Leucemia de células plasmáticas primaria: experiencia en un hospital público chileno

Background: Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM). Aim: To report the features of patients with pPCL. Material and Methods: Review of databases of the Hematology Department and the Hematology laboratory. Results: Of 178...

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Autores principales: Mendoza,María Gabriela, Valladares,Ximena, Roa,Macarena, Reyes,Margarita, Osorio,Rocío, Undurraga,María Soledad, Legües,María Eugenia, Cabrera,María Elena, Peña,Camila
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2019
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872019000100018
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spelling oai:scielo:S0034-988720190001000182019-04-03Leucemia de células plasmáticas primaria: experiencia en un hospital público chilenoMendoza,María GabrielaValladares,XimenaRoa,MacarenaReyes,MargaritaOsorio,RocíoUndurraga,María SoledadLegües,María EugeniaCabrera,María ElenaPeña,Camila Bortezamib Leukemia, Plasma Cell Paraproteinemias Background: Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM). Aim: To report the features of patients with pPCL. Material and Methods: Review of databases of the Hematology Department and the Hematology laboratory. Results: Of 178 patients with monoclonal gammopathies, five (2.8%) patients aged 33 to 64 years (three females) had a pPCL. The mean hemoglobin was 7.3 g/dL, the mean white blood cell count was 52,500/mm3, with 58% plasma cells, and the mean platelet count was 83,600/mm3. The mean bone marrow infiltration was 89%, LDH was 2,003 IU/L, serum calcium was 13 mg/dL, and creatinine 1.5 mg/dL. Two patients had bone lesions. Three were IgG, one IgA lambda and one lambda light chain. CD20 was positive in one, CD56 was negative in all and CD117 was negative in 3 cases. By conventional cytogenetic analysis, two had a complex karyotype. By Fluorescence in situ Hybridization, one was positive for TP53 and another for t (11; 14). One patient did not receive any treatment, three patients received VTD PACE and one CTD. None underwent transplant. Three patients are alive. The mean survival was 14 months. Conclusions: These patients with pPCL were younger and had a more aggressive clinical outcome than in multiple myeloma.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.147 n.1 20192019-01-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872019000100018es10.4067/S0034-98872019000100018
institution Scielo Chile
collection Scielo Chile
language Spanish / Castilian
topic Bortezamib
Leukemia, Plasma Cell
Paraproteinemias
spellingShingle Bortezamib
Leukemia, Plasma Cell
Paraproteinemias
Mendoza,María Gabriela
Valladares,Ximena
Roa,Macarena
Reyes,Margarita
Osorio,Rocío
Undurraga,María Soledad
Legües,María Eugenia
Cabrera,María Elena
Peña,Camila
Leucemia de células plasmáticas primaria: experiencia en un hospital público chileno
description Background: Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM). Aim: To report the features of patients with pPCL. Material and Methods: Review of databases of the Hematology Department and the Hematology laboratory. Results: Of 178 patients with monoclonal gammopathies, five (2.8%) patients aged 33 to 64 years (three females) had a pPCL. The mean hemoglobin was 7.3 g/dL, the mean white blood cell count was 52,500/mm3, with 58% plasma cells, and the mean platelet count was 83,600/mm3. The mean bone marrow infiltration was 89%, LDH was 2,003 IU/L, serum calcium was 13 mg/dL, and creatinine 1.5 mg/dL. Two patients had bone lesions. Three were IgG, one IgA lambda and one lambda light chain. CD20 was positive in one, CD56 was negative in all and CD117 was negative in 3 cases. By conventional cytogenetic analysis, two had a complex karyotype. By Fluorescence in situ Hybridization, one was positive for TP53 and another for t (11; 14). One patient did not receive any treatment, three patients received VTD PACE and one CTD. None underwent transplant. Three patients are alive. The mean survival was 14 months. Conclusions: These patients with pPCL were younger and had a more aggressive clinical outcome than in multiple myeloma.
author Mendoza,María Gabriela
Valladares,Ximena
Roa,Macarena
Reyes,Margarita
Osorio,Rocío
Undurraga,María Soledad
Legües,María Eugenia
Cabrera,María Elena
Peña,Camila
author_facet Mendoza,María Gabriela
Valladares,Ximena
Roa,Macarena
Reyes,Margarita
Osorio,Rocío
Undurraga,María Soledad
Legües,María Eugenia
Cabrera,María Elena
Peña,Camila
author_sort Mendoza,María Gabriela
title Leucemia de células plasmáticas primaria: experiencia en un hospital público chileno
title_short Leucemia de células plasmáticas primaria: experiencia en un hospital público chileno
title_full Leucemia de células plasmáticas primaria: experiencia en un hospital público chileno
title_fullStr Leucemia de células plasmáticas primaria: experiencia en un hospital público chileno
title_full_unstemmed Leucemia de células plasmáticas primaria: experiencia en un hospital público chileno
title_sort leucemia de células plasmáticas primaria: experiencia en un hospital público chileno
publisher Sociedad Médica de Santiago
publishDate 2019
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872019000100018
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