Kuendgen Andrea, Kasprzak Annika, Germing Ulrich
Department of Hematology, Oncology, and Clinical Immunology, Heinrich-Heine-University Hospital Duesseldorf, Duesseldorf, Germany.
Front Oncol. 2021 Nov 16;11:778741. doi: 10.3389/fonc.2021.778741. eCollection 2021.
The WHO-category Myelodysplastic/Myeloproliferative neoplasms (MDS/MPNs) recognizes a unique group of clonal myeloid malignancies exhibiting overlapping features of myelodysplastic as well as myeloproliferative neoplasms. The group consists of chronic myelomonocytic leukemia (CMML), atypical chronic myeloid leukemia, -negative (aCML), juvenile myelomonocytic leukemia (JMML), myelodysplastic/myeloproliferative neoplasm with ringed sideroblasts and thrombocytosis (MDS/MPN-RS-T), and myelodysplastic/myeloproliferative neoplasms, unclassifiable (MDS/MPN-U). The most frequent entity in this category is CMML, while all other diseases are extremely rare. Thus, only very limited data on the epidemiology of these subgroups exists. An appropriate diagnosis and classification can be challenging since the diagnosis is still largely based on morphologic criteria and myelodysplastic as well as myeloproliferative features can be found in various occurrences. The diseases in this category share several features that are common in this specific WHO-category, but also exhibit specific traits for each disease. This review summarizes published data on epidemiological features and offers a brief overview of the main diagnostic criteria and clinical characteristics of the five MDS/MPN subgroups.
世界卫生组织（WHO）分类中的骨髓增生异常/骨髓增殖性肿瘤（MDS/MPNs）是一组独特的克隆性髓系恶性肿瘤，具有骨髓增生异常和骨髓增殖性肿瘤的重叠特征。该组包括慢性粒单核细胞白血病（CMML）、非典型慢性髓系白血病（aCML）、幼年型粒单核细胞白血病（JMML）、伴有环形铁粒幼细胞和血小板增多的骨髓增生异常/骨髓增殖性肿瘤（MDS/MPN-RS-T）以及无法分类的骨髓增生异常/骨髓增殖性肿瘤（MDS/MPN-U）。该类别中最常见的类型是CMML，而其他所有疾病都极为罕见。因此，关于这些亚组流行病学的资料非常有限。由于诊断仍主要基于形态学标准，且在不同病例中均可发现骨髓增生异常及骨髓增殖性特征，所以做出恰当的诊断和分类颇具挑战性。该类别中的疾病具有一些在WHO这一特定分类中常见的特征，但每种疾病也有其特定的特点。本综述总结了已发表的关于流行病学特征的数据，并简要概述了五个MDS/MPN亚组的主要诊断标准和临床特征。
