The Latin-American Experience in POEMS Syndrome: A Study of the GELAMM (Grupo de Estudio Latinoamericano de Mieloma Múltiple).

Autor:	Gallardo-Pérez MM; Centro de Hematología y Medicina Interna, Clínica Ruiz, Puebla, Mexico, mgallardo@hsctmexico.com., Negrete-Rodríguez P; Centro de Hematología y Medicina Interna, Clínica Ruiz, Puebla, Mexico.; Universidad de las Américas Puebla, Puebla, Mexico., Gertz MA; Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA., Peña C; Hospital del Salvador, Santiago, Chile., Riva E; Hematology Department, Hospital Británico & Hospital de Clinicas, Montevideo, Uruguay., Gilli V; Hospital San Martín, Paraná, Argentina., Rodríguez G; Hospital General Docente 'Camilo Cienfuegos', Sancti Spíritus, Cuba., Samánez C; Clinica Oncosalud-AUNA, Lima, Peru., Ferreira J; Hospital Base Osorno, Osorno, Chile., Portiño S; Clínica Universidad de Los Andes - Bradford Hill, Santiago de Chile, Chile., Montaña J; Hospital Ancud, Ancud, Chile., León P; Hospital van Buren Valparaiso, Valparaiso, Chile., Gutiérrez Y; Hospital de Temuco, Temuco, Chile., Del-Castanhel C; Instituto Nacional del Cancer, Santiago, Chile., Seehaus C; Hospital Italiano de Buenos Aires, Buenos Aires, Argentina., Funes ME; Sanatorio Britanico Rosario, Rosario, Argentina., Meneces-Bustillo R; Sanatorio Allende, Cordoba, Argentina., Duarte P; Centro de Educación Médica e Investigaciones Clínicas, Buenos Aires, Argentina., Shanley C; Hospital Británico Buenos Aires, Buenos Aires, Argentina., Elvira G; Hospital Central Mendoza, Mendoza, Argentina., Ochoa P; Instituto Alexander Fleming, Buenos Aires, Argentina., López-Vidal H; Clínica Dávila, Recoleta, Chile., Martinez-Cordero H; Hospital Militar Central, Bogotá, Colombia., Vasquez J; Instituto Nacional de Enfermedades Neoplásicas, Surquillo, Peru., von-Glasenapp A; Instituto de Prevision Social, Asuncion, Paraguay., Donoso J; Clínica Alemana - Sotero del Río, Puente Alto, Chile., Viñuela JL; Clínica Alemana - Sotero del Río, Puente Alto, Chile., Ruiz-Delgado GJ; Centro de Hematología y Medicina Interna, Clínica Ruiz, Puebla, Mexico.; Universidad Popular Autónoma del Estado de Puebla, Puebla, Mexico., Ruiz-Argüelles GJ; Centro de Hematología y Medicina Interna, Clínica Ruiz, Puebla, Mexico.; Universidad Popular Autónoma del Estado de Puebla, Puebla, Mexico.
Jazyk:	angličtina
Zdroj:	Acta haematologica [Acta Haematol] 2024 Aug 10, pp. 1-9. Date of Electronic Publication: 2024 Aug 10.
DOI:	10.1159/000540890
Abstrakt:	Introduction: POEMS syndrome is a rare paraneoplastic syndrome caused by an underlying plasma cell disorder. The acronym refers to the following features: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal paraproteinemia, and skin changes. Methods: The study was conducted at 24 hematological centers across 8 Latin-American countries. The study included a total of 46 patients {median age was 52 years (interquartile range [IQR]: 42-61.5), 30 males and 16 females} fulfilling the POEMS syndrome criteria diagnosed over a period of 12 years (January 1, 2011, through July 31, 2023). Epidemiological and clinical data were collected in an ad hoc database sent to the members of GELAMM, as well as the Kolmogorov-Smirnov test and Kaplan-Meier estimates. Results: All patients had polyneuropathy and monoclonal gammopathy; 89% had bone marrow plasma cell infiltration, 33% had sclerotic bone lesions. Only 10 patients underwent vascular endothelial growth factor (VEGF) testing in plasma samples. The paraproteinemia was IgG λ in 32% and IgA λ in 30%. 59% patients presented with cutaneous changes, mainly hyperpigmentation, 54% had organomegaly, and 74% endocrinopathy. The median interval from symptom onset to diagnosis was 7.7 months (IQR: 4.0-12.6). 69% of patients received a single line of treatment. The median follow-up period was 25 months (IQR: 9.37-52.0) and the 2-year overall survival rate was 100%. All patients who underwent transplantation (43%) are alive, with a median follow-up of 45.62 months (IQR: 15.46-70). Conclusion: This study investigates POEMS syndrome in Latin America and presents an initial overview of the disease in the region. VEGF usage is recommended for accurate diagnosis, but only 7 hematology centers in the region used it. Survival rate in Latin America is comparable with those observed internationally. (© 2024 S. Karger AG, Basel.)
Databáze:	MEDLINE
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