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Kidney Week

Abstract: FR-PO0722

Eltrombopag-Associated Membranous Nephropathy in a Child with Immune Thrombocytopenia

Session Information

Category: Pediatric Nephrology

  • 1900 Pediatric Nephrology

Authors

  • Elsayed, Heba, Weill Cornell Medicine, New York, New York, United States
  • Salvatore, Steven, Weill Cornell Medicine, New York, New York, United States
  • Akchurin, Oleh M., Weill Cornell Medicine, New York, New York, United States
Introduction

Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by autoantibodies against platelet surface antigens and platelet destruction/impaired production. Eltrombopag (ELT), an oral thrombopoietin receptor agonist, stimulates megakaryocyte proliferation and differentiation to increase platelet production. Renal complications of ELT therapy have been reported in adults. To our knowledge, this is the first reported case of biopsy-proven membranous nephropathy in a child receiving ELT for ITP.

Case Description

An 8-year-old boy with a 3-year history of ITP (ELT-treated for 32 months) and hypogammaglobulinemia (HGG) presented with generalized edema along with symptoms of URI and otitis media (Table). Work-up revealed: nephrotic range proteinuria, hypoalbuminemia, hyperlipidemia, normal kidney ultrasound. Kidney biopsy showed diffuse membranous nephropathy (Figure). Prednisolone treatment for 6 weeks in tandem with ELT discontinuation led to complete remission (Figure). Despite two steroid-responsive relapses at age 9, patient remained relapse-free for 8 years (though developed microalbuminuria).
Family history: negative for kidney disease. The father has HGG and brother has ITP and HGG.
Final diagnosis: steroid-responsive nephrotic syndrome due to membranous nephropathy, likely induced by ELT therapy in a child with HGG.

Discussion

The mechanisms of ELT-induced nephropathies remain unclear. Whether the nephropathy in our case can be attributed exclusively to the over 2-year pre-onset course of ELT therapy is uncertain. ELT may have set the stage for a "second hit"–a viral illness–that triggered membranous nephropathy. Careful monitoring for proteinuria should be considered in pediatric patients receiving ELT, particularly those with immunologic risk factors.

Presentation
Vital Signso Temperature 36.3 °C
o HR 98-113 bpm
o RR 21-24 / min
o BP 125/84
o SpO2 100% on RA
o Dry Weight 24.7 kg
Generalo Tired, Not in acute distress
o Generalized edema
o No respiratory distress
o Slightly diminished breath sounds at bases
Gastrointestinal
SystemsHENT
o Bilateral periorbital edema
o Dull, erythematous tympanic membranes b/l

Cardiovascular
o Tachycardic, well-perfused

Respiratory
o No respiratory distress
o Slightly diminished breath sounds at bases
Gastrointestinal
o Distended abdomen with positive fluid wave
o Soft and non-tender

Musculoskeletal
o +LE pitting edema
Laboratory TestingRBCs/HPF 25-50
WBCs/HPF 25-50
Urine Protein/Creatinine Ratio 12 mg/mg
Serum Albumin 1.3 g/dL
Total Cholesterol 406 mg/d
Triglycerides 176 mg/dL
Uric Acid 3.2 mg/dL (ref 4.8-8.7)
WBC 9.9 x103/μL
Hemoglobin 12.4 g/dL
Hematocrit 37.2%
Platelets 75 x103/μL
Serum Creatinine 0.37 mg/dL
BUN 12 mg/dL
C-Reactive Protein 2.11 mg/dL (ref 0.00 - 0.99)
ESR 129 mm/h
RVP Rhinovirus/Enterovirus RNA detected
C3 / C4 complement normal
ANA / dsDNA negative
Serum PLA2R antibodies negative

Laboratory parameters and kidney biopsy

Digital Object Identifier (DOI)