Hematopoietic stem-cell transplantation for advanced systemic mastocytosis.
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Ustun Celalettin, Reiter Andreas, Scott Bart L, Nakamura Ryotaro, Damaj Gandhi, Kreil Sebastian, Shanley Ryan, Hogan William J, Perales Miguel-Angel, Shore Tsiporah, Baurmann Herrad, Stuart Robert, Gruhn Bernd, Doubek Michael, Hsu Jack W, Tholouli Eleni, Gromke Tanja, Godley Lucy A, Pagano Livio, Gilman Andrew, Wagner Eva Maria, Shwayder Tor, Bornhäuser Martin, Papadopoulos Esperanza B, Böhm Alexandra, Vercellotti Gregory, Van Lint Maria Teresa, Schmid Christoph, Rabitsch Werner, Pullarkat Vinod, Legrand Faezeh, Yakoub-Agha Ibrahim, Saber Wael, Barrett John, Hermine Olivier, Hagglund Hans, Sperr Wolfgang R, Popat Uday, Alyea Edwin P, Devine Steven, Deeg H Joachim, Weisdorf Daniel, Akin Cem, Valent Peter
Аннотация
PATIENTS AND METHODS: In a global effort to define the value of HCT in SM, 57 patients with the following subtypes of SM were evaluated: SM associated with clonal hematologic non-mast cell disorders (SM-AHNMD; n = 38), mast cell leukemia (MCL; n = 12), and aggressive SM (ASM; n = 7). Median age of patients was 46 years (range, 11 to 67 years). Donors were HLA-identical (n = 34), unrelated (n = 17), umbilical cord blood (n = 2), HLA-haploidentical (n = 1), or unknown (n = 3). Thirty-six patients received myeloablative conditioning (MAC), and 21 patients received reduced-intensity conditioning (RIC).
RESULTS: Responses in SM were observed in 40 patients (70%), with complete remission in 16 patients (28%). Twelve patients (21%) had stable disease, and five patients (9%) had primary refractory disease. Overall survival (OS) at 3 years was 57% for all patients, 74% for patients with SM-AHNMD, 43% for those with ASM, and 17% for those with MCL. The strongest risk factor for poor OS was MCL. Survival was also lower in patients receiving RIC compared with MAC and in patients having progression compared with patients having stable disease or response.
CONCLUSION: AlloHCT was associated with long-term survival in patients with advanced SM. Although alloHCT may be considered as a viable and potentially curative therapeutic option for advanced SM in the meantime, given that this is a retrospective analysis with no control group, the definitive role of alloHCT will need to be determined by a prospective trial.