title: Glomerulonephritis after hematopoietic cell transplantation: IgA nephropathy with increased excretion of galactose-deficient IgA1.
abstract: We report the development of IgA nephropathy (IgAN) following full myeloablative allogeneic hematopoietic cell transplantation in two patients with human leukocyte antigen (HLA) matched sibling donors, unrelated to active or chronic graft-versus-host disease. Both recipients had elevated urinary levels of galactose-deficient IgA1, and one donor-recipient pair had elevated serum levels of galactose-deficient IgA1. We propose that IgAN developed after bone marrow transplantation due to a non-graft-versus-host-disease-related multi-hit process associated with glomerular deposition of galactose-deficient IgA1. These two cases provide unique insight into the kinetics of overproduction of galactose-deficient IgA1 and its glomerular deposition and consequential renal injury in IgAN.
authors.affiliation: Division of Kidney Disease and Hypertension, Department of Medicine, Brown University, Warren Alpert Medical School, Providence, RI, USA. Shu@Lifespan.org
authors.forename: Susie L
authors.initials: SL
authors.lastname: Hu
authors.forename: Gerald A
authors.initials: GA
authors.lastname: Colvin
authors.forename: Abdalla
authors.initials: A
authors.lastname: Rifai
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Suzuki
authors.forename: Jan
authors.initials: J
authors.lastname: Novak
authors.forename: Alfredo
authors.initials: A
authors.lastname: Esparza
authors.forename: Shahida
authors.initials: S
authors.lastname: Farooqi
authors.forename: Bruce A
authors.initials: BA
authors.lastname: Julian
mesh_terms: D000328:Adult; D005690:Galactose / Q000172:deficiency*; D005921:Glomerulonephritis / Q000209:etiology* / Q000473:pathology; D005922:Glomerulonephritis, IGA / Q000209:etiology* / Q000473:pathology; D006086:Graft vs Host Disease / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007070:Immunoglobulin A / Q000378:metabolism*; D007668:Kidney / Q000473:pathology; D008297:Male; D008875:Middle Aged
chemical_list: D007070:Immunoglobulin A; D005690:Galactose