Clinicopathologic and prognostic features of TdT-negative pediatric B-lymphoblastic leukemia.

Autor: Klairmont MM; Department of Pathology, New York University School of Medicine, New York, NY, USA.; Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN, USA., Zhou Y; Department of Biostatistics, St. Jude Children's Research Hospital, Memphis, TN, USA., Cheng C; Department of Biostatistics, St. Jude Children's Research Hospital, Memphis, TN, USA., Pui CH; Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA., Jeha S; Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA., Gruber TA; Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA., Liu Y; Department of Pharmaceutical Sciences, St. Jude Children's Research Hospital, Memphis, TN, USA., Inaba H; Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA., Choi JK; Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN, USA. johnchoi@uabmc.edu.; Department of Pathology, University of Alabama at Birmingham, Birmingham, AL, USA. johnchoi@uabmc.edu.
Jazyk: angličtina
Zdroj: Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc [Mod Pathol] 2021 Nov; Vol. 34 (11), pp. 2050-2054. Date of Electronic Publication: 2021 Jun 19.
DOI: 10.1038/s41379-021-00853-3
Abstrakt: Little is known about B-lymphoblastic leukemia (B-ALL) that lacks expression of terminal deoxynucleotidyl transferase (TdT). To address this, we performed the largest study to date of TdT-negative B-ALL using data from St. Jude Total XV and XVI clinical trials. Compared to TdT-positive B-ALL (n = 896), TdT-negative B-ALL (n = 21) was associated with younger age (median, 1.4 versus 6.8 years, P < 0.001), higher white blood cell count (median, 52.8 versus 9.9 × 10 9 /L, P < 0.001), absence of hyperdiploidy (0 versus 27.8%, P = 0.002), KMT2A rearrangement (100 versus 1.9%, P < 0.001), and inferior 5-year event-free survival (EFS) (76.2 versus 90.3%, P = 0.047). In the context of KMT2A-rearranged B-ALL (n = 38), TdT-negativity was significantly associated with the MLLT1 rearrangement partner (P = 0.026) but was not independently predictive of survival, suggesting that the high-risk features of TdT-negative B-ALL are secondary to underlying KMT2A rearrangements. Finally, we compared the sensitivity of TdT-negativity to neuron-glial antigen 2 (NG.2) expression for the detection of KMT2A rearrangements and found that 63% of KMT2A-rearranged B-ALL cases not identified by NG.2 were TdT-negative. The results of this study expand the spectrum of immunophenotypic features that are specific for high-risk KMT2A rearrangements in pediatric B-ALL and can be readily implemented using existing standard acute leukemia flow cytometry panels.
(© 2021. The Author(s), under exclusive licence to United States & Canadian Academy of Pathology.)
Databáze: MEDLINE
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