γδ t-cell acute lymphoblastic leukemia/lymphoma: discussion of two pediatric cases and its distinction from other mature γδ t-cell malignancies

γδ t-cell acute lymphoblastic leukemia/lymphoma: discussion of two pediatric cases and its distinction from other mature γδ t-cell malignancies

;Eric X. Wei;Vasiliki Leventaki;John K. Choi;Susana C. Raimondi;Elizabeth M. Azzato;Sheila A. Shurtleff;Menchu G. Ong;Diana M. Veillon;James D. Cotelingam;Rodney E. Shackelford
Turkish journal of haematology : official journal of Turkish Society of Haematology 2017 Vol. 2017 pp. -
211
wei2017case

Abstract

Gamma delta (γδ) T-cell antigen receptor (TCR) expression and its related T-cell differentiation are not commonly reported in T-cell acute lymphoblastic leukemia/lymphoma (T-ALL). Here we report two pediatric T-ALL cases and present their clinical features, histology, immunophenotypes, cytogenetics, and molecular diagnostic findings. The first patient is a two-year-old girl with leukocytosis, circulating lymphoblasts, and a cryptic insertion of a short-arm segment at 10p12 into the long-arm segment of 11q23 resulting in an MLL and AF10 fusion transcript, which may be the first reported in γδ T-ALL. She responded to the chemotherapy protocol poorly and had persistent diseases. Following an allogeneic bone marrow transplant, she went into remission. The second patient is an eleven-year-old boy with a normal white cell count, circulating blasts, and a normal karyotype, but without any immature cellular markers by flow cytometric analysis. He responded to the chemotherapy well and achieved a complete remission. These cases demonstrate the diverse phenotypic, cytogenetic, and molecular aspects of γδ T-ALL. Early T-precursor- (ETP-) ALL and their differential diagnosis from other mature γδ T-cell leukemia/lymphomas are also discussed.

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