Introduction

Cutaneous T-cell lymphomas (CTCLs), such as Mycosis Fungoides (MF) and Sézary Syndrome (SS), are rare subtypes of non-Hodgkin lymphoma that significantly impact quality of life and have poor outcomes, particularly in advanced stages where survival rates can be less than 5 years. Diagnosing CTCL is challenging and requires a multidisciplinary approach involving dermatologists, pathologists, hematologists, and radiotherapists. The management of these conditions is further complicated by the limited availability of new drugs and the need to establish adequate therapeutic sequences. Risk factors for poorer outcomes include older age, advanced stage, and large cell transformation. Few treatments have been shown to improve progression-free survival (PFS) in advanced CTCL cases, and the role of allogeneic transplant (Allo) remains debated. The CUTALLO trial, the only prospective, randomized study in this field, demonstrated a benefit in PFS and quality of life but did not show a survival advantage. There is a notable lack of real-world data regarding the efficacy of allogeneic transplants in MF and SS.

Objective

The objective of the present study is to evaluate clinical-epidemiology characteristics and outcomes of patients with advanced stage MF/SS submitted to allogenic transplant in a single center in Brazil.

Methodology

We compiled data from patients aged ≥18 y/o diagnosed with MF/SS submitted to allogenic transplant from our institution. Clinical information included coded patient, gender, age, date of diagnostic, sites of disease, TNM stage, number of lines of therapy and its response. Allogenic transplant characteristics and outcomes were also described. Treatment outcome was determined by OS and PFS. REDcap Platform (by Vanderbilt) has been used to collect and store data and for descriptive analysis the IBM-SPSS version 24 was applied. Kaplan-Meier method estimated the OS, whereas Log-Rank tests to compare its curves.

Results

In this descriptive analysis, we examined five patients who underwent Allo transplant for advanced forms of MF or SS. The cohort consisted of three females and two males, with a median age of 49 years (ranging from 26 to 55 years). Most patients (4 out of 5) had received two or more lines of therapy prior to transplantation. Their treatments included low to intermediate intensity therapies such as interferon, methotrexate, brentuximab, or total skin therapy. Only two patients had been treated with polychemotherapy. Notably, these two patients were also the ones who did not receive care from a multidisciplinary group comprising dermatologists, hematologists, and radiotherapists.

Only 1 out of 5 patients is alive at the time of analysis, 4 years after the transplant, and this patient has no evidence of disease. Median overall survival in this cohort was 16 months (ranging from 1 month to 4 years).

Conclusion

Allogeneic transplant has always been part of treatment algorithms for MF/SS particularly for young patients with unfavorable prognostic factors and those who have clinically progressed within two years. Consequently, it continues to be offered worldwide. However, data and real-world experience show that, despite improvements in progression-free survival (PFS), most patients still do not achieve long remission or even cure. Subclassifying MF/SS based on molecular profiling may help in selecting better candidates and preform it earlier. There is still a lack of knowledge regarding this rare disease, which impacts the quality of life with permanent psicological damage to patients and their families.

Disclosures

No relevant conflicts of interest to declare.

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