Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos

Blastic plasmacytoid dendritic cell neoplasm is a rare hematological malignancy derived from immature plasmacytoid dendritic cells. The tumor cells have an immature blastic appearance, and diagnosis is based on the expression of CD4, CD56 y CD123 in the absence of other lymphoid, natural killer, or...

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Autores principales:	Avilés-Salas,Alejandro, Candelaria,Myrna, Ponce,Mayra, Cañavera-Constantino,Abraham, Burgos,Sebastián
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2017
Materias:	Dentritic Cells Hematologic Neoplasms Killer Cells Natural
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872017000901208
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spelling	oai:scielo:S0034-988720170009012082017-12-07Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicosAvilés-Salas,AlejandroCandelaria,MyrnaPonce,MayraCañavera-Constantino,AbrahamBurgos,Sebastián Dentritic Cells Hematologic Neoplasms Killer Cells Natural Blastic plasmacytoid dendritic cell neoplasm is a rare hematological malignancy derived from immature plasmacytoid dendritic cells. The tumor cells have an immature blastic appearance, and diagnosis is based on the expression of CD4, CD56 y CD123 in the absence of other lymphoid, natural killer, or myeloid antigens. The majority of affected individuals are older people with a mean age of 66 years. Male to female ratio is approximately 3:1. Common presentation includes cutaneous lesions followed by tumor dissemination. Treatment with conventional chemotherapy is ineffective and allogeneic hematopoietic stem cell transplantation is required to achieve remission. We report three male patients, aged 23, 27 and 51 years with the disease. All had multiple, infiltrated pink plaques and nodules on the skin of their face, neck and thorax, measuring 1 to 12 cm in diameter. All tumors were histologically characterized by a monotonous proliferation of medium size cells with blastic features. Tumor cells were positive for CD123, CD56, CD4 and CD7 in all cases. After a mean of follow-up of 14.6 months, one patient died of the disease, one patient is alive and the disease relapsed after 17 months of remission and one patient is alive with no evidence of the disease.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.145 n.9 20172017-09-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872017000901208es10.4067/s0034-98872017000901208
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Dentritic Cells Hematologic Neoplasms Killer Cells Natural
spellingShingle	Dentritic Cells Hematologic Neoplasms Killer Cells Natural Avilés-Salas,Alejandro Candelaria,Myrna Ponce,Mayra Cañavera-Constantino,Abraham Burgos,Sebastián Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos
description	Blastic plasmacytoid dendritic cell neoplasm is a rare hematological malignancy derived from immature plasmacytoid dendritic cells. The tumor cells have an immature blastic appearance, and diagnosis is based on the expression of CD4, CD56 y CD123 in the absence of other lymphoid, natural killer, or myeloid antigens. The majority of affected individuals are older people with a mean age of 66 years. Male to female ratio is approximately 3:1. Common presentation includes cutaneous lesions followed by tumor dissemination. Treatment with conventional chemotherapy is ineffective and allogeneic hematopoietic stem cell transplantation is required to achieve remission. We report three male patients, aged 23, 27 and 51 years with the disease. All had multiple, infiltrated pink plaques and nodules on the skin of their face, neck and thorax, measuring 1 to 12 cm in diameter. All tumors were histologically characterized by a monotonous proliferation of medium size cells with blastic features. Tumor cells were positive for CD123, CD56, CD4 and CD7 in all cases. After a mean of follow-up of 14.6 months, one patient died of the disease, one patient is alive and the disease relapsed after 17 months of remission and one patient is alive with no evidence of the disease.
author	Avilés-Salas,Alejandro Candelaria,Myrna Ponce,Mayra Cañavera-Constantino,Abraham Burgos,Sebastián
author_facet	Avilés-Salas,Alejandro Candelaria,Myrna Ponce,Mayra Cañavera-Constantino,Abraham Burgos,Sebastián
author_sort	Avilés-Salