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

• DOI: 10.1111/petr.12828
• Publication Date: 2016-09-27T04:20:00Z
• Authors: Ryszard Grenda; Wioletta Jarmużek; Joanna Latoszyńska; Sylwester Prokurat; Jacek Rubik
• Keywords: Plasmapheresis; Eltrombopag; Thrombopoietin receptor; Romiplostim

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