2022年WHO骨髓增生异常综合征分型的编码探讨

Investigation on the Coding of WHO(2022)Classification of Myelodysplastic Syndrome

骨髓增生异常综合征最新发布的WHO(2022)分型在WHO(2016)分型的基础上删减了3个分型,又新增了3个分型,当前分型对于MDS的ICD编码最早来源于FAB分型,而内在的分组逻辑与既往分型相比发生了较多的变化。MDS的WHO(2022)分型按是否伴有遗传学异常分为了2大类。在伴有遗传学异常的分型中,分为5q缺失、SF3B1突变和TP53双等位基因改变3种分型;在不伴有遗传学分型中,则是以原始细胞数量进行分类,分为MDS伴低原始细胞和MDS伴原始细胞增多,其中低原始细胞有亚型骨髓低增生MDS,高原始细胞有亚型1型、2型和骨髓纤维化MDS。最终得到关于MDS的2022年WHO分型如下:5q缺失型MDS编码为D46.7,SF3B1突变型MDS编码为D46.1,biTP53型MDS编码为D46.7,MDS伴低原始细胞编码为D46.0,MDS伴原始细胞增多编码为D46.2,儿童MDS伴低原始细胞编码为D46.7,儿童MDS伴原始细胞增多编码为D46.2。

On the latest WHO(2022)classification of MDS,three types were deleted and three new types were added on the basis of WHO(2016)classification.The ICD code for MDS in the current classification was originally inherited by FAB classification,and the internal grouping logic was changed a lot compared with the previous classification.Therefore,how to use ICD coding to better reflect the WHO(2022)typing of MDS is worth exploring.The WHO(2022)classification of MDS is divided into two categories according to whether there are genetic abnormalities.Among the types with genetic abnormalities,there are three types:5q-(MDS-5q),SF3B1 mutation(MDS-SF3B1)and TP53 double allelic gene change(MDS-biTP53);In the type without heredity,it is classified according to the number of primitive cells,which can be divided into MDS with low primitive cells(MDS-LB)and MDS with increased primitive cells(MDS-IB).MDS-LB has subtypes of myelodysplasia MDS(MDS-h),MDS-IB has subtypes MDS-IB1,MDS-IB2(the same as RAEB-1 and RAEB-2)and myelofibrosis MDS(MDS-f).The WHO(2022)classification of MDS is finally obtained as follows:MDS-5q code is D46.7,MDS-SF3B1 code is D46.1,MDS-biTP53 code is D46.7,MDS-LB and MDS-h code is D46.0,MDS-IB and MDS-f code is D46.2,cMDS-h and cMDS-LB code is D46.7,and cMDS-IB code is D46.2.