Characteristics and outcome of patients with acute myeloid leukaemia and t(8;16)(p11;p13): results from an International Collaborative Study

Autor: Uwe Platzbecker, Maria R. Baer, Benedikt Brors, Zuzana Šustková, Richard F. Schlenk, Tomáš Szotkowski, Pavel Zak, Carsten Müller-Tidow, Gregor Warsow, Francesca Guijarro, Sabine Kayser, Alan Kenneth Burnett, Angela Schulz, Mark J. Levis, Roland B. Walter, Carole Shaw, David Grimwade, Jordi Esteve, Christoph Röllig, Gerhard Ehninger, Christian Thiede, Elihu H. Estey, Petr Cetkovsky, Andrew M. Brunner, Anthony D. Ho, Robert Kerrin Hills, Ralitsa Langova, Nigel H. Russell, Zdeněk Ráčil, Michael Kramer, Jiri Mayer
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Male
Oncogene Proteins
Fusion
International Cooperation
medicine.medical_treatment
Abnormal Karyotype
Gastroenterology
Translocation
Genetic
chemistry.chemical_compound
0302 clinical medicine
hemic and lymphatic diseases
Antineoplastic Combined Chemotherapy Protocols
Remission Induction
Hematopoietic Stem Cell Transplantation
Neoplasms
Second Primary
Hematology
Middle Aged
Combined Modality Therapy
3. Good health
Leukemia
Myeloid
Acute
RUNX1
030220 oncology & carcinogenesis
Disease Progression
Female
Myeloid leukaemia
Abnormality
Chromosomes
Human
Pair 8
Adult
medicine.medical_specialty
Adolescent
MLH1
Disease-Free Survival
03 medical and health sciences
Internal medicine
Complex Karyotype
medicine
Humans
Survival rate
neoplasms
Aged
Chemotherapy
Whole Genome Sequencing
business.industry
Cytogenetics
Survival Analysis
Consolidation Chemotherapy
chemistry
Myelodysplastic Syndromes
Mutation
business
Chromosomes
Human
Pair 16
Follow-Up Studies
030215 immunology
ISSN: 0007-1048
Popis: In acute myeloid leukaemia (AML) t(8;16)(p11;p13)/MYST3-CREBBP is a very rare abnormality. Previous small series suggested poor outcome. We report on 59 patients with t(8;16) within an international, collaborative study. Median age was 52 (range: 16-75) years. AML was de novo in 58%, therapy-related (t-AML) in 37% and secondary after myelodysplastic syndrome (s-AML) in 5%. Cytogenetics revealed a complex karyotype in 43%. Besides MYST3-CREBBP, whole-genome sequencing on a subset of 10 patients revealed recurrent mutations in ASXL1, BRD3, FLT3, MLH1, POLG, TP53, SAMD4B (n = 3, each), EYS, KRTAP9-1 SPTBN5 (n = 4, each), RUNX1 and TET2 (n = 2, each). Complete remission after intensive chemotherapy was achieved in 84%. Median follow-up was 5·48 years; five-year survival rate was 17%. Patients with s-/t-AML (P = 0·01) and those with complex karyotype (P = 0·04) had an inferior prognosis. Allogeneic haematopoietic cell transplantation (allo-HCT) was performed in 21 (36%) patients, including 15 in first complete remission (CR1). Allo-HCT in CR1 significantly improved survival (P = 0·04); multivariable analysis revealed that allo-HCT in CR1 was effective in de novo AML but not in patients with s-AML/t-AML and less in patients exhibiting a complex karyotype. In summary, outcomes of patients with t(8;16) are dismal with chemotherapy, and may be substantially improved with allo-HCT performed in CR1.
Databáze: OpenAIRE
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