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Mendelian susceptibility to mycobacterial disease-Challenges in hematopoietic stem cell transplantation

Authors :
Venkateswaran Vellaichamy Swaminathan
Shivani Patel
Kesavan Melarcode Ramanan
Nikila Ravichandran
Ramya Uppuluri
Revathi Raj
Source :
Pediatric bloodcancerREFERENCES. 67(5)
Publication Year :
2019

Abstract

We present our experience in the hematopoietic stem cell transplantation (HSCT) in two children diagnosed with Mendelian susceptibility to mycobacterial diseases. The first child underwent a haploidentical HSCT with posttransplant cyclophosphamide using a reduced intensity conditioning following which he had primary graft failure. He was subsequently found to have interferon-γ1 receptor deficiency. He had immune reconstitution and is on antitubercular therapy. The second child diagnosed with IL12RB1 gene mutation underwent matched sibling donor HSCT with myeloablative conditioning following pretransplant immunosuppression with fludarabine and dexamethasone. He is 13 months post-HSCT with complete and remains disease free.

Details

ISSN :
15455017
Volume :
67
Issue :
5
Database :
OpenAIRE
Journal :
Pediatric bloodcancerREFERENCES
Accession number :
edsair.doi.dedup.....10126adba548a05ac490c6c4ee8a1a6c