Case report: Use of emapalumab-lzsg for secondary hemophagocytic lymphohistiocytosis in a patient with chronic granulomatous disease
Presentation Time: 03:15 PM - 04:30 PM
Poster Board Number: B658
Abstract ID: 5145
Presenting Author:
Raveena B Patel , Pediatric Resident Physician at Nemours Children's Hosp.
Abstract:
Background: Chronic granulomatous disease (CGD) is an immunodeficiency marked by infections due to NADPH oxidase defects. A hyperinflammatory response predisposes to granuloma formation and hemophagocytic lymphohistiocytosis (HLH), a syndrome defined by uncontrolled cytokine release. We present an adolescent with undiagnosed CGD who developed secondary HLH, requiring the use of emapalumab-lzsg, a human anti-interferon-gamma antibody. To our knowledge, this is the first report of emapalumab-Izsg use in a patient with secondary HLH with CGD.
Case Presentation: A 16-year-old male with Chron’s disease presented with six months of fevers. Imaging showed reticulonodular disease and granulomas throughout the lungs. Respiratory status deteriorated, requiring intubation and ECMO. Blood cultures grew Burkholderia cepacia. Flow cytometry was abnormal and genetic tests confirmed CGD. Further clinical and lab derangements led to HLH testing. HLH was confirmed and treated with steroids, etoposide, and intravenous immunoglobulin. With no clinical improvement, emapalumab-Izsg was recommended. Six-week course of emapalumab-Izsg led to disease regression, with decline of CXCL-9, a marker of active disease.
Conclusion: Emapalumab-Izsg has been used in primary HLH while utility in secondary HLH remains undefined. The standard of care for secondary HLH includes immunosuppressive and chemotherapeutic therapies. We propose that emapalumab-Izsg be strongly considered an option in secondary HLH.
Case report: Use of emapalumab-lzsg for secondary hemophagocytic lymphohistiocytosis in a patient with chronic granulomatous disease
Category
Poster