Myeloid neoplasms and acute leukemias include different entities that have been recently re-classified taking into account molecular and clinicopathological features. The myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN) category comprises a heterogeneous group of hybrid neoplastic myeloid diseases characterized by the co-occurrence of clinical and pathological features of both myelodysplastic and myeloproliferative neoplasms. The most frequent entity in this category is chronic myelomonocytic leukemia (CMML) which is, after acute myeloid leukemia (AML), the main myeloid disorder prone to develop cutaneous manifestations. Skin lesions associated with myelodysplastic and myeloproliferative neoplasms include a broad clinical, histopathological and molecular spectrum of lesions, poorly understood and without a clear-cut classification in the current medical literature. The aim of this review is to describe and classify the main clinical, histopathological and molecular patterns of cutaneous lesions in the setting of MDS/MPN in order to improve the diagnostic skills of the dermatologists, hematologists and pathologists who deal with these patients.
髓系肿瘤和急性白血病包含多种不同实体,近期已根据分子与临床病理学特征进行了重新分类。骨髓增生异常综合征/骨髓增殖性肿瘤(MDS/MPN)类别包含一组异质性混合型髓系肿瘤疾病,其特征是同时存在骨髓增生异常和骨髓增殖性肿瘤的临床与病理学表现。该类别中最常见的实体是慢性粒-单核细胞白血病(CMML),这是继急性髓系白血病(AML)之后最易出现皮肤表现的主要髓系疾病。与骨髓增生异常及骨髓增殖性肿瘤相关的皮肤病变涵盖广泛的临床、组织病理学和分子谱系,目前医学文献对此认识不足且缺乏明确分类。本综述旨在系统描述和分类MDS/MPN背景下皮肤病变的主要临床、组织病理学及分子模式,以提升皮肤科医生、血液科医生和病理医生对此类患者的诊断能力。