Durable Clinical, Cytogenetic, and Molecular Remissions After Allogeneic Hematopoietic Cell Transplantation for Refractory Sezary Syndrome and Mycosis Fungoides

2005 ◽  
Vol 23 (25) ◽  
pp. 6163-6171 ◽  
Author(s):  
Arturo Molina ◽  
Jasmine Zain ◽  
Daniel A. Arber ◽  
Maria Angelopolou ◽  
Margaret O’Donnell ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Transplantation ◽  
Mycosis Fungoides ◽  
Conditioning Regimen ◽  
Chain Gene ◽  
Sézary Syndrome ◽  
Sezary Syndrome ◽  
Hematopoietic Stem ◽  
Allogeneic Hsct ◽  
Unrelated Donors

Purpose Sezary syndrome (SS) and tumor-stage mycosis fungoides (MF) are generally incurable with currently available treatments. We conducted a retrospective study to evaluate the outcome of allogeneic hematopoietic stem-cell transplantation (HSCT) in this patient population. Patient and Methods From August 1996 through October 2002, eight patients with advanced MF/SS underwent allogeneic HSCT at our institution. All patients were heavily pretreated, having failed a median number of seven prior therapies (range, five to 12). Clonal T-cell populations in peripheral blood or bone marrow were detectable by polymerase chain reaction analyses of T-cell receptor γ-chain gene rearrangements in six patients and cytogenetics in three patients. The conditioning regimen included total-body irradiation and cyclophosphamide (n = 3), busulfan and cyclophosphamide (n = 1), and the reduced-intensity regimen of fludarabine and melphalan (n = 4). Allogeneic hematopoietic stem cells were obtained from HLA-matched siblings (n = 4) and unrelated donors (n = 4). Results All patients achieved complete clinical remission and resolution of molecular and cytogenetic markers of disease within 30 to 60 days after HSCT. Two patients died from transplantation-related complications; graft-versus-host disease (GVHD; n = 1) and respiratory syncytial virus pneumonia (n = 1). With a median follow-up of 56 months, six patients remain alive and without evidence of lymphoma. Conclusion Our results suggest that allogeneic HSCT from both HLA–matched sibling and unrelated donors can induce durable clinical, molecular, and cytogenetic remissions in patients with advanced cutaneous T-cell lymphoma that is refractory to standard therapies.

Safety and outcome of allogeneic stem cell transplantation in mycosis fungoides and Sezary syndrome: A seven-year tertiary center analysis.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e20038-e20038
Author(s):  
Selami Kocak Toprak ◽  
Guldane Cengiz Seval ◽  
Erden Atilla ◽  
Sinem Civriz Bozdag ◽  
Meltem Kurt Yuksel ◽  
...  
Keyword(s):  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Mycosis Fungoides ◽  
Sézary Syndrome ◽  
Sezary Syndrome ◽  
Advanced Stage ◽  
Hematopoietic Stem ◽  
Tertiary Center ◽  
Unrelated Donors

e20038 Background: Allogeneic hematopoietic stem cell transplantation (AHSCT) is a promising strategy for treatment of advanced-stage mycosis fungoides/Sezary syndrome (MF/SS). In this study, we retrospectively analyzed the outcomes of AHSCT for MF/SS and found that MF/SS appears to be susceptible to graft versus leukemia (GVL) effects. Methods: We studied outcomes in a consecutive series of 14 patients with MM/SS who underwent AHSCT between January 2012-November 2019 at our center. Treatment response was evaluated according to the Consensus WHO criteria for MF/SS. Results: The median age of patients 51.5 years (range: 28-68 years). Eight patients had MF and six patients had SS. Nine of those 14 patients had large cell transformation. All patients had advanced disease (stages IIB: n = 1, IIIA: n = 2, IIIB: n = 6, IVA1: n = 2 and IVB = 3). The median interval from disease onset to AHSCT was 3.7 years (range: 10.5 mos-11.3 years). All of the patients received at least three lines of treatments prior to AHSCT. Four patients achieved CR (3 are alive and still in CR) and all the other patients had progressive disease (PD) at the time of AHSCT. Graft source was peripheral blood stem cell in all patients. Six patients underwent transplantation from 9/10 HLA-matched unrelated donors and eight patients from HLA-identical sibling donors. Most of the patients (12/14) received RIC regimen (Flu-Cy-TBI) and ATG-Fresenius also added the protocol in AHSCT from unrelated donors. Five patients had stage 2-4 cutaneous acute GVHD and chronic GVHD developed in four patients (mild: n = 1, moderate: n = 2, severe: n = 1). Six patients who underwent transplantation at PD attained CR after AHSCT however relapse occurred in four patients at a median of four months (range: 3-10 months) after HSCT. At the time of data collection, nine patients have deceased; three were due to early TRM, three were underlying disease, two had aspergillus infection and one had nocardia infection. Two patients were alive with disease being treated by the Brentixumab vedotin, gemcitabine and DLI. With a median follow up period of 8.7 months after AHSCT, the estimated 1-year OS and PFS was 40.4%±5% and 35.4%±3.9%, respectively. Conclusions: AHSCT is an effective treatment option for advanced-stage MF/SS. Further studies are warranted to improve the outcome after AHSCT.

