Nina S Ma1, Ami J Shah, Mitchell E Geffner, Neena Kapoor. 1. Division of Endocrinology, Diabetes, and Metabolism, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA. nina.ma@childrens.harvard.edu
Abstract
CONTEXT: Children with severe combined immunodeficiency who receive a T cell-depleted hematopoietic stem cell transplantation (HSCT) have delayed immune reconstitution. OBJECTIVE: To examine the use of recombinant human insulin-like growth factor-I (rhIGF-I) therapy to stimulate thymopoiesis after HSCT. CLINICAL CASE: A child with Omenn syndrome failed T cell reconstitution 6 months after HSCT. She started rhIGF-I therapy just before age 18 months. The initial dose (40 microg/kg twice daily) was increased every 2 weeks to a maximum dose of 120 microg/kg twice daily. RESULTS: The patient's absolute T cells increased from 7 to 132/mm(3) and 662/mm(3) after 3 and 5 months of rhIGF-I therapy, respectively, and her blastogenic response to phytohemagglutinin normalized. Three months after discontinuation of rhIGF-I therapy, the T cells continued to increase (to 2,427/mm(3)) although the blastogenic response to phytohemagglutinin decreased. CONCLUSION: This is the first known use of rhIGF-I therapy to help restore thymopoiesis in a child.
CONTEXT: Children with severe combined immunodeficiency who receive a T cell-depleted hematopoietic stem cell transplantation (HSCT) have delayed immune reconstitution. OBJECTIVE: To examine the use of recombinant humaninsulin-like growth factor-I (rhIGF-I) therapy to stimulate thymopoiesis after HSCT. CLINICAL CASE: A child with Omenn syndrome failed T cell reconstitution 6 months after HSCT. She started rhIGF-I therapy just before age 18 months. The initial dose (40 microg/kg twice daily) was increased every 2 weeks to a maximum dose of 120 microg/kg twice daily. RESULTS: The patient's absolute T cells increased from 7 to 132/mm(3) and 662/mm(3) after 3 and 5 months of rhIGF-I therapy, respectively, and her blastogenic response to phytohemagglutinin normalized. Three months after discontinuation of rhIGF-I therapy, the T cells continued to increase (to 2,427/mm(3)) although the blastogenic response to phytohemagglutinin decreased. CONCLUSION: This is the first known use of rhIGF-I therapy to help restore thymopoiesis in a child.
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