Inv(11)(q21q23); KMT2A-MAML2, a Recurrent Genetic Abnormality in T-Cell Therapy–related Acute Lymphoblastic Leukemia. Issue 4 (May 2020)

• Record Type: Journal Article
• Authors: Mariani, Rachel A.; Silva, Mercedes; Caparelli, Edward; Jennings, Lawrence J.; Yap, Kai Lee; Leuer, Katrin M.; Weinstein, Joanna; Gong, Shunyou
• Is Part Of: Journal of pediatric hematology/oncology. Volume 42:Issue 4(2020)
• Publication Date: 2020-05
• Subjects: therapy-related acute lymphoblastic leukemia -- T-cell ALL -- inv(11)(q21q23) -- KMT2A -- MAML2; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000001572
• Languages: English
• ISSNs: 1077-4114

Abstract

Abstract : T-cell therapy–related acute lymphoblastic leukemia (T-t-ALL) is a rare condition associated with previous cytotoxic therapy for another disease. Here we report T-t-ALL with inv(11)(q21q23), which involves KMT2A and MAML2, a transcriptional coactivator of NOTCH proteins, that occurred after chemotherapy for Philadelphia chromosome–positive B-cell acute lymphoblastic leukemia. This case describes the youngest patient with T-t-ALL harboring inv(11)(q21q23) and is the first independent report following an initial series also occurring in children. Our results lend further support to the observation that the KMT2A-MAML2 fusion gene resulting from inv(11)(q21q23) is likely a recurrent cytogenetic abnormality in T-t-ALL and appears to be associated with pediatric cases.