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Leuk Lymphoma ; 64(4): 816-821, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36695519

RESUMO

Primary plasma cell leukemia (pPCL) is an infrequent and aggressive plasma cell disorder. The prognosis is still very poor, and the optimal treatment remains to be established. A retrospective, multicentric, international observational study was performed. Patients from 9 countries of Latin America (LATAM) with a diagnosis of pPCL between 2012 and 2020 were included. 72 patients were included. Treatment was based on thalidomide in 15%, proteasome inhibitors (PI)-based triplets in 38% and chemotherapy plus IMIDs and/or PI in 29%. The mortality rate at 3 months was 30%. The median overall survival (OS) was 18 months. In the multivariate analysis, frontline PI-based triplets, chemotherapy plus IMIDs and/or PI therapy, and maintenance were independent factors of better OS. In conclusion, the OS of pPCL is still poor in LATAM, with high early mortality. PI triplets, chemotherapy plus IMIDs, and/or PI and maintenance therapy were associated with improved survival.


Assuntos
Leucemia Plasmocitária , Humanos , Leucemia Plasmocitária/diagnóstico , Leucemia Plasmocitária/epidemiologia , Leucemia Plasmocitária/terapia , Prognóstico , Bortezomib/uso terapêutico , Estudos Retrospectivos , Resultado do Tratamento , América Latina/epidemiologia , Agentes de Imunomodulação , Demografia
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