Abstract：
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.
摘要：
世界卫生组织（WHO）分类系统将晚期系统性肥大细胞增多症（SM-Adv）分类为侵袭性SM（ASM），肥大细胞白血病(MCL),和SM与相关血液肿瘤（SM-AHN）。相比之下,国际共识分类(ICC)要求“未成熟的”MC细胞形态学诊断MCL,并将SM-AHN限制为髓系肿瘤(SM-AMN).目前的研究包括329名SM-Adv患者(中位年龄65岁，范围18-88；男性58%)：WHO亚类SM-AHN(N=212；64%)，ASM（N=99；30%），和MCL(N=18；6%)；ICC子类别SM-AMN(N=190；64%)，ASM（N=99；33%），和MCL（N=9；3％）；WHO定义的具有“成熟”MC细胞形态学和与淋巴样肿瘤相关的SM-AHN的MCL在手术上标记为“MCL成熟”（N=9）和SM-ALN（N=22），分别,并区别于ICC定义的MCL和SM-AMN。多变量分析包括SM患者生存的Mayo联盟风险因素(年龄>60岁，贫血,血小板减少症,碱性磷酸酶升高)显示ICC相对于WHO分类顺序更准确的生存预测:(i)MCL未成熟相对于MCL成熟的生存率显著更差(危险比[HR]15;p<.01),（ii）MCL和SM-AHN/AMN之间的预后区别在ICC的背景下得到证实（HR9.3；p<.01），但没有WHO分类顺序（p=.99），（iii）MCL成熟和SM-AMN之间的生存率相似（p=0.18），（iv）SM-AMN（HR1.7；p<0.01）而不是SM-ALN（p=.37）在预后上与ASM不同。当前的研究为ICC系统对SM-Adv和Mayo联盟风险因素对SM生存的独立预后贡献提供了证据。
