Department of Nephrology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, People's Republic of China.
State Key Laboratory of Reproductive Medicine, Center of Clinical Reproductive Medicine, Center of Stem Cell Research and Clinical Practice, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, People's Republic of China.
Ren Fail. 2023 Dec;45(1):2218483. doi: 10.1080/0886022X.2023.2218483.
Calciphylaxis is a rare cutaneous vascular disease that manifests with intolerable pains, non-healing skin wounds, histologically characterized by calcification, fibrointimal hyperplasia, and microvessel thrombosis. Currently, there are no standardized guidelines for this disease. Recent studies have recognized a high prevalence of thrombophilias and hypercoagulable conditions in calciphylaxis patients. Here, we report a case of uremic calciphylaxis patient whom was refractory to conventional treatments and then received a salvage strategy with intravenous and local hAMSC application. In order to investigate the therapeutic mechanism of hAMSCs from the novel perspective of hypercoagulability, coagulation-related indicators, wound status, quality of life and skin biopsy were followed up. Polymerase chain reaction (PCR) was performed to determine the distribution of hAMSCs in multiple tissues including lung, kidney and muscle after infusion of hAMSCs for 24 h, 1 week and 1 month in mice aiming to investigate whether hAMSCs retain locally active roles after intravenous administration. Improvement of hypercoagulable condition involving correction of platelet, D-dimer and plasminogen levels, skin regeneration and pain alleviation were revealed after hAMSC administration over one-year period. Skin biopsy pathology suggested regenerative tissues after 1 month hAMSC application and full epidermal regeneration after 20 months hAMSC treatment. PCR analysis indicated that hAMSCs were homing in lung, kidney and muscle tissues of mice even until tail vein injection of hAMSCs for 1 month. We propose that hypercoagulability is a promising therapeutic target of calciphylaxis patients, which can be effectively improved by hAMSC treatment.
钙化防御是一种罕见的皮肤血管疾病,表现为难以忍受的疼痛、皮肤伤口不愈合,组织学上表现为钙化、纤维内膜增生和微血管血栓形成。目前,这种疾病没有标准化的治疗指南。最近的研究已经认识到钙化防御患者中存在高发性血栓形成倾向和高凝状态。在这里,我们报告了一例尿毒症性钙化防御患者,对常规治疗无效,随后接受了静脉和局部 hAMSC 应用的挽救性治疗策略。为了从高凝状态的新视角探讨 hAMSCs 的治疗机制,我们对凝血相关指标、伤口状况、生活质量和皮肤活检进行了随访。为了研究 hAMSCs 在静脉注射后是否能在局部保持活性,我们在小鼠中进行了聚合酶链反应(PCR),以确定 hAMSCs 在肺、肾和肌肉等多个组织中的分布,分别在注射 hAMSCs 后 24 小时、1 周和 1 个月。在长达一年的时间里,hAMSC 给药后改善了高凝状态,包括纠正血小板、D-二聚体和纤维蛋白原水平、皮肤再生和疼痛缓解。皮肤活检病理学提示 1 个月 hAMSC 应用后再生组织,20 个月 hAMSC 治疗后完全表皮再生。PCR 分析表明,hAMSCs 即使在尾静脉注射 hAMSCs 1 个月后,仍能归巢到小鼠的肺、肾和肌肉组织中。我们提出,高凝状态是钙化防御患者的一个有前途的治疗靶点,可以通过 hAMSC 治疗有效改善。