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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
Author(s) -
Grenda Ryszard,
Jarmużek Wioletta,
Latoszyńska Joanna,
Prokurat Sylwester,
Rubik Jacek
Publication year - 2016
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/petr.12828
Subject(s) - medicine , plasmapheresis , eltrombopag , thrombopoietin receptor , immune thrombocytopenia , thrombopoietin , romiplostim , agonist , gastroenterology , immunology , receptor , antibody , genetics , stem cell , haematopoiesis , biology
Abstract SIOD is rare disorder related to SMARCAL 1 or SMARCAL 2 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 GPII b/ III a, GPI b, and GPI a II a 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 .