In:
British Journal of Haematology, Wiley, Vol. 192, No. 5 ( 2021-03), p. 832-842
Abstract:
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.
Type of Medium:
Online Resource
ISSN:
0007-1048
,
1365-2141
Language:
English
Publisher:
Wiley
Publication Date:
2021
detail.hit.zdb_id:
1475751-5
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