Interpretation of clinical practice guidelines for myelodysplastic syndrome (version 1, 2023) of National Comprehensive Cancer Nerwork(NCCN)

doi:10.16150/j.1671-2870.2023.02.002

Abstract

Abstract:

Myelodysplastic syndrome (MDS) is a diverse and common group of myeloid malignant clonal diseases in the hematologic system, with different classification and grouping, clinical manifestations and natural course, and the iteration of different classification systems and versions in history, resulting distress in the correct diagnosis, classification and treatment options. The National Comprehensive Cancer Network (NCCN) released the first version （2023） of Clinical Practice Guidelines for MDS in September 2022. The guideline is based on the clinical practice and standards of high-level evidence and the latest research progress, and its attracts wide attention from clinicians worldwide. Currently, many clinicians in China have insufficient understanding of MDS, and there is still a certain gap with the international diagnosis and treatment level of MDS. This paper interprets the content of the new guidelines, and compares it with the previous guidelines, in order to provide a reference for the standardized diagnosis and treatment of MDS at the current stage in China.

Key words: Myelodysplastic syndrome, Diagnosis, Prognosis, Guideline, Interprelation

CLC Number:

R733.3

SONG Luqian, CHANG Chunkang. Interpretation of clinical practice guidelines for myelodysplastic syndrome (version 1, 2023) of National Comprehensive Cancer Nerwork(NCCN)[J]. Journal of Diagnostics Concepts & Practice, 2023, 22(02): 116-120.

Figures/Tables 2

Table 1

Table 2

References 12

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WHO第5版分类	WHO第4版分类修订版	原始细胞
MDS，遗传学定义
MDS-5q（低原始细胞）	MDS-del(5q)	骨髓中<5%，外周血中<2%
MDS-SF3B1（低原始细胞）	MDS-伴环形铁粒幼细胞	骨髓中<5%
MDS-biTP53	-	骨髓及外周血中<20%
MDS，形态学定义
MDS伴低原始细胞	MDS伴单系发育不良,MDS伴多系发育不良	骨髓中<5%，外周血中2%~4%
MDS低增生型 MDS伴原始细胞增多	-	骨髓中<5%
MDS-伴原始细胞增多1	MDS-EB1	骨髓中5%~9%或外周血中2%~4%
MDS-伴原始细胞增多2	MDS-EB2	骨髓中10%~19%或外周血中5%~19%，奥氏小体
MDS伴骨髓纤维化	-
AML	AML	WHO：骨髓中≥20%； ICC：骨髓中≥20%或≥10%，并伴有特定分子异常

分类	预后因素	附加说明
临床因素	骨髓原始细胞比例	连续观察的指标
	血小板计数	-
	血红蛋白	-
IPSS-R细胞遗传学	低危	积分与IPSS-R相同
风险类别	中危	-
	高危	-
基因突变	16个预后基因突变	每个个体变量权重
	15个其他基因突变	该组突变的数量特征
	16个预后基因：TP53^multi、MLL^PTD、FLT3^ITD+TKD、SF3B1^5q、NPM1、RUNX1、NRAS、ETV6、IDH2、CBL、EZH2、U2AF1、SRSF2、DNMT3A、ASXL1、KRAS、SF3B1^a）
	15种其他基因突变：BCOR、BCORL1、CEBPA、ETNK1、GATA2、GNB1、IDH1、NF1、PHF6、PPM1D、PRPF8、PTPN11、SETBP1、STAG2、WT1

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