Kim Tong Yoon, Eom Ki-Seong, Lee Ji Yoon, Lee Jong-Mi, Kim Myungshin, Lee Sung-Eun
Department of Hematology, Catholic Hematology Hospital, Yeouido St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Department of Hematology, Catholic Hematology Hospital, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Front Med (Lausanne). 2024 Nov 7;11:1461421. doi: 10.3389/fmed.2024.1461421. eCollection 2024.
Myelofibrosis, which includes primary myelofibrosis (PMF) and secondary myelofibrosis (SMF), can exhibit cytopenic features associated with poor outcomes; however, the underlying mechanisms are unclear. Moreover, characterized by its aggressive nature and limited therapeutic options, myelofibrosis poses a major clinical challenge in hematology. Therefore, in this study, we aimed to identify genetic and immunologic features associated with thrombocytopenia progression and poor prognosis.
The study involved 226 patients with PMF or SMF, who were categorized into three groups: platelet count ≥ 100 × 10/L (PLT ≥ 100 group; n = 131), progression to thrombocytopenia (PROG group; n = 64), and platelet count < 100 × 10/L (PLT < 100 group; n = 31).
Survival analysis revealed 4-year overall survival rate of 57.7%, 89.4%, and 93.9% for the PLT < 100, PROG, and PLT ≥ 100 groups, respectively. Time-dependent covariate analysis of the PLT ≥ 100 and PROG groups revealed inferior overall survival rate of the PROG group. Multivariate analysis indicated that progression to thrombocytopenia and and mutations were associated with poor overall survival. Flow cytometry revealed fewer CD45RACD4 T cells in the PROG group than in the PLT ≥ 100 group. mutations were more prevalent in the PROG group than in the other groups, correlating with a reduced number of CD45RACD4 T cells.
mutation and low CD45RACD4 T-cell counts correlated with progression to thrombocytopenia. Our findings underscore the clinical significance of thrombocytopenia dynamics in MF progression and prognosis, with implications for patient management and therapeutic interventions.
骨髓纤维化包括原发性骨髓纤维化（PMF）和继发性骨髓纤维化（SMF），可表现出与不良预后相关的血细胞减少特征；然而，其潜在机制尚不清楚。此外，骨髓纤维化具有侵袭性且治疗选择有限，是血液学领域的一项重大临床挑战。因此，在本研究中，我们旨在确定与血小板减少进展和不良预后相关的遗传和免疫特征。
该研究纳入了226例PMF或SMF患者，这些患者被分为三组：血小板计数≥100×10⁹/L（PLT≥100组；n = 131）、进展为血小板减少组（PROG组；n = 64）和血小板计数<100×10⁹/L（PLT<100组；n = 31）。
生存分析显示，PLT<100组、PROG组和PLT≥100组的4年总生存率分别为57.7%、89.4%和93.9%。对PLT≥100组和PROG组进行的时间依赖性协变量分析显示，PROG组的总生存率较低。多变量分析表明，进展为血小板减少以及[此处原文缺失部分内容]突变与不良总生存相关。流式细胞术显示，PROG组中的CD45RA⁺CD4⁺T细胞比PLT≥100组少。[此处原文缺失部分内容]突变在PROG组中比在其他组中更普遍，与CD45RA⁺CD4⁺T细胞数量减少相关。
[此处原文缺失部分内容]突变和低CD45RA⁺CD4⁺T细胞计数与进展为血小板减少相关。我们的研究结果强调了血小板减少动态变化在骨髓纤维化进展和预后中的临床意义，对患者管理和治疗干预具有启示意义。
