Characteristics and Survival Outcome Analysis of Extramedullary Involvement in Adult Patients With t(8;21) Acute Myeloid Leukemia

Sung Soo Park, Jae Ho Yoon, Hee Je Kim, Young Woo Jeon, Sung Eun Lee, Byung Sik Cho, Ki Seong Eom, Yoo Jin Kim, Seok Lee, Chang Ki Min, Seok Goo Cho, Dong Wook Kim, Jong Wook Lee, Woo Sung Min

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6 Scopus citations

Abstract

Extramedullary (EM) involvement in acute myeloid leukemia with t(8;21) is not a rare manifestation. EM involvement has been regarded as a poor prognostic factor although t(8;21) is a favorable-risk karyotype. We analyzed 154 patients and identified EM involvement was associated with young age, leukocytosis, and c-kit mutation. Outcomes were similar after transplantation-based treatment strategy regardless of EM involvement. Background Acute myeloid leukemia (AML) with t(8;21)(q22;q22) is classified into a favorable-risk group. Extramedullary (EM) involvement has frequently been reported in this subgroup as resulting in a poor prognosis. However, characteristics or standard treatments of t(8;21) AML with EM involvement (EM-positive t(8;21)) have not yet been elucidated. Patients and Methods We retrospectively analyzed 154 adult AML patients with t(8;21). Among them, 17 were EM positive and 137 were EM negative at the time of diagnosis. EM involvement was evaluated only when a patient showed suspicious signs or symptoms. All EM-positive patients were treated according to a strategy based on allogeneic hematopoietic cell transplantation (allo-HCT). Results Central nervous system was the most frequently involved site (70.6%). EM-positive t(8;21) was associated with age ≤ 45 years, leukocytosis (≥ 30 × 109/L), and c-kit mutation compared to EM-negative t(8;21) in multivariate analysis. After intensive chemotherapy with or without local therapy, high-risk t(8;21) AML including EM-positive t(8;21) underwent allo-HCT for postremission therapy. Three-year OS (52.3% vs. 60.0%, P = .658) and event-free survival (51.5% vs. 58.0%, P = .496) were not different between the 2 groups. The subgroup of patients who underwent allo-HCT also showed similar outcomes. Conclusion EM-positive t(8;21) was associated with young age, leukocytosis, and c-kit mutation, and central nervous system was frequently involved. Allo-HCT resulted in good outcomes in EM-positive t(8;21).

Original languageEnglish
Pages (from-to)38-45.e2
JournalClinical Lymphoma, Myeloma and Leukemia
Volume17
Issue number1
DOIs
StatePublished - 1 Jan 2017

Bibliographical note

Publisher Copyright:
© 2016 Elsevier Inc.

Keywords

  • c-kit
  • Central nervous system
  • Core-binding factor
  • Extramedullary
  • Hematopoietic cell transplantation

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