| Literature DB >> 31250550 |
Saman K Hashmi1, Jyotinder N Punia2, Andrea N Marcogliese2, Amos S Gaikwad2, Kevin E Fisher1,2, Angshumoy Roy2, Pulivarthi Rao2, Dolores H Lopez-Terrada2, Jo Ringrose2, Mignon L Loh3, Charlotte M Niemeyer4,5,6, Rachel E Rau1.
Abstract
Juvenile myelomonocytic leukemia (JMML) has a poor prognosis in general, with hematopoietic stem cell transplant (HSCT) remaining the standard of care for cure. The hypomethylating agent, azacitidine, has been used as a bridging therapy to transplant. However, no patients have been treated with azacitidine without an HSCT post azacitidine. We report on an infant with JMML with somatic KRAS G12A mutation and monosomy 7 who achieved sustained remission following azacitidine monotherapy. He also developed an aberrant B-lymphoblast population which declined with similar kinetics as his JMML-associated abnormalities, suggesting that a B-lymphoblast population in JMML does not always progress to acute leukemia.Entities:
Keywords: B-lymphoblasts; JMML; azacitidine
Mesh:
Substances:
Year: 2019 PMID: 31250550 PMCID: PMC7328527 DOI: 10.1002/pbc.27905
Source DB: PubMed Journal: Pediatr Blood Cancer ISSN: 1545-5009 Impact factor: 3.167