ISSN: 1300-7777 E-ISSN: 1308-5263
Transformation of Mycosis fungoides/Sezary syndrome: clinical characteristics and prognosis [Turk J Hematol]
Turk J Hematol. Ahead of Print: TJH-15679 | DOI: 10.4274/tjh.2016.0502  

Transformation of Mycosis fungoides/Sezary syndrome: clinical characteristics and prognosis

Seçil Vural1, Bengü Nisa Akay1, Ayşenur Botsali1, Erden Atilla2, Nehir Parlak3, Aylin Okcu Heper4, Hatice Şanlı1
1Ankara University, Faculty of Medicine, Department of Dermatology and Venereology
2Ankara University, Faculty of Medicine, Department of Dermatology and Heamatology
3Ankara University, Faculty of Medicine, Department of Dermatology and Venereology, Etimesgut State Hospital, Department of Dermatology and Venereology
4Ankara University, Faculty of Medicine, Department of Dermatology and Pathology

Introduction: Transformed mycosis fungoides (T-MF) is a rare variant of mycosis fungoides (MF) with an aggressive course.
Objectives: In this study we aimed to describe characteristics MF/Sezary syndrome (SS) cases with transformation.
Materials and Methods: Patients diagnosed with transformed MF (T-MF) among MF/SS patients between 2000 and 2014 in a single center are evaluated retrospectively.Demographic, clinical, laboratory, immunophenotype features, response to treatment, survival and histopathologic features were analyzed.
Results: Among 254 MF patients, 25 patients with T-MF are identified (10.2%) and included in the study. Male to female ratio was 2.6/1. The median time between MF diagnosisand transformation was 32 months (range 0-192). Nine (36%) patients were diagnosed initially with T-MF. Advanced disease stage and high serum lactate dehydrogenase levels were indicators of poor prognosis and treatment response. Five of the 18 patients with progressive disease had undergone allogeneic hematopoietic stem cell transplantation (Allo-HSCT). Allo-HSCT resulted in complete remission in three (60%) patients. Ten (40%) patients died as a result of disease progression. Mean survival time was 25,2±14,9 (2-56) months after transformation.
Conclusion: Advanced stage, high serum lactate dehydrogenase levels and loss of CD26 and CD7 expression in peripheral blood are bad prognostic factors in T-MF. Treatment resistant tumors and nodules should be cautionary for T-MF. Patients with T-MF have a shortened survival. Some patients may respond to first line treatments. However, majority of patients do not respond to second or third line therapies. Allo-HSCT may be an alternative option in patients with T-MF.

Keywords: Anaplastic transformation, mycosis fungoides, transformed, allogeneic hematopoietic stem cell transplantation, sezary syndrome




Corresponding Author: Seçil Vural, Türkiye


TOOLS
Uncorrected Full Text PDF
Print
Download citation
RIS
EndNote
BibTex
Medlars
Procite
Reference Manager
Share with email
Share
Send email to author

Similar articles
PubMed
Google Scholar


 



Impact Factor (2016) = 0.686