Niscola Pasquale, Tendas Andrea, Luo Xiao-dan, Catalano Gianfranco, Scaramucci Laura, Cupelli Luca, Giovannini Marco, Ferrannini Michele, Bondanini Francesco, Piccioni Daniela, Dentamaro Teresa, Palumbo Roberto, Perrotti Alessio Pio, Liu Qi-fa, de Fabritiis Paolo
Hematology Unit, Saint Eugenio Hospital, Tor Vergata University of Rome, Italy.
Cardiovasc Hematol Agents Med Chem. 2013 Mar;11(1):67-76. doi: 10.2174/1871525711311010011.
membranous glomerulopathy (MG) is an immunomediated disorder which accounts for the most common cause of nephrotic syndrome (NS) following allogeneic hematopoietic stem cell transplantation (HSCT).
to provide an update on the issue by reviewing pertinent literature on the MEDLINE database.
sixty-nine post allogenic HSCT patients (42 male) with MG were identified. The median age was 43 (5 to 68) years. Time interval from allogenic HSCT to MG diagnosis ranged from 3 to 134 months (median 17). Most MG patients had a history of acute (70%) or chronic (84%) graft versus host disease (GVHD). Corticosteroids and cyclosporine were the most common therapeutic agents used in this setting; alternative therapies, including rituximab, were given to a lower number of patients. Outcome data were available in 64 out of 69 MG patients; 38 (59%) and 18 (28%) patients achieved a complete and a partial response respectively, whereas treatment failure was recorded in the remaining 8 (13%).
MG after allogenic HSCT appears to be associated with a sub clinical or overt cGVHD, which follows the discontinuation of immunosuppressive prophylaxis. Although a standard therapeutic approach has not been established, the application of available measures can induce favorable response in more than 80% of affected patients, but treatment-failure and progressive deterioration of the renal function may occur in about one fifth of cases.
膜性肾小球病(MG)是一种免疫介导性疾病,是异基因造血干细胞移植(HSCT)后肾病综合征(NS)最常见的病因。
通过检索MEDLINE数据库中的相关文献,对该问题进行更新。
共识别出69例异基因HSCT后发生MG的患者(42例男性)。中位年龄为43岁(5至68岁)。从异基因HSCT到MG诊断的时间间隔为3至134个月(中位时间17个月)。大多数MG患者有急性(70%)或慢性(84%)移植物抗宿主病(GVHD)病史。糖皮质激素和环孢素是该情况下最常用的治疗药物;包括利妥昔单抗在内的其他治疗方法应用于较少数量的患者。69例MG患者中有64例有结局数据;38例(59%)患者完全缓解,18例(28%)患者部分缓解,其余8例(13%)治疗失败。
异基因HSCT后的MG似乎与亚临床或显性慢性GVHD相关,这发生在免疫抑制预防措施停用之后。虽然尚未确立标准的治疗方法,但应用现有措施可使超过80%的受影响患者获得良好反应,但约五分之一的病例可能出现治疗失败和肾功能进行性恶化。