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A long-term survivor of congenital KMT2A-R B-lymphoblastic leukemia with persistently positive bone marrow MRD and multiple CNS relapses
Journal article   Open access   Peer reviewed

A long-term survivor of congenital KMT2A-R B-lymphoblastic leukemia with persistently positive bone marrow MRD and multiple CNS relapses

Taxiarhia J Arabatzis, Biren Desai, Sandra Baril, Susan Rasul, Girmay Tekle-Yohannes and Michal Ann Miller
BMC pediatrics, Vol.25(1), pp.151-4
02/28/2025
PMID: 40016709

Abstract

Bone Marrow - pathology Central Nervous System Neoplasms - therapy Hematopoietic Stem Cell Transplantation Histone-Lysine N-Methyltransferase - genetics Humans Infant Infant, Newborn Male Myeloid-Lymphoid Leukemia Protein - genetics Neoplasm, Residual Precursor B-Cell Lymphoblastic Leukemia-Lymphoma - congenital Precursor B-Cell Lymphoblastic Leukemia-Lymphoma - genetics Precursor B-Cell Lymphoblastic Leukemia-Lymphoma - pathology Precursor B-Cell Lymphoblastic Leukemia-Lymphoma - therapy Recurrence Survivors
Here we describe the case of an infant incidentally diagnosed with congenital KMT2A-rearranged (KMT2A-r) B-cell ALL on Day of Life 4. He received the first dose of intrathecal methotrexate on DOL 5, and induction systemic therapy on DOL 6. He demonstrated morphologic remission at the end of induction but had positive bone marrow. Minimal residual disease (MRD) was 1.4%. He experienced isolated CNS disease after consolidation and immunotherapy. At 8 months of age he underwent hematopoietic stem cell transplantation (HSCT). At 14 months of age he had medullary and CNS relapse, and at 16 months of age underwent CD19 CAR-T therapy. At 6 years of age he remains in remission with tolerable developmental delays and a good quality of life.
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https://doi.org/10.1186/s12887-024-05093-3View
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