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🌱 來自: myelodysplastic syndromes

Prognosis of myelodysplastic syndromes

• mutations inTP53, ASXL1, cZH2, RUNX1, ETV6 (NEJM 2011; 364:2496) ↓ survival

  • 在 TP53、ASXL1、cZH2、RUNX1、ETV6 基因中的突變會導致患者的生存率下降。

• IPSS score—commonly used & best validated prognostic scoring system

• Correlates w/ OS & evolution to AML (Blood 1997;89:2079)

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• Revised IPSS (IPSS-R) score:
  • Assigns greater prognostic significance to cytogenetics & utilizes 5 rather than 3 prognostic subgroups; uses modified cytopenia thresholds—neutropenia as <800/uL vs. <1,800/uL (Blood 2012;120:2454)
• Complex karyotype & t-MDS have poor prog, as do Mts in TP53, EZH2, ETV6, RUNX1 & ASXL1 (NEJM 2011:364:2496); poor outcomes observed w/ TP53 Mts are specific to biallelic TP53 loss but not monoallelic TP53 Mts (Nature Med 2020;26:1549)