Monoclonal IgG4/2κ Deposition Following Eculizumab Therapy for Recurrent Atypical Hemolytic Uremic Syndrome in Kidney Transplantation.
Eculizumab
aHUS
thrombotic microangiopathy
Journal
Kidney medicine
ISSN: 2590-0595
Titre abrégé: Kidney Med
Pays: United States
ID NLM: 101756300
Informations de publication
Date de publication:
Historique:
entrez:
1
8
2020
pubmed:
9
5
2019
medline:
9
5
2019
Statut:
epublish
Résumé
Eculizumab is an emerging therapy for atypical hemolytic uremic syndrome (aHUS). Early identification and treatment of recurrent aHUS after kidney transplantation requires a high clinical suspicion but results in improved graft function and patient outcome. We present a patient who developed recurrent aHUS after kidney transplantation that responded to eculizumab therapy. A kidney biopsy was performed to confirm resolution of thrombotic microangiopathy 8 weeks after eculizumab treatment initiation and revealed no features of thrombotic microangiopathy. Instead, the biopsy revealed monoclonal immunoglobulin G (IgG)4/2κ deposition in the glomerular tufts, vasculature, and atrophic tubular basement membranes. IgG4/2κ deposits are a rare pathologic finding following eculizumab therapy, and the long-term effect of these deposits on kidney function remains unknown.
Identifiants
pubmed: 32734194
doi: 10.1016/j.xkme.2019.03.005
pii: S2590-0595(19)30030-5
pmc: PMC7380410
doi:
Types de publication
Case Reports
Langues
eng
Pagination
139-143Subventions
Organisme : NHLBI NIH HHS
ID : R01 HL132325
Pays : United States
Commentaires et corrections
Type : ErratumIn
Informations de copyright
© 2019 The Authors.
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