Allogeneic hematopoietic stem cell transplantation for refractory mycosis fungoides (MF) and Sezary syndrome (SS)

2017 ◽  
Vol 106 (3) ◽  
pp. 426-430 ◽  
Author(s):  
Erden Atilla ◽  
Pinar Ataca Atilla ◽  
Sinem Civriz Bozdag ◽  
Meltem Kurt Yuksel ◽  
Selami Kocak Toprak ◽  
...  
Keyword(s):  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Mycosis Fungoides ◽  
Sézary Syndrome ◽  
Sezary Syndrome ◽  
Hematopoietic Stem

scholarly journals Safety and Outcome of Allogeneic Hematopoietic Stem Cell Transplantation in Mycosis Fungoides and Sezary Syndrome: A 7-year Tertiary Center Analysis

LLM Dergi ◽  
2020 ◽  
Vol 4 (2) ◽  
pp. 23-27
Author(s):  
Güldane CENGİZ SEVAL ◽  
Erden ATİLLA ◽  
Pınar ATACA ATİLLA ◽  
Sinem CİVRİZ BOZDAĞ ◽  
Meltem KURT YÜKSEL ◽  
...  
Keyword(s):  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Mycosis Fungoides ◽  
Sézary Syndrome ◽  
Sezary Syndrome ◽  
Hematopoietic Stem ◽  
Tertiary Center

scholarly journals What factors guide treatment selection in mycosis fungoides and Sezary syndrome?

Hematology ◽  
2021 ◽  
Vol 2021 (1) ◽  
pp. 303-312
Author(s):  
Youn H. Kim
Keyword(s):  
T Cell ◽  
Mycosis Fungoides ◽  
Active Sites ◽  
Sézary Syndrome ◽  
Cell Transplant ◽  
Sezary Syndrome ◽  
Skin Involvement ◽  
Disease Heterogeneity ◽  
Early Stage Disease ◽  
Hematopoietic Stem

Abstract Cutaneous T-cell lymphoma (CTCL) comprises a spectrum of T-cell lymphomas with primary skin involvement. Mycosis fungoides (MF) and Sezary syndrome (SS) are the common subtypes of CTCL in which patients present with widely diverse profiles of skin involvement and varying extents of extracutaneous disease. Patients with early-stage disease have an excellent prognosis and are managed primarily with skin-directed therapies; however, those with advanced-stage MF or SS often require multiple lines and recurrent courses of systemic therapies. Many options are available when considering systemic agents, and it is often challenging to know how to prioritize therapies to address a patient's objective disease and quality of life issues. Appreciating the disease heterogeneity and understanding the patient's overall disease profile (eg, skin, lymph nodes, blood, large cell transformation) serve as a useful framework in aligning therapies that can optimally treat active sites of disease. Tissue or blood biomarkers can be integrated into our process of prioritizing therapies and personalizing management in MF or SS. Multidisciplinary management and optimizing supportive care are additional key elements for a favorable outcome. Appropriate patients with high-risk disease should be considered for allogeneic hematopoietic stem cell transplant.

Reduced-Intensity Conditioning Allogeneic Hematopoietic Stem Cell Transplantation Represents a Feasible and Effective Treatment Strategy for Advanced Mycosis Fungoides and Sezary Syndrome.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 2997-2997
Author(s):  
Davide Soligo ◽  
Francesco Onida ◽  
Giorgia Saporiti ◽  
Aldo Della Volpe ◽  
Cristian Marino ◽  
...  
Keyword(s):  
Overall Survival ◽  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Mycosis Fungoides ◽  
Sézary Syndrome ◽  
Sezary Syndrome ◽  
Hematopoietic Stem

Abstract Sezary syndrome (SS) and advanced tumor-stage mycosis fungoides (MF) have an aggressive clinical course, ultimately leading to a very short survival. Because patients are often elderly and show poor clinical conditions, conventional allogeneic hematopoietic stem cell transplantation is associated with a undesirable high TRM; therefore, allogeneic reduced-intensity conditioning (RIC) HSCT represent an attractive and worth-investigating strategy of cure in this disease setting. In our Institutions, from September 2000 to May 2006, fifteen patients with advanced mycosis fungoides or Sezary Sindrome underwent RIC allogeneic hematopoietic stem cell transplantation either from HLA-identical sibling, or from HLA-matched unrelated donor, or from unrelated cord blood. Conditioning regimens for patients with HLA-identical sibling included fludarabine/cyclophosphamide/TBI200 up to 2001, then pentostatin/TBI200. Patients who underwent unrelated transplants were treated with melphalan/CP1H/Fludarabine/TBI200 combination. All patients (10 males and 5 females) had a clinical diagnosis of MF (10 pts) or SS (5 pts) confirmed by histopathology, immunohistochemistry and molecular biology. Median age was 48 years (range 38–65). Median time from diagnosis to transplant was 48 months (range 13 to 252). Disease stage for all patients was from IIIB to IVB. GVHD prophylaxis included oral Cy-A from day 0 to day +100 and oral MMF from day 0 to day +27, then tapered within 2 weeks. The source of donor stem cells was peripheral blood and bone marrow for siblings and MUD, respectively. A complete clinical remission was achieved in 10 patients, whereas 2 patients obtained a VGPR. Five patients died from various complications (3 with progressive disease, 1 in CR e 1 in PR) within a period ranging from 33 days to 8 months after transplant. A full donor chimerism was achieved within 6 months in 8 out of 10 evaluable patients, whereas no engraftemnt was observed in 2 patients (1 MUD and 1 UCB). 5 patients experienced acute GVHD of grade I–II, while limited chronic GVHD occurred in 7 patients. With a median follow-up of 38 months (range 2–70), ten patients were alive with an estimated overall survival greater than 60% at five years (Figure 1), whereas 7 patients were alive and disease-free at the last follow-up. Based on these highly encouraging results, we conclude that RIC allogeneic HSCT represents a feasible and effective treatment for advanced mycosis fungoides and Sezary Syndrome. Further studies in larger patient populations are warranted to confirm the curative potential of this strategy. Overall Survival in MF/SS following RIC allo-HSCT Overall Survival in MF/SS following RIC allo-HSCT

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