Sigmund Freud University, Vienna, Austria.
Department of Internal Medicine V with Hematology, Oncology and Palliative Medicine, Hospital Hietzing, Wolkersbergenstraße 1, 1130, Vienna, Austria.
Wien Klin Wochenschr. 2019 Sep;131(17-18):410-418. doi: 10.1007/s00508-019-1526-1. Epub 2019 Jul 18.
In the Austrian biodatabase for chronic myelomonocytic leukemia (ABCMML) clinicolaboratory real-life data have been captured from 606 CMML patients from 14 different hospitals over the last 30 years. It is the only large biodatabase worldwide in which functional methods such as semisolid in vitro cultures complement modern molecular methods such as next generation sequencing. This provides the possibility to comprehensively study the biology of CMML. The aim of this study was to compare patient characteristics with published CMML cohorts and to validate established prognostic parameters in order to examine if this real-life database can serve as a representative and useful data source for further research. After exclusion of patients in transformation characteristics of 531 patients were compared with published CMML cohorts. Median values for age, leukocytes, hemoglobin, platelets, lactate dehydrogenase (LDH) and circulating blasts were within the ranges of reported CMML series. Established prognostic parameters including leukocytes, hemoglobin, blasts and adverse cytogenetics were able to discriminate patients with different outcome. Myeloproliferative (MP) as compared to myelodysplastic (MD)-CMML patients had higher values for circulating blasts, LDH, RAS-pathway mutations and for spontaneous myelomonocytic colony growth in vitro as well as more often splenomegaly. This study demonstrates that the patient cohort of the ABCMML shares clinicolaboratory characteristics with reported CMML cohorts from other countries and confirms phenotypic and genotypic differences between MP-CMML and MD-CMML. Therefore, results obtained from molecular and biological analyses using material from the national cohort will also be applicable to other CMML series and thus may have a more general significance.
在奥地利慢性粒单核细胞白血病(CMML)的生物数据库(ABCMML)中,从过去 30 年来的 14 家不同医院的 606 名 CMML 患者中获取了临床实验室的真实数据。这是全球唯一的大型生物数据库,其中功能方法(如半固体体外培养)补充了现代分子方法(如下一代测序)。这为全面研究 CMML 的生物学提供了可能。本研究的目的是比较患者特征与已发表的 CMML 队列,并验证已建立的预后参数,以检查这个真实世界的数据库是否可以作为进一步研究的代表性和有用的数据源。在排除转化患者后,比较了 531 例患者的特征与已发表的 CMML 队列。年龄、白细胞、血红蛋白、血小板、乳酸脱氢酶(LDH)和循环blasts 的中位数值均在报道的 CMML 系列范围内。已建立的预后参数包括白细胞、血红蛋白、blasts 和不良细胞遗传学,能够区分不同结局的患者。与骨髓增生异常(MD)-CMML 患者相比,骨髓增生性(MP)-CMML 患者的循环blasts、LDH、RAS 通路突变和体外自发髓单核细胞集落生长更高,脾肿大更常见。这项研究表明,ABCMML 的患者队列与来自其他国家的报道的 CMML 队列具有相似的临床实验室特征,并证实了 MP-CMML 和 MD-CMML 之间的表型和基因型差异。因此,使用来自国家队列的材料进行的分子和生物学分析结果也将适用于其他 CMML 系列,因此可能具有更普遍的意义。