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. 2020 Jul;67(7):e28341.
doi: 10.1002/pbc.28341. Epub 2020 Apr 23.

Nationwide study of pediatric B-cell precursor acute lymphoblastic leukemia with chromosome 8q24/MYC rearrangement in Japan

Kimiyoshi Sakaguchi  1   2 , Toshihiko Imamura  2   3 , Sae Ishimaru  4   5 , Chihaya Imai  6 , Hidemi Shimonodan  7 , Naoto Fujita  8 , Keiko Okada  9 , Takeshi Taketani  10 , Rie Kanai  10 , Hisamichi Tauchi  11 , Motohiro Kato  12 , Yasuko Kojima  13 , Arata Watanabe  14 , Takao Deguchi  15 , Yoshiko Hashii  16 , Nobutaka Kiyokawa  17 , Tomohiko Taki  18 , Akiko M Saito  2 , Keizo Horibe  2 , Atsushi Manabe  19 , Atsushi Sato  20 , Katsuyoshi Koh  21
Affiliations

Nationwide study of pediatric B-cell precursor acute lymphoblastic leukemia with chromosome 8q24/MYC rearrangement in Japan

Kimiyoshi Sakaguchi et al. Pediatr Blood Cancer. 2020 Jul.

Abstract

Background: Rearrangements of chromosome 8q24/MYC (8q24/MYC-r), resulting from t(8;14)(q24;q32), t(2;8)(p11;q24), or t(8;22)(q24;q11), are mainly associated with Burkitt lymphoma/leukemia (BL) and rarely observed in patients with B-cell precursor acute lymphoblastic leukemia (BCP-ALL). The characteristics of BCP-ALL with 8q24/MYC-r are poorly understood.

Procedure: A retrospective nationwide study of data from patients with pediatric BCP-ALL with 8q24/MYC-r in Japan was conducted to clarify the clinical and biological characteristics associated with 8q24/MYC-r BCP-ALL.

Results: Ten patients with BCP-ALL with 8q24/MYC-r, including three with double-hit leukemia (DHL) (two with t(8;14)(q24;q32) and t(14;18)(q32;q21) and one with t(8;14) and t(3;22)(q27;q11)), were identified. Patients with BCP-ALL with 8q24/MYC-r had higher median age and uric acid and lactate dehydrogenase levels, than those without 8q24/MYC-r. All patients were initially treated with ALL-type chemotherapy; however, four, including one with DHL, were switched to BL-type chemotherapy, based on cytogenetic findings. One patient relapsed after standard-risk ALL-type chemotherapy, and two patients with DHL did not attain complete remission with chemotherapy; all three died within 11 months. The other seven patients treated with BL-type or high-risk ALL-type chemotherapy are alive without disease.

Conclusions: The clinical and laboratory features of BL with IG-MYC rearrangement, displaying a BCP immunophenotype (Wagener et al. and Herbrueggen et al. termed it as pre-BLL), are similar to those of BCP-ALL with 8q24/MYC-r. Low-risk ALL-type chemotherapy may not be appropriate for them, and further studies are required to establish an adequate therapeutic strategy. Further studies of DHL to identify new treatment strategies are also needed.

Keywords: 8q24/MYC rearrangement; B-cell precursor acute lymphoblastic leukemia; Burkitt lymphoma/leukemia; double-hit lymphoma/leukemia; immunophenotype.

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References

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