Institute of Clinical Radiology and Nuclear Medicine, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.
Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Theodor-Kutzer-Ufer 1-3, 68167, Mannheim, Germany.
Sci Rep. 2022 Aug 20;12(1):14228. doi: 10.1038/s41598-022-18537-6.
Systemic mastocytosis (SM) is characterized by multifocal accumulation of neoplastic mast cells (MCs), predominately affecting the bone marrow (BM). Imaging with computed tomography (CT) is used for assessment of bone mineral density and structure. However, the value of functional imaging with dual-energy CT (DECT) and the assessment of virtual-non-calcium attenuation values (VNCa-AV) for visualization of BM disease burden in SM has not yet been assessed. DECT of the axial skeleton was performed in 18 patients with SM (indolent SM [ISM], n = 6; smoldering SM [SSM]/advanced SM [AdvSM], n = 12) and 18 control subjects. VNCa-AV were obtained in 5 representative vertebraes per patient and correlated with laboratory, morphologic and molecular parameters. VNCa-AV strongly correlated with quantitative BM MC infiltration (r = 0.7, R = 0.49, P = 0.001) and serum tryptase levels (r = 0.7, R = 0.54, P < 0.001). Mean VNCa-AV were significantly higher in SSM/AdvSM as compared to ISM (- 9HU vs. - 54HU, P < 0.005) and controls (- 38HU, P < 0.005). Nine of 10 (90%) patients with a VNCa-AV > - 30HU and 7/7 (100%) patients with a VNCa-AV > - 10HU had SSM or AdVSM. BM VNCa-AV provide information about the MC burden of SM patients and correlate with SM subtypes. DECT may therefore serve as a supplementary tool for SM diagnosis, subclassification and monitoring in a one-stop-shop session.
系统性肥大细胞增多症（SM）的特征是多灶性的肿瘤性肥大细胞（MC）积聚，主要影响骨髓（BM）。计算机断层扫描（CT）用于评估骨矿物质密度和结构。然而，双能 CT（DECT）的功能成像和虚拟非钙衰减值（VNCa-AV）评估 SM 中 BM 疾病负担的价值尚未得到评估。对 18 例 SM 患者（惰性 SM [ISM]，n=6；冒烟型 SM [SSM]/进展型 SM [AdvSM]，n=12）和 18 例对照者进行了轴向骨骼 DECT 检查。在每个患者的 5 个代表性椎体中获得 VNCa-AV，并与实验室、形态学和分子参数相关。VNCa-AV 与定量 BM MC 浸润强烈相关（r=0.7，R=0.49，P=0.001）和血清胰蛋白酶水平（r=0.7，R=0.54，P<0.001）。SSM/AdvSM 的平均 VNCa-AV 明显高于 ISM（-9HU 与-54HU，P<0.005）和对照组（-38HU，P<0.005）。VNCa-AV> - 30HU 的 10 例患者中的 9 例（90%）和 VNCa-AV> - 10HU 的 7 例患者中的 7 例（100%）患有 SSM 或 AdvSM。BM VNCa-AV 提供了关于 SM 患者 MC 负担的信息，并与 SM 亚型相关。因此，DECT 可以作为 SM 诊断、分类和监测的补充工具，在一次就诊中进行。
