Eltrombopag (thrombopoietin‐receptor agonist) and plasmapheresis as rescue therapy of acute post‐renal transplant immune thrombocytopenia in a child with Schimke immuno‐osseous dysplasia—case report. (26th September 2016)

• Record Type: Journal Article
• Authors: Grenda, Ryszard; Jarmużek, Wioletta; Latoszyńska, Joanna; Prokurat, Sylwester; Rubik, Jacek
• Is Part Of: Pediatric transplantation. Volume 20:Number 8(2016:Dec.)
• Page Start: 1148
• Page End: 1151
• Publication Date: 2016-09-26
• Subjects: acute immune thrombocytopenia -- plasmapheresis -- renal transplantation -- Schimke immuno‐osseous dysplasia -- thrombopoietin‐receptor agonist; Transplantation of organs, tissues, etc. in children -- Periodicals; 617.95408305
• DOI: 10.1111/petr.12828
• Languages: English
• ISSNs: 1397-3142

Abstract

Abstract: SIOD is rare disorder related to SMARCAL1 or SMARCAL2 gene mutation, including (among other comorbidities) T‐cell immunodeficiency, nephrotic syndrome, and renal failure. Up to 22% of primary patients may develop various autoimmune disorders. We report the case of 11‐year‐old male with SIOD, who presented ITP at 2 years after renal transplantation with decrease in platelet count (from normal) to 56 000/μL and then (gradually) to 2000/μL. There was no effect of iv. methylprednisolone/dexamethasone. As the presence of antibodies against GPIIb/IIIa, GPIb, and GPIaIIa platelet glycoproteins was confirmed, patient was given 50 g of IVIG and then was put on plasmapheresis; however, both showed poor direct effect. As we were afraid to give rituximab (due to expected overimmunosuppression), we prescribed the oral TPO‐receptor agonist (eltrombopag). Patient responded after 17 days of therapy, to the final dose of 50 mg/d (approx. 2 mg/kg). The antiplatelet antibodies disappeared after four plasmapheresis. Overall, the therapy was continued for 7 weeks and was stopped at platelet count of 433 000/μL. Platelet count remained stable in 8‐month follow‐up. Combination of plasmapheresis and TPO‐receptor agonist was effective in post‐renal transplant acute ITP in patient with SIOD.