Beyar-Katz Ofrat, Davila Etty Kruzel, Zuckerman Tsila, Fineman Riva, Haddad Nuhad, Okasha Doaa, Henig Israel, Leiba Ronit, Rowe Jacob M, Ofran Yishai
Department of Hematology and Bone Marrow Transplantation, Rambam Health Care Campus, Haifa, Israel.
Department of Nephrology, Rambam Health Care Campus, Haifa, Israel.
Biol Blood Marrow Transplant. 2016 Jun;22(6):975-981. doi: 10.1016/j.bbmt.2015.12.014. Epub 2015 Dec 29.
Nephrotic syndrome (NS) after allogeneic hematopoietic stem cell transplantation (HSCT) is a rare phenomenon usually associated with graft-versus-host disease (GVHD). This systematic review of post-HSCT NS cases reported in the literature aimed to identify risk factors and unique features of the disease in this clinical setting. One hundred sixteen cases of post-HSCT NS published in the English literature between 1988 and 2015 were revealed and analyzed. The median onset of NS was 20.5 months (range, 3 to 174) post-HSCT. NS development was associated with acute or chronic GVHD in 87.2% of cases. Membranous nephropathy (MGN) was the most frequent pathology (65.5%), followed by minimal change disease (MCD) (19%). Complete remission of the NS was achieved in 63.5% of patients (59.1% of MGN cases and 81.3% of MCD cases; P = .15). Patients presenting with MCD recovered at a median of 1.75 months (range, 1 to 12) and with MGN a median of 7 months (range, 1 to 53) (P = .001). NS was treated with corticosteroids alone in 16.8% of patients and with a combination of corticosteroids and other immunosuppressive agents in 73.5% of patients. Univariate analysis failed to identify a single predictive factor of response to therapy. In conclusion, post-HSCT NS usually develops concomitant to GVHD and is associated with high rates of response to therapy. Although most patients were treated with a combination of immunosuppressive drugs, single-agent therapy with steroids may be sufficient in some cases.
异基因造血干细胞移植（HSCT）后发生的肾病综合征（NS）是一种罕见现象，通常与移植物抗宿主病（GVHD）相关。本对文献中报道的HSCT后NS病例的系统评价旨在确定该临床背景下该疾病的危险因素和独特特征。对1988年至2015年间发表在英文文献中的116例HSCT后NS病例进行了揭示和分析。NS的中位发病时间为HSCT后20.5个月（范围3至174个月）。87.2%的病例中NS的发生与急性或慢性GVHD相关。膜性肾病（MGN）是最常见的病理类型（65.5%），其次是微小病变病（MCD）（19%）。63.5%的患者实现了NS的完全缓解（MGN病例为59.1%，MCD病例为81.3%；P = 0.15）。表现为MCD的患者中位恢复时间为1.75个月（范围1至12个月），表现为MGN的患者中位恢复时间为7个月（范围1至53个月）（P = 0.001）。16.8%的患者仅用皮质类固醇治疗NS，73.5%的患者用皮质类固醇和其他免疫抑制剂联合治疗。单因素分析未能确定治疗反应的单一预测因素。总之，HSCT后NS通常与GVHD同时发生，且对治疗的反应率较高。尽管大多数患者接受了免疫抑制药物联合治疗，但在某些情况下，单用类固醇治疗可能就足够了。
