymphoma: Multinational Phase II-III Trial Results
J. Clin. Oncol.,
May 1, 2001;
19(9):
2456 - 2471.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Duvic, A. G. Martin, Y. Kim, E. Olsen, G. S. Wood, C. A. Crowley, R. C. Yocum, and for the Worldwide Bexarotene Study Group
Phase 2 and 3 Clinical Trial of Oral Bexarotene (Targretin Capsules) for the Treatment of Refractory or Persistent Early-Stage Cutaneous T-Cell Lymphoma
Arch Dermatol,
May 1, 2001;
137(5):
581 - 593.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Li, D. T. Ross, M. E. Kadin, P. O. Brown, and M. A. Wasik
Comparative Genome-Scale Analysis of Gene Expression Profiles in T Cell Lymphoma Cells during Malignant Progression Using a Complementary DNA Microarray
Am. J. Pathol.,
April 1, 2001;
158(4):
1231 - 1237.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. S. Siegel, T. Pandolfino, J. Guitart, S. Rosen, and T. M. Kuzel
Primary Cutaneous T-Cell Lymphoma: Review and Current Concepts
J. Clin. Oncol.,
August 15, 2000;
18(15):
2908 - 2925.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. Vergier, A. de Muret, M. Beylot-Barry, L. Vaillant, D. Ekouevi, G. Chene, A. Carlotti, N. Franck, P. Dechelotte, P. Souteyrand, et al.
Transformation of mycosis fungoides: clinicopathological and prognostic features of 45 cases
Blood,
April 1, 2000;
95(7):
2212 - 2218.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. M. Muche, A. Lukowsky, J. Heim, M. Friedrich, H. Audring, and W. Sterry
Demonstration of Frequent Occurrence of Clonal T Cells in the Peripheral Blood But Not in the Skin of Patients With Small Plaque Parapsoriasis
Blood,
August 15, 1999;
94(4):
1409 - 1417.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
