Successful management of factor IX inhibitor-associated nephrotic syndrome in a hemophilia B patient

Priya Verghese, Stephen Darrow, Margaret H. Kurth, Robyn C. Reed, Youngki Kim, Susan Kearney

Research output: Contribution to journalArticlepeer-review

11 Scopus citations

Abstract

Background: Nephrotic syndrome (NS) is a recognized complication of immune tolerance induction (ITI) therapy, a treatment strategy used to treat inhibitors in patients with hemophilia B receiving factor IX concentrate. Case diagnosis/treatment: We present a 4-year-old boy with hemophilia B and an inhibitor who underwent ITI, and developed NS 19 months into this therapy. A percutaneous renal biopsy was safely performed with factor IX (FIX) concentrate administration both preceding and following the procedure. The patient's inhibitor level had increased to 1.4-1.6 Bethesda Units just prior to the onset of proteinuria. Histology confirmed segmental membranous nephropathy (MGN). The patient was continued on FIX concentrate as ITI and also received 4 weekly doses of rituximab and ongoing immunosuppression with mycophenolate mofetil. This resulted in the complete resolution of his inhibitor and his NS. He continues with a modified ITI regimen and remains inhibitor-free without proteinuria >12 months post-biopsy. Conclusions: Hemophilia B patients undergoing ITI should be regularly screened for NS. At first detection of proteinuria, with proper precautions, a percutaneous kidney biopsy can be performed safely in patients with low levels of inhibitor. Our patient had segmental MGN with complete remission of NS.

Original languageEnglish (US)
Pages (from-to)823-826
Number of pages4
JournalPediatric Nephrology
Volume28
Issue number5
DOIs
StatePublished - May 1 2013

Keywords

  • Factor IX
  • Hemophilia B
  • Nephrotic syndrome
  • Segmental membranous nephropathy

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