Use of extracorporeal immunomodulation in a toddler with hemophagocytic lymphohistiocytosis and multisystem organ failure.
Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a dysregulated immune disorder in children, associated with Epstein–Barr virus (EBV) infection or malignancies. In severe forms, HLH presents with signs and symptoms of hyperinflammation that progress to life-threatening multiorgan failure. I...
| Publicado en: | Pediatric Nephrology Vol. 38; no. 3; pp. 927 - 932 |
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| Autores principales: | , , , , , , |
| Formato: | case study pictorial research tables/charts Journal Article |
| Publicado: |
Springer Nature
Mar2023
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=161327149&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 161327149 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 0931041X EF1 jtl: Pediatric Nephrology issn: 0931041X maglogo: N pubinfo: dt: Mar2023 vid: 38 iid: 3 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 161327149 161327149 161327149 10.1007/s00467-022-05692-1 161327149 ppf: 927 ppct: 5 formats: fmt: – @attributes: type: T – @attributes: type: P tig: atl: Use of extracorporeal immunomodulation in a toddler with hemophagocytic lymphohistiocytosis and multisystem organ failure. aug: au: Goldstein, Stuart L. Yessayan, Lenar T. Krallman, Kelli A. Collins, Michaela Benoit, Stefanie Westover, Angela Humes, H. David affil: Center for Acute Care Nephrology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, MLC 7022, 45229, Cincinnati, Ohio, USA sug: subj: Hemophagocytic Lymphohistiocytosis Diagnosis Hemophagocytic Lymphohistiocytosis Therapy Epstein-Barr Virus Infections Diagnosis Epstein-Barr Virus Infections Therapy Lymphoma, T-Cell Diagnosis Continuous Renal Replacement Therapy Methods Hematopoietic Stem Cell Transplantation Methods Treatment Outcomes Infant Multiple Organ Dysfunction Syndrome Biological Markers Blood Immune System Physiology Extracorporeal Circulation Infant: 1-23 months ab: Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a dysregulated immune disorder in children, associated with Epstein–Barr virus (EBV) infection or malignancies. In severe forms, HLH presents with signs and symptoms of hyperinflammation that progress to life-threatening multiorgan failure. Intervention with an extracorporeal immunomodulatory treatment utilizing a selective cytopheretic device (SCD) could be beneficial. The SCD with regional citrate anticoagulation selectively binds the most highly activated circulating neutrophils and monocytes and deactivates them before release to the systemic circulation. Multiple clinical studies, including a multicenter study in children, demonstrate SCD therapy attenuates hyperinflammation, resolves ongoing tissue injury and allows progression to functional organ recovery. We report the first case of SCD therapy in a patient with HLH and multi-organ failure. Case diagnosis/treatment: A previously healthy 22-month-old toddler presented with fever, abdominal distension, organomegaly, pancytopenia, and signs of hyperinflammation. EBV PCR returned at > 25 million copies. The clinical and laboratory pictures were consistent with systemic EBV-positive T-cell lymphoma with symptoms secondary to HLH. The patient met inclusion criteria for an ongoing study of integration of the SCD with a continuous kidney replacement therapy (CKRT) as part of standard of care. The patient received CKRT-SCD for 4 days with normalization of serum markers of sepsis and inflammation. The patient underwent hematopoietic stem cell transplantation 52 days after presentation and has engrafted with normal kidney function 8 months later. Conclusions: SCD treatment resulted in improvement of poor tissue perfusion reflected by rapid decline in serum lactate levels, lessened systemic capillary leak with discontinuation of vasoactive agents, and repair and recovery of lung and kidney function with extubation and removal of hemodialysis support. pubtype: Academic Journal doctype: case study pictorial research tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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