Primary plasma cell leukemia in Latin America: demographic, clinical, and prognostic characteristics. A study of GELAMM group

Peña C.; Riva E.; Schutz N.; Ramírez A.; Vásquez J.; del Carpio D.; Seehaus C.; Ochoa P.; Vengoa R.; Duarte P.; Martínez-Cordero H.; Figueredo Y.; Ríos R.O.; Ramírez J.; Bove V.; Roa M.; Russo M.; Espinoza M.; Rodriguez G.; Remaggi G.; Enciso M.E.; Chandía M.; Fantl D. · Leukemia and Lymphoma (2023) · DOI: 10.1080/10428194.2023.2171266

Resumen

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.