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Steven M. Horwitz, Elise A. Olsen, Madeleine Duvic, Pierliugi Porcu and Youn H. Kim

. Willemze R Jaffe ES Burg G . WHO-EORTC classification for cutaneous lymphomas . Blood 2005 ; 105 : 3768 – 3785 . 3. Kim YH Liu HL Mraz-Gernhard S . Long-term outcome of 525 patients with mycosis fungoides and Sezary syndrome: clinical

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Matthew Ulrickson, Fred Okuku, Victoria Walusansa, Oliver Press, Sam Kalungi, David Wu, Fred Kambugu, Corey Casper and Jackson Orem

Cutaneous T-cell lymphomas (CTCL) constitute 2% of all non-Hodgkin’s lymphoma diagnoses in the United States. The most common subtypes are mycosis fungoides (MF) and its leukemic presentation, Sézary syndrome (SS). 1 CTCLs were noted to occur

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Neha Mehta-Shah, Steven M. Horwitz, Stephen Ansell, Weiyun Z. Ai, Jeffrey Barnes, Stefan K. Barta, Mark W. Clemens, Ahmet Dogan, Kristopher Fisher, Aaron M. Goodman, Gaurav Goyal, Joan Guitart, Ahmad Halwani, Bradley M. Haverkos, Richard T. Hoppe, Eric Jacobsen, Deepa Jagadeesh, Matthew A. Lunning, Amitkumar Mehta, Elise A. Olsen, Barbara Pro, Saurabh A. Rajguru, Satish Shanbhag, Aaron Shaver, Andrei Shustov, Lubomir Sokol, Pallawi Torka, Carlos Torres-Cabala, Ryan Wilcox, Basem M. William, Jasmine Zain, Mary A. Dwyer, Hema Sundar and Youn H. Kim

involvement (lymph nodes, blood, or less commonly other organs) or large cell transformation (LCT) may be seen in advanced-stage disease. Sézary syndrome (SS) is a rare leukemic subtype of CTCL characterized by significant blood involvement, erythroderma, and

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Andrew D. Zelenetz, Jeremy S. Abramson, Ranjana H. Advani, C. Babis Andreadis, John C. Byrd, Myron S. Czuczman, Luis Fayad, Andres Forero, Martha J. Glenn, Jon P. Gockerman, Leo I. Gordon, Nancy Lee Harris, Richard T. Hoppe, Steven M. Horwitz, Mark S. Kaminski, Youn H. Kim, Ann S. LaCasce, Tariq I. Mughal, Auyporn Nademanee, Pierluigi Porcu, Oliver Press, Leonard Prosnitz, Nashitha Reddy, Mitchell R. Smith, Lubomir Sokol, Lode Swinnen, Julie M. Vose, William G. Wierda, Joachim Yahalom and Furhan Yunus

expression of PAX5. CTCLs (Non-Anaplastic Morphology): Mycosis fungoides and Sézary syndrome are the most common types of CTCLs lacking anaplastic morphology. Primary CTCLs are very rare. In the WHO classification, 3 rare provisional entities are included

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Andrew D. Zelenetz, Jeremy S. Abramson, Ranjana H. Advani, C. Babis Andreadis, Nancy Bartlett, Naresh Bellam, John C. Byrd, Myron S. Czuczman, Luis E. Fayad, Martha J. Glenn, Jon P. Gockerman, Leo I. Gordon, Nancy Lee Harris, Richard T. Hoppe, Steven M. Horwitz, Christopher R. Kelsey, Youn H. Kim, Ann S. LaCasce, Auayporn Nademanee, Pierluigi Porcu, Oliver Press, Barbara Pro, Nashitha Reddy, Lubomir Sokol, Lode J. Swinnen, Christina Tsien, Julie M. Vose, William G. Wierda, Joachim Yahalom and Nadeem Zafar

. TNM classification system for primary cutaneous lymphomas other than 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

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Dana-Farber/Brigham and Women's Cancer Center | Massachusetts General Hospital Cancer Center

Non-Hodgkin's lymphomas (NHL) are a heterogeneous group of lymphoproliferative disorders originating in B-lymphocytes, T-lymphocytes, or natural killer (NK) lymphocytes. An estimated 63,190 new cases of NHL, including 34,200 in men and 28,990 in women, will be diagnosed in 2007, and 18,660 deaths will occur. NHL is the fifth leading type of new cancer diagnosed and the ninth leading cause of cancer deaths among men and the seventh among women. The median age of individuals with NHL has risen in the past 2 decades, resulting in a patient population that may also have significant comorbid conditions, complicating treatment options. Important updates to these guidelines for 2008 include expanded second-line therapy options for patients with diffuse large B-cell lymphoma and those with peripheral T-cell lymphomas. In addition, the mycosis fungoides/Sézary syndrome section now includes pages on updated staging and classification systems and suggested treatment regimens.

For the most recent version of the guidelines, please visit

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.0032 Review of the Treatment of Mycosis Fungoides and Sézary Syndrome: A Stage-Based Approach Horwitz Steven M. MD Olsen Elise A. MD Duvic Madeleine MD Porcu Pierliugi MD Kim Youn H. MD 04 2008 6 6 4 4 436 436 442 442

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Sophia Koo and Lindsey R. Baden

chemotherapy-refractory peripheral T-cell lymphomas . Blood 2004 ; 103 : 2920 – 2924 . 48. Lundin J Hagberg H Repp R . Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome

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Andy I. Chen and Ranjana H. Advani

Edited by Kerrin G. Robinson

fungoides/Sezary syndrome . Blood 2003 ; 101 : 4267 – 4272 . 20. Dearden CE Matutes E Cazin B . High remission rate in T-cell prolymphocytic leukemia with CAMPATH-1H . Blood 2001 ; 98 : 1721 – 1726 . 21. Enblad G Hagberg H

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Ali Bazarbachi, Hamdy A. Azim, Hussain Alizadeh, Mahmoud Aljurf, Ibrahim Barista, Naeem A. Chaudhri, Zahira Fahed, Omar A. Fahmy, Ardeshir Ghavamzadeh, Mohamed H. Khalaf, Sami Khatib, Aghiad Kutoubi, Semra Paydas, Hanadi Rafii Elayoubi, Ghazi Zaatari, Hamdy M. Zawam and Andrew D. Zelenetz

chemotherapy is recommended (methotrexate, cytarabine, and corticosteroids). Cutaneous T-Cell Lymphomas Background Many patients with smoldering ATL can be misdiagnosed with mycosis fungoides or Sézary syndrome if HTLV-I serology is not