Nachtkamp K, Schulz F, Kasprzak A, Strupp C, Hildebrandt B, Pfeilstöcker M, Valent P, Blum B, Giagounidis A, Götze K, Flatten V, Dietrich S, Kobbe G, Haase D, Gattermann N, Germing U
Department of Hematology, Oncology and Clinical Immunology, Heinrich Heine University, Duesseldorf, Germany.
Department of Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University, Moorenstr. 5, 40225, Düsseldorf, Germany.
Ann Hematol. 2025 Jun 9. doi: 10.1007/s00277-025-06443-6.
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.
骨髓增生异常综合征（MDS）患者的预后具有异质性，可通过国际预后评分系统修订版（IPSS-R）进行分层，以得出治疗意义。基于618例根据IPSS-R属于细胞遗传学中危组的骨髓增生异常肿瘤患者，我们发现该组在总生存期和急性髓系白血病（AML）累积风险方面存在异质性。根据其预后影响，该组可重新分为亚组。一小部分孤立性-X或der(1;7)患者可被视为极低危患者，中位生存期为112个月，2年后AML累积进展率为9%。一大组具有一条染色体多种畸变或-Y加一种额外畸变的患者疾病进程良性，中位生存期为46个月，2年后AML累积进展率为26%。一大组存在+8、+19、i(17q)、+21、+mar、del(9q)、+8加另一种畸变或del(7q)的患者预后较差，中位生存期为26个月，2年后AML累积进展率为32%。在一小部分11三体患者中，疾病进程与极高危患者相似，中位生存期仅为17个月，2年后AML累积进展率为100%。这些发现可能会使预后评分系统如IPSS-R和IPSS-M得到完善。
