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American Academy of Pediatrics
Case Report

Emicizumab-Induced Seronegative Full-House Lupus Nephritis in a Child

Hassib Chehade, Francois Cachat, Maja Beck-Popovic, Samuel Rotman, Léonore Diezi, Manuela Albisetti, Lorenzo Alberio, Guy Young and Mattia Rizzi
Pediatrics November 2020, 146 (5) e20200123; DOI: https://doi.org/10.1542/peds.2020-0123
Hassib Chehade
aPediatric Nephrology Unit and
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Francois Cachat
aPediatric Nephrology Unit and
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Maja Beck-Popovic
bPediatric Hematology-Oncology Unit, Division of Pediatrics, Department Woman-Mother-Child and
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Samuel Rotman
cServices of Clinical Pathology and
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Léonore Diezi
dClinical Pharmacology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland;
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Manuela Albisetti
eDepartment of Pediatrics, University Children’s Hospital Zurich and University of Zurich, Zurich, Switzerland; and
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Lorenzo Alberio
fDivision of Hematology and Central Hematology Laboratory and
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Guy Young
gCancer and Blood Disease Institute, Children’s Hospital Los Angeles and Keck School of Medicine, University of Southern California, Los Angeles, California
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Mattia Rizzi
bPediatric Hematology-Oncology Unit, Division of Pediatrics, Department Woman-Mother-Child and
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Abstract

Hemophilia A (HA) is a serious inherited bleeding disorder resulting from a deficiency of coagulation factor VIII (FVIII). Replacement therapy with intravenous infusion of FVIII can be associated with treatment failure in approximately one-third of patients secondary to the development of neutralizing alloantibodies (inhibitor). Emicizumab is a recombinant, humanized, bispecific monoclonal antibody that binds factor IXa and factor X and mimics FVIII. It has been licensed in many countries for the treatment of patients with HA with and without inhibitors with a favorable efficacy and safety profile. A 7-year-old child with severe HA and FVIII inhibitors, refractory to immune tolerance therapy, developed hematuria with nephrotic-range proteinuria after the first dose of emicizumab and subsequently also after a second dose 6 weeks later, which was associated with mild and transient leukopenia. Renal biopsy revealed a pattern of a full-house lupus nephritis. The patient fully and spontaneously recovered between 2 weeks after symptoms onset. In this report, we provide insights on a new and so far unreported renal complication associated to emicizumab treatment. Although emicizumab offers significant benefits for patient with HA, clinicians should be aware of this rare and potential serious renal adverse effect.

  • Accepted March 13, 2020.
  • Copyright © 2020 by the American Academy of Pediatrics

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Pediatrics
Vol. 146, Issue 5
1 Nov 2020
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Emicizumab-Induced Seronegative Full-House Lupus Nephritis in a Child
Hassib Chehade, Francois Cachat, Maja Beck-Popovic, Samuel Rotman, Léonore Diezi, Manuela Albisetti, Lorenzo Alberio, Guy Young, Mattia Rizzi
Pediatrics Nov 2020, 146 (5) e20200123; DOI: 10.1542/peds.2020-0123

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Emicizumab-Induced Seronegative Full-House Lupus Nephritis in a Child
Hassib Chehade, Francois Cachat, Maja Beck-Popovic, Samuel Rotman, Léonore Diezi, Manuela Albisetti, Lorenzo Alberio, Guy Young, Mattia Rizzi
Pediatrics Nov 2020, 146 (5) e20200123; DOI: 10.1542/peds.2020-0123
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