TY - JOUR
T1 - Granularity in disease classification impacts survival prediction in advanced systemic mastocytosis
T2 - A single institution study of 329 informative cases
AU - Tefferi, Ayalew
AU - Abdelmagid, Maymona
AU - Al-Kali, Aref
AU - Patnaik, Mrinal
AU - Hogan, William J.
AU - Begna, Kebede
AU - Gangat, Naseema
AU - Orazi, Attilio
AU - Chen, Dong
AU - Reichard, Kaaren K.
AU - Pardanani, Animesh
N1 - Publisher Copyright:
© 2023 Wiley Periodicals LLC.
PY - 2024/1
Y1 - 2024/1
N2 - The World Health Organization (WHO) classification system categorizes advanced systemic mastocytosis (SM-Adv) into aggressive SM (ASM), mast cell leukemia (MCL), and SM with associated hematological neoplasm (SM-AHN). By contrast, the International Consensus Classification (ICC) requires “immature” MC cytomorphology for the diagnosis of MCL and limits SM-AHN to myeloid neoplasms (SM-AMN). The current study includes 329 patients with SM-Adv (median age 65 years, range 18–88; males 58%): WHO subcategories SM-AHN (N = 212; 64%), ASM (N = 99; 30%), and MCL (N = 18; 6%); ICC subcategories SM-AMN (N = 190; 64%), ASM (N = 99; 33%), and MCL (N = 9; 3%); WHO-defined MCL with “mature” MC cytomorphology and SM-AHN associated with lymphoid neoplasms were operationally labeled as “MCL-mature” (N = 9) and SM-ALN (N = 22), respectively, and distinguished from ICC-defined MCL and SM-AMN. Multivariable analysis that included the Mayo alliance risk factors for survival in SM (age >60 years, anemia, thrombocytopenia, increased alkaline phosphatase) revealed more accurate survival prediction with the ICC versus WHO classification order: (i) survival was significantly worse with MCL-immature versus MCL-mature (hazard ratio [HR] 15; p <.01), (ii) prognostic distinction between MCL and SM-AHN/AMN was confirmed in the context of ICC (HR 9.3; p <.01) but not WHO classification order (p =.99), (iii) survival was similar between MCL-mature and SM-AMN (p =.18), and (iv) SM-AMN (HR 1.7; p <.01) but not SM-ALN (p =.37) was prognostically distinct from ASM. The current study provides evidence for the independent prognostic contribution of both the ICC system for SM-Adv and the Mayo alliance risk factors for survival in SM.
AB - The World Health Organization (WHO) classification system categorizes advanced systemic mastocytosis (SM-Adv) into aggressive SM (ASM), mast cell leukemia (MCL), and SM with associated hematological neoplasm (SM-AHN). By contrast, the International Consensus Classification (ICC) requires “immature” MC cytomorphology for the diagnosis of MCL and limits SM-AHN to myeloid neoplasms (SM-AMN). The current study includes 329 patients with SM-Adv (median age 65 years, range 18–88; males 58%): WHO subcategories SM-AHN (N = 212; 64%), ASM (N = 99; 30%), and MCL (N = 18; 6%); ICC subcategories SM-AMN (N = 190; 64%), ASM (N = 99; 33%), and MCL (N = 9; 3%); WHO-defined MCL with “mature” MC cytomorphology and SM-AHN associated with lymphoid neoplasms were operationally labeled as “MCL-mature” (N = 9) and SM-ALN (N = 22), respectively, and distinguished from ICC-defined MCL and SM-AMN. Multivariable analysis that included the Mayo alliance risk factors for survival in SM (age >60 years, anemia, thrombocytopenia, increased alkaline phosphatase) revealed more accurate survival prediction with the ICC versus WHO classification order: (i) survival was significantly worse with MCL-immature versus MCL-mature (hazard ratio [HR] 15; p <.01), (ii) prognostic distinction between MCL and SM-AHN/AMN was confirmed in the context of ICC (HR 9.3; p <.01) but not WHO classification order (p =.99), (iii) survival was similar between MCL-mature and SM-AMN (p =.18), and (iv) SM-AMN (HR 1.7; p <.01) but not SM-ALN (p =.37) was prognostically distinct from ASM. The current study provides evidence for the independent prognostic contribution of both the ICC system for SM-Adv and the Mayo alliance risk factors for survival in SM.
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U2 - 10.1002/ajh.27113
DO - 10.1002/ajh.27113
M3 - Article
C2 - 37772442
AN - SCOPUS:85173485141
SN - 0361-8609
VL - 99
SP - 21
EP - 27
JO - American journal of hematology
JF - American journal of hematology
IS - 1
ER -