Igf-I stimulates in vivo thymopoiesis after stem cell transplantation in a child with Omenn syndrome.
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
This is the first known use of rhIGF-I therapy to help restore thymopoiesis in a child.
Ma
Nina S
NS
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
Shah
Ami J
AJ
Geffner
Mitchell E
ME
Kapoor
Neena
N
D001424:Bacterial Infections / Q000150:complications / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*; D002452:Cell Count; D003967:Diarrhea; D064420:Drug-Related Side Effects and Adverse Reactions; D005260:Female; D005334:Fever; D006801:Humans; D007114:Immunization; D007223:Infant; D007334:Insulin-Like Growth Factor I / Q000008:administration & dosage* / Q000009:adverse effects; D008213:Lymphocyte Activation / Q000187:drug effects; D038041:Lymphopoiesis / Q000187:drug effects; D010835:Phytohemagglutinins / Q000276:immunology; D012008:Recurrence; D016511:Severe Combined Immunodeficiency / Q000150:complications / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*; D033581:Stem Cell Transplantation; D013601:T-Lymphocytes / Q000187:drug effects* / Q000276:immunology; D013950:Thymus Gland / Q000187:drug effects* / Q000254:growth & development / Q000473:pathology
D010835:Phytohemagglutinins; D007334:Insulin-Like Growth Factor I