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目的探讨异基因造血干细胞移植(allo-HSCT)后迟发性肾病综合征(NS)的临床表现、病理特点、诊疗体会及转归,分析可能的致病因素。方法2001年2月至2006年8月 allo-HSCT后生存期大于3个月的恶性血液病患者167例,对迟发性 NS 患者的临床表现、病理特点和治疗反应进行分析总结,并分析患者性别、年龄、移植方式、预处理方案、人类白细胞抗原(HLA)配型、供受者关系、急性移植物抗宿主病(aGVHD)、慢性移植物抗宿主病(cGVHD)和巨细胞病毒抗原(CMV-Ag)定性与发生迟发性 NS 的关系。统计学分析采用 SPSS 11.0统计学软件单因素和多因素 Logistic 回归分析方法。结果167例 allo-HSCT 患者中5例发生迟发性 NS 患者,发病率为2.99%,其中膜性肾病(MGN)4例,微小病变型肾病(MCD)1例,肾脏免疫球蛋白(Ig)免疫组化3例为 IgG,1例 IgM,1例为为 IgG 和 IgM 同时存在;2例血清 ANA 阳性,1例 IgG 升高、1例 IgM 升高。糖皮质激素联合环磷酰胺为主的治疗方案效果较理想,1例完全有效、3例部分有效、1例稳定。Logistic 回归分析结果显示:移植后迟发性 NS 的发生与性别、年龄、移植方式、预处理方案、HLA 配型、供受者关系、aGVHD、cGVHD 和 CMV-Ag 定性关联均无统计学意义。结论 allo-HSCT 后迟发性 NS 病理以 MGN 为主,其次为 MCD。其发病机理可能与体液免疫异常有关,采用糖皮质激素联合环磷酰胺为主的治疗方法具有一定疗效。
Objective To investigate the clinical manifestations, pathological features, diagnosis and treatment of patients with delayed nephrotic syndrome (NS) after allo-HSCT (allo-HSCT) and to assess the possible causative factors. Methods From February 2001 to August 2006, 167 patients with malignant hematological malignancies after allo-HSCT with a survival of more than 3 months were analyzed. The clinical manifestations, pathological features and treatment responses of patients with late-onset NS were analyzed and analyzed. Gender, age, mode of transplantation, preconditioning regimen, human leukocyte antigen (HLA) matching, donor-donor relationship, acute graft versus host disease (aGVHD), chronic graft versus host disease (cGVHD), and cytomegalovirus antigen CMV-Ag) qualitative and delayed the occurrence of NS. Statistical analysis using SPSS 11.0 statistical software univariate and multivariate Logistic regression analysis. Results The incidence of delayed NS in 5 of 167 patients with allo-HSCT was 2.99%, including 4 cases of membranous nephropathy (MGN), 1 case of minimal change nephropathy (MCD), 1 case of kidney immunoglobulin (Ig) Three cases of immunohistochemistry were IgG and one case of IgM. One case had both IgG and IgM. Two cases of serum ANA were positive, one case of IgG was elevated, and one case of IgM was elevated. Glucocorticoid combined with cyclophosphamide-based treatment is more effective, 1 was completely effective, 3 cases were partially effective, 1 case was stable. Logistic regression analysis showed that the incidence of delayed NS positivity had no statistical significance with sex, age, transplantation mode, pretreatment protocol, HLA matching, donor-donor relationship, aGVHD, cGVHD and CMV-Ag. Conclusions Allo-HSCT possesses MGN as the main pathologic pathology followed by MCD. The pathogenesis may be related to humoral immune abnormalities, the use of glucocorticoid combined with cyclophosphamide-based treatment has a certain effect.