Pugliese Novella, Cavaliere Carlo, Basso Luca, Fazio Laura De, Malafronte Rosalia, Giordano Claudia, Vincenzi Annamaria, Varricchio Silvia, Mascolo Massimo, Martinelli Vincenzo, Picardi Marco, Salvatore Marco, Pane Fabrizio
Hematology and Hematopoietic Stem Cell Transplant Center, Department of Medicine and Surgery, University of Naples Federico II, Via S. Pansini 5, Naples, 80131, Italy.
IRCCS SYNLAB SDN, Naples, Italy.
Ann Hematol. 2025 Jan;104(1):383-388. doi: 10.1007/s00277-024-06177-x. Epub 2025 Jan 21.
Splenomegaly is a quite common clinical feature of Philadelphia (Ph) negative chronic myeloproliferative neoplasms (MPNs) and its presence may, in some cases, drives treatment decision. Most importantly, palpable splenomegaly is a minor criterion for both pre-fibrotic/early primary myelofibrosis and primary myelofibrosis (PMF) diagnosis, even if clinical assessment by physical examination is poorly reliable and accurate. On the other hand, despite the International Working Group-Myeloproliferative Neoplasms Research and Treatment and European LeukemiaNet guidelines defined spleen response criteria by palpation, they also recognized the highly subjective nature of spleen size assessment by physical examination, and recommended objective confirmation of volume reduction via computed tomography or magnetic resonance imaging (MRI). In particular, spleen volume (SV) reduction of at least 35% via MRI is typically the primary endpoint in PMF and in some polycythemia vera clinical trials. Nevertheless, this technique seems inconvenient in routine clinical practice. To simplify serial monitoring of spleen size by using ultrasonography (US), we retrospectively analyzed medical records of 39 newly diagnosed MPN patients who underwent spleen ultrasonography as well as MRI. The median SV assessed by US was 600 ml (range 200-5000 ml) while median SV evaluated by MRI was 553.1 ml (range 172-5140 ml), revealing a strong linear relationship between methods, with a correlation coefficient of r = 0.96 (95% CI 0.92-0.98, P < 0.0001). Our findings support the role of US into pre-screening assessments for clinical trials and practice, offering a pragmatic solution for evaluating SV in MPN patients and ultimately improving patient care and clinical decision-making in this complex disease landscape.
脾肿大是费城(Ph)阴性慢性骨髓增殖性肿瘤(MPN)相当常见的临床特征,在某些情况下,其存在可能会推动治疗决策。最重要的是,可触及的脾肿大是纤维化前期/早期原发性骨髓纤维化和原发性骨髓纤维化(PMF)诊断的次要标准,即使体格检查的临床评估可靠性和准确性较差。另一方面,尽管国际工作组 - 骨髓增殖性肿瘤研究与治疗组织和欧洲白血病网指南通过触诊定义了脾脏反应标准,但他们也认识到通过体格检查评估脾脏大小具有高度主观性,并建议通过计算机断层扫描或磁共振成像(MRI)客观确认体积缩小情况。特别是,通过MRI使脾脏体积(SV)至少减少35%通常是PMF和一些真性红细胞增多症临床试验的主要终点。然而,这项技术在常规临床实践中似乎不太方便。为了使用超声检查(US)简化脾脏大小的系列监测,我们回顾性分析了39例新诊断的MPN患者的病历,这些患者同时接受了脾脏超声检查和MRI检查。US评估的SV中位数为600 ml(范围200 - 5000 ml),而MRI评估的SV中位数为553.1 ml(范围172 - 5140 ml),显示两种方法之间存在很强的线性关系,相关系数r = 0.96(95% CI 0.92 - 0.98,P < 0.0001)。我们的研究结果支持US在临床试验和实践的预筛查评估中的作用,为评估MPN患者的SV提供了一种实用的解决方案,并最终改善这种复杂疾病情况下的患者护理和临床决策。