Instituto de Estudios de Mastocitosis de Castilla La Mancha, Hospital Virgen del Valle, Toledo, Spain.
Mod Pathol. 2011 Sep;24(9):1157-68. doi: 10.1038/modpathol.2011.84. Epub 2011 May 6.
Diagnosis and classification of mastocytosis is currently based on the World Health Organization (WHO) criteria. Here, we evaluate the utility of the WHO criteria for the diagnosis and classification of a large series of mastocytosis patients (n=133), and propose a new algorithm that could be routinely applied for refined diagnosis and classification of the disease. Our results confirm the utility of the WHO criteria and provide evidence for the need of additional information for (1) a more precise diagnosis of mastocytosis, (2) specific identification of new forms of the disease, (3) the differential diagnosis between cutaneous mastocytosis vs systemic mastocytosis, and (4) improved distinction between indolent systemic mastocytosis and aggressive systemic mastocytosis. Based on our results, a new algorithm is proposed for a better diagnostic definition and prognostic classification of mastocytosis, as confirmed prospectively in an independent validation series of 117 mastocytosis patients.
肥大细胞增多症的诊断和分类目前基于世界卫生组织（WHO）标准。在这里，我们评估了 WHO 标准在诊断和分类一系列肥大细胞增多症患者（n=133）中的实用性，并提出了一种新的算法，可常规用于精细诊断和分类该疾病。我们的结果证实了 WHO 标准的实用性，并提供了额外信息的必要性证据，用于（1）更精确地诊断肥大细胞增多症，（2）特定识别新形式的疾病，（3）皮肤肥大细胞增多症与系统性肥大细胞增多症之间的鉴别诊断，以及（4）改善惰性系统性肥大细胞增多症和侵袭性系统性肥大细胞增多症之间的区分。基于我们的结果，提出了一种新的算法，用于更好地诊断定义和预测分类肥大细胞增多症，在一项独立验证系列的 117 例肥大细胞增多症患者中得到了前瞻性证实。
