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再生障碍性贫血患者造血干细胞龛的损伤

Impairment of hematopoietic stem cell niches in patients with aplastic anemia.

作者信息

Wu Liangliang, Mo Wenjian, Zhang Yuping, Deng Hui, Li Yumiao, Zhou Ruiqing, Zhang Lu, Pan Shiyi, Wang Shunqing

机构信息

Department of Hematology, Guangzhou First People's Hospital, Guangzhou Medical University, 1 Panfu Road, Guangzhou, 510180, Guangdong, People's Republic of China.

Department of Blood Transfusion, Guangzhou First People's Hospital, Guangzhou Medical University, Guangzhou, People's Republic of China.

出版信息

Int J Hematol. 2015 Dec;102(6):645-53. doi: 10.1007/s12185-015-1881-2. Epub 2015 Oct 6.

DOI:10.1007/s12185-015-1881-2
PMID:26440975
Abstract

Aplastic anemia (AA) is a serious hematological disorder characterized by pancytopenia and defective bone marrow (BM) microenvironment. Previous investigations have demonstrated that there is a defect of angiogenesis in the BM of patients with AA. However, whether abnormalities of the BM microenvironment, particularly the overall specialization of niches, which have been classified into osteoblastic, vascular, and perivascular niches, are involved in the pathogenesis of AA is unknown. In the present study, 46 patients with AA and 15 controls were selected. The cellular elements of the BM microenvironment, including endosteal, vascular, and perivascular cells, were analyzed by immunohistochemical staining in situ. Patients with AA showed markedly fewer endosteal cells [0.33 vs 3.67 per high-power field (hpf); P < 0.05], vascular cells (8.00 vs 12.67 per hpf; P < 0.05), and perivascular cells (7.17 vs 10.67 per hpf; P < 0.05) compared with controls. These data indicate that AA is associated with impaired hematopoietic stem cell niches.

摘要

再生障碍性贫血(AA)是一种严重的血液系统疾病,其特征为全血细胞减少和骨髓(BM)微环境缺陷。既往研究表明,AA患者的骨髓存在血管生成缺陷。然而,骨髓微环境的异常,特别是已被分类为成骨细胞龛、血管龛和血管周围龛的龛位整体特化是否参与AA的发病机制尚不清楚。在本研究中,选取了46例AA患者和15例对照。通过原位免疫组化染色分析骨髓微环境的细胞成分,包括骨内膜、血管和血管周围细胞。与对照组相比,AA患者的骨内膜细胞[每高倍视野(hpf)0.33个对3.67个;P < 0.05]、血管细胞(每hpf 8.00个对12.67个;P < 0.05)和血管周围细胞(每hpf 7.17个对10.67个;P < 0.05)明显减少。这些数据表明,AA与造血干细胞龛受损有关。

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Hematopoietic stem cell loss and hematopoietic failure in severe aplastic anemia is driven by macrophages and aberrant podoplanin expression.严重再生障碍性贫血中的造血干细胞损失和造血衰竭是由巨噬细胞和异常的 podoplanin 表达驱动的。
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