Outcomes and prognostic factors in patients with systemic mastocytosis and an associated myeloid neoplasm: an international ECNM registry study.
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Hermine Olivier, Debeaupuis Orianne, Damaj Gandhi, Lübke Johannes, Schwaab Juliana, Heizmann Marc, Siebenhaar Frank, Pyatilova Polina, Nojarov Nicole, Niedoszytko Marek, Gorska Aleksandra, Mattsson Mattias, Shoumariyeh Khalid, de Bra Stephan, Wortmann Friederike, von Bubnoff Nikolas, Bumbea Horia, Soare Delia, Elena Chiara, Várkonyi Judit, Fortina Anna Belloni, Erdem Simge, Brockow Knut, Franz Tobias Michael, Panse Jens, Christen Deborah, Breynaert Christine, Yavuz Akif Selim, Malcovati Luca, Doubek Michael, Sabato Vito, Tanasi Ilaria, Bonifacio Massimiliano, Hartmann Karin, Rüfer Axel, Sciumè Mariarita, Bellani Valentina, Angelova-Fischer Irena, Stefan Alex, Triggiani Massimo, Parente Roberta, Klein Saskia K, Makris Michael, Papageorgiou Sotirios, van Daele Paul, Dybedal Ingunn, Baffoe Daniel, Gülen Theo, Jentzsch Madlen, Lortholary Olivier, Heiblig Mael, Livideanu Cristina Bulai, Barete Stéphane, Gourguechon Clément, Dimicoli-Salazar Sophie, Tournilhac Olivier, Frenzel Laurent, Arock Michel, Mital Andrzej, Woltsche Johannes N, Kluin-Nelemans Hanneke C, Elberink Joanna N G Oude, Gotlib Jason, Sperr Martina, Reiter Andreas, Valent Peter, Sperr Wolfgang R, Rossignol Julien
Аннотация
METHODS: We analyzed the 10th data wave of the European Competence Network on Mastocytosis registry (34 European centers and 1 US center). SM and AHN diagnoses followed the 2022 World Health Organization classification. Baseline characteristics and overall survival (OS) were compared between patients with myeloid SM-AHN and SM without AHN (SM-no-AHN). Within SM-AHN, outcomes were analyzed by SM component (advanced: aggressive SM [ASM] or MC leukemia [MCL] vs. non-advanced: bone marrow mastocytosis, indolent SM, or smoldering SM) and AHN subtype.
RESULTS: Among 3,925 patients with SM, 467 (11.9%) had myeloid SM-AHN. Myelodysplastic/myeloproliferative neoplasms (MDS/MPN) were the most frequent AHN category (41.1%), with chronic myelomonocytic leukemia as the most common subtype (29.6%). Compared with SM-no-AHN, SM-AHN patients were older, more often male, and less frequently had skin involvement. The SM component was advanced in 55.0% of SM-AHN versus 5.8% of SM-no-AHN (p < 0.001). OS was shorter in SM-AHN than SM-no-AHN (median 36.1 vs. 340.9 months; p < 0.001) and was reduced across SM subtypes, including ASM (31.0 vs. 81.1 months) and MCL (7.1 vs. 23.2 months). Within SM-AHN, advanced SM-AHN had shorter OS than non-advanced SM-AHN (28.3 vs. 70.9 months; p < 0.001). Median OS differed by AHN subtype (57.0 months in SM-MPN, 35.7 in SM-MDS, 34.2 in SM-MDS/MPN, and 14.7 in SM associated with acute myeloid leukemia; p < 0.001). In multivariable analysis, non-advanced SM (vs. advanced) remained independently associated with improved OS (hazard ratio = 0.44; p < 0.001).
CONCLUSIONS: SM-AHN is associated with reduced survival compared with SM without AHN across SM subtypes. Outcomes in SM-AHN are driven primarily by the aggressiveness of the mastocytosis component, supporting recognition and classification of SM in patients with concomitant myeloid neoplasms, given approved KIT-targeted tyrosine kinase inhibitors for advanced SM.