title: Adult Nephrotic Syndrome after Hematopoietic Stem Cell Transplantation: Renal Pathology is the Best Predictor of Response to Therapy.
abstract: 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.
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Ofrat
authors.initials: O
authors.lastname: Beyar-Katz
authors.affiliation: Department of Nephrology, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Etty Kruzel
authors.initials: EK
authors.lastname: Davila
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, Rambam Health Care Campus, Haifa, Israel; Rappaport Faculty of Medicine, Technion, Israel Institute of Technology, Haifa, Israel.
authors.forename: Tsila
authors.initials: T
authors.lastname: Zuckerman
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Riva
authors.initials: R
authors.lastname: Fineman
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Nuhad
authors.initials: N
authors.lastname: Haddad
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Doaa
authors.initials: D
authors.lastname: Okasha
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Israel
authors.initials: I
authors.lastname: Henig
authors.affiliation: Department of Statistics, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Ronit
authors.initials: R
authors.lastname: Leiba
authors.affiliation: Rappaport Faculty of Medicine, Technion, Israel Institute of Technology, Haifa, Israel; Department of Hematology, Shaare Zedek Medical Center, Jerusalem, Israel.
authors.forename: Jacob M
authors.initials: JM
authors.lastname: Rowe
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, Rambam Health Care Campus, Haifa, Israel; Rappaport Faculty of Medicine, Technion, Israel Institute of Technology, Haifa, Israel. Electronic address: y_ofran@rambam.health.gov.il.
authors.forename: Yishai
authors.initials: Y
authors.lastname: Ofran
mesh_terms: D000328:Adult; D006086:Graft vs Host Disease / Q000473:pathology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000209:etiology*; D011379:Prognosis; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents
keywords: Allogeneic hematopoietic stem cell transplantation; Graft-versus-host disease; Nephrotic syndrome