Thomas Kesiya, Rao Ranjitha, G V Chaithra, Rai Sharada, Rao A R Sneha, Basavaraju Vatsala Kudurugundi
Department of Pathology, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, Karnataka, India.
F1000Res. 2023 Oct 31;12:503. doi: 10.12688/f1000research.130522.2. eCollection 2023.
Myeloproliferative neoplasms (MPN) are clonal hematopoietic stem cell diseases characterised by myeloid cell growth from one or more lineages. Angiogenesis, in contrast to other subtypes, plays a substantial role in the pathophysiology of primary myelofibrosis (PMF). Research expressing the correlation of microvessel density (MVD), blasts, fibrosis and mast cell count in MPN cases are rarely conducted. We aimed to study the significance of MVD in correlation with CD34 blasts, mast cells and fibrosis in bone marrow biopsies of MPN patients. The current research was a cross sectional study conducted on 66 cases diagnosed as MPN during a six-year period. This comprised of 32 chronic myeloid leukemia (CML), 31 PMF and three essential thrombocythemia (ET) cases. Routine staining along with reticulin stain to look for fibrosis and immunohistochemistry (IHC) using CD34 and mast cell tryptase (MCT) were performed. We found increased MVD in PMF, when compared to CML and ET (p = 0.042). Further, mean MVD was observed to be increased with high blast counts (p = 0.036). On follow up, raised mean MVD was seen in those cases with relapse/deceased as compared to disease-free patients, which was highly significant (p = 0.000). Increased MVD score was mostly associated with PMF subtype among all the MPNs. Further, higher MVD was observed to be associated with increased blast count and poor prognosis. With angiogenesis playing a critical role in disease outcome, we now have drugs to regulate angiogenesis that are supported by contemporary research. However, further studies with larger cohorts to establish the theranostic role of MVD in MPNs is recommended.
骨髓增殖性肿瘤(MPN)是一类克隆性造血干细胞疾病,其特征为一个或多个谱系的髓系细胞生长。与其他亚型不同,血管生成在原发性骨髓纤维化(PMF)的病理生理学中起着重要作用。关于MPN病例中微血管密度(MVD)、原始细胞、纤维化和肥大细胞计数之间相关性的研究很少。我们旨在研究MPN患者骨髓活检中MVD与CD34原始细胞、肥大细胞和纤维化之间相关性的意义。本研究为一项横断面研究,对6年期间诊断为MPN的66例患者进行了研究。其中包括32例慢性髓性白血病(CML)、31例PMF和3例原发性血小板增多症(ET)病例。进行了常规染色以及用于检测纤维化的网硬蛋白染色,并用CD34和肥大细胞类胰蛋白酶(MCT)进行免疫组织化学(IHC)检测。我们发现,与CML和ET相比,PMF中的MVD增加(p = 0.042)。此外,观察到平均MVD随着高原始细胞计数而增加(p = 0.036)。随访发现,与无病患者相比,复发/死亡患者的平均MVD升高,差异具有高度统计学意义(p = 0.000)。在所有MPN中,MVD评分增加主要与PMF亚型相关。此外,观察到较高的MVD与原始细胞计数增加和预后不良相关。由于血管生成在疾病转归中起关键作用,我们现在有当代研究支持的调节血管生成的药物。然而,建议进行更大样本量的进一步研究,以确立MVD在MPN中的诊疗作用。