Acute megakaryoblastic leukaemia shows high frequency of chromosome 1q aberrations and dismal outcome.
Br J Haematol
; 202(6): 1165-1177, 2023 09.
Article
em En
| MEDLINE
| ID: mdl-37455345
ABSTRACT
Acute megakaryoblastic leukaemia (AMKL) is associated with poor prognosis. Limited information is available on its cytogenetics, molecular genetics and clinical outcome. We performed genetic analyses, evaluated prognostic factors and the value of allogeneic haematopoietic stem cell transplantation (allo-HSCT) in a homogenous adult AMKL patient cohort. We retrospectively analysed 38 adult patients with AMKL (median age 58 years, range 21-80). Most received intensive treatment in AML Cooperative Group (AMLCG) trials between 2001 and 2016. Cytogenetic data showed an accumulation of adverse risk markers according to ELN 2017 and an unexpected high frequency of structural aberrations on chromosome arm 1q (33%). Most frequently, mutations occurred in TET2 (23%), TP53 (23%), JAK2 (19%), PTPN11 (19%) and RUNX1 (15%). Complete remission rate in 33 patients receiving intensive chemotherapy was 33% and median overall survival (OS) was 33 weeks (95% CI 21-45). Patients undergoing allo-HSCT (n = 14) had a superior median OS (68 weeks; 95% CI 11-126) and relapse-free survival (RFS) of 27 weeks (95% CI 4-50), although cumulative incidence of relapse after allo-HSCT was high (62%). The prognosis of AMKL is determined by adverse genetic risk factors and therapy resistance. So far allo-HSCT is the only potentially curative treatment option in this dismal AML subgroup.
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Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Leucemia Mieloide Aguda
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Leucemia Megacarioblástica Aguda
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Transplante de Células-Tronco Hematopoéticas
Tipo de estudo:
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Adult
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Humans
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Middle aged
Idioma:
En
Revista:
Br J Haematol
Ano de publicação:
2023
Tipo de documento:
Article
País de afiliação:
Alemanha