Pediatric Acute Myeloid Leukemia with KMT2A rearrangement -- can Partner
Gene Help Determine Need for Hematopoietic Stem Cell Transplant?
Abstract
Objective: Pediatric acute myeloid leukemia (AML) with KMT2A
rearrangement is seen in 15-20% of patients. KMT2A has been shown to
rearrange with more than 80 distinct partner genes. In this study we
examined the pattern of the various KMT2A rearrangements and their
treatment outcomes in our patient population. Methods: We
retrospectively analyzed pediatric AML patients with KMT2A rearrangement
seen in our institution between January 2005 and December 2015. Results:
There were 18 evaluable patients with equal genders. The median age was
4.12 years (range 0-13.5). FAB classification M5 was the most common
morphology. Common translocation partner was KMT2A/MLLT3. Ten patients
were treated with chemotherapy only and 8 with hematopoietic stem cell
transplantation (HSCT). There were 50% patients alive in each group.
Conclusion: KMT2A patients can be treated with both chemotherapy and
HSCT. The different fusion partners can lead to heterogeneous outcomes
in children with KMT2A rearranged AML. Further prospective studies are
needed to delineate the high-risk sub-sets in KMT2A rearranged AML that
will benefit from HSCT.