Refinement of intermediate-risk Karyotypes according to the IPSS-R in patients with myelodysplastic neoplasms (MDS).

Abstract

MDS patients show a heterogenous prognosis which can be stratified by the IPSS-R in order to derive therapeutic implications. Based on 618 patients with myelodysplastic neoplasms belonging to the cytogenetic intermediate-risk group according to IPSS-R, we show that this group is heterogeneous in terms of overall survival and cumulative risk of AML. The group can be reorganized into subgroups according to their prognostic impact. A small subgroup of patients with isolated -X or der(1;7) can be regarded as very-low-risk patients with a median survival time of 112 months and a cumulative AML progression rate of 9% after 2 years. A larger group of patients with either diverse aberrations of one chromosome or -Y + one additional aberration shows a benign course of the disease with a median survival time of 46 months and a cumulative AML progression rate of 26% after 2 years. A very large group of patients presenting with either + 8, +19, i(17q), + 21, +mar, del(9q), + 8 plus one other aberration, or del(7q) have a poor prognosis with a median survival time of 26 months and a cumulative AML progression rate of 32% after 2 years. In a very small set of patients with trisomy 11 the course of disease was similar to very-high-risk patients with a median survival time of 17 months only and a cumulative AML progression rate of 100% after 2 years. These findings could lead to a refinement of prognostic scoring systems such as the IPSS-R and the IPSS-M.