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系统性肥大细胞增多症患者的骨密度:与临床和组织病理学特征的相关性。

Bone mineral density in patients with systemic mastocytosis: correlations with clinical and histopathological features.

机构信息

Department of Diagnostic and Interventional Radiology, University of Leipzig, Germany.

Department of Haematology and Oncology, University of Leipzig, Germany.

出版信息

Clin Exp Rheumatol. 2021 Jan-Feb;39(1):52-57. doi: 10.55563/clinexprheumatol/y4ksv9. Epub 2020 Apr 17.

DOI:10.55563/clinexprheumatol/y4ksv9
PMID:32301423
Abstract

OBJECTIVES

Systemic mastocytosis (SM) is a heterogeneous haematological entity characterised by proliferation of mast cells. Skeletal abnormalities of SM include osteolysis, osteopenia and osteoporosis but also osteosclerosis. A routinely used modality to assess bone density is dual-energy x-ray absorptiometry (DXA). The present study sought to elucidate possible associations between DXA findings with both clinical and bone marrow biopsy findings in SM.

METHODS

Patient records of the local oncology and haematology department from 2007 to 2018 were screened for patients with SM. Overall, 39 patients (18 women and 21 men) with sufficient DXA images and clinical data were identified. We evaluated cKit mutation, tryptase level in serum, alkaline phosphatase, calcium level in serum, haemoglobin level, leucocytes and thrombocytes. Bone marrow biopsies were also evaluated.

RESULTS

There were no significant differences between the different bone marrow patterns and in regard of cKit mutations. Significant lower bone mineral density (BMD) - T-score and Z-score values were identified for the indolent type compared to aggressive type. Correlation analysis revealed an association between BMD and tryptase level (r=0.35, p=0.049), mast cell proportion in bone marrow biopsy (r=0.45, p=0.01) and with the years since diagnosis (r=-0.42, p=0.02). Moreover, the correlations differed between the indolent and aggressive type.

CONCLUSIONS

DXA findings are associated with clinical and bone marrow biopsy parameters in SM. A positive association with tryptase level and mast cell amount in bone marrow biopsies was identified. This corroborates the usefulness of DXA in SM beyond the sole assessment of osteopenia and osteoporosis.

摘要

目的

系统性肥大细胞增多症(SM)是一种以肥大细胞增殖为特征的异质性血液学实体。SM 的骨骼异常包括溶骨性、骨质疏松和骨质减少,但也包括骨质硬化。双能 X 线吸收法(DXA)是一种常用的评估骨密度的方法。本研究旨在阐明 DXA 结果与 SM 中的临床和骨髓活检结果之间可能存在的关联。

方法

筛选 2007 年至 2018 年当地肿瘤学和血液学部门的患者记录,寻找 SM 患者。总共确定了 39 名(18 名女性和 21 名男性)具有足够 DXA 图像和临床数据的患者。我们评估了 cKit 突变、血清中的类胰蛋白酶水平、碱性磷酸酶、血清中的钙水平、血红蛋白水平、白细胞和血小板。还评估了骨髓活检。

结果

不同的骨髓模式和 cKit 突变之间没有显著差异。与侵袭性类型相比,惰性类型的骨矿物质密度(BMD)-T 评分和 Z 评分值显著较低。相关性分析显示 BMD 与类胰蛋白酶水平(r=0.35,p=0.049)、骨髓活检中肥大细胞比例(r=0.45,p=0.01)和诊断后年限(r=-0.42,p=0.02)之间存在关联。此外,这些相关性在惰性和侵袭性类型之间存在差异。

结论

DXA 结果与 SM 的临床和骨髓活检参数相关。与骨髓活检中的类胰蛋白酶水平和肥大细胞数量呈正相关。这证实了 DXA 在 SM 中的有用性不仅仅是评估骨质疏松和骨质减少。

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