Standards of Pathology in the Diagnosis of Systemic Mastocytosis: Recommendations of the EU-US Cooperative Group.
Standards of Pathology in the Diagnosis of Systemic Mastocytosis: Recommendations of the EU-US Cooperative Group.
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系统性肥大细胞增多症诊断的病理学标准：欧盟-美国合作小组的建议。
DOI：
10.1016/j.jaip.2022.05.036
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发表时间：
2022
期刊：
影响因子：
--
通讯作者：
Kluin-Nelema
中科院分区：
文献类型：
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作者：
Sotlar,Karl;George,TracyI;Kluin,Philip;Reiter,Andreas;Schwaab,Juliana;Panse,Jens;Brockow,Knut;Hartmann,Karin;Sperr,WolfgangR;Kristensen,Thomas;Nedoszytko,Boguslaw;Carter,Melody;Bonadonna,Patrizia;Lyons,JonathanJ;Kluin-Nelema
病理学在系统性肥大细胞增多症（SM）的诊断、与其他肿瘤和反应性疾病的区分以及治疗中SM的监测中起着核心作用。SM的形态学标志是骨髓（BM）和其他皮外组织中梭形、低颗粒肥大细胞（MC）的积聚。病理学家可以解决5个世界卫生组织定义的诊断标准中的4个（即，致密MC聚集体[=主要标准];非典型MC形态;激活KIT点突变; MC中CD 25和/或CD 2和/或CD 30的异常表达[=次要标准]）。SM变异的最终分类为BM肥大细胞增多症、惰性SM、阴燃型SM、侵袭性SM（ASM）、SM伴相关血液学肿瘤（SM-AHN）或MC白血病（MCL）具有重要的预后意义，需要整合某些形态学、临床、放射学和生化数据，称为B-和C-结果。病理学家和临床医生可能面临重大的诊断挑战，特别是在所谓的晚期SM变体中，即ASM、MCL和SM-AHN。在这篇文章中，更新的建议，欧盟-美国合作小组关于标准的病理诊断SM，在2020年9月在维也纳举行的工作会议期间提出的，报告。
Pathology plays a central role in the diagnosis of systemic mastocytosis (SM), its delineation from other neoplasms and reactive conditions, and in monitoring of SM under therapy. The morphologic hallmark of SM is the accumulation of spindle-shaped, hypogranulated mast cells (MCs) in bone marrow (BM) and other extracutaneous tissues. Four of the 5 World Health Organization–defined diagnostic criteria (ie, compact MC aggregates [=major criterion]; atypical MC morphology; activatingKITpoint mutations; aberrant expression of CD25 and/or CD2 and/or CD30 in MCs [=minor criteria]) can be addressed by the pathologist. The final classification of SM variants as either BM mastocytosis, indolent SM, smoldering SM, aggressive SM (ASM), SM with an associated hematologic neoplasm (SM-AHN), or MC leukemia (MCL) has important prognostic significance and requires the integration of certain morphological, clinical, radiological, and biochemical data, referred to as B- and C-findings. Substantial diagnostic challenges may be posed to the pathologist and clinician especially in the so-called advanced SM variants, that is, ASM, MCL, and SM-AHN. In this article, updated recommendations of the EU-US Cooperative Group regarding standards of pathology in the diagnosis of SM, presented during the year 2020 Working Conference held in September in Vienna, are reported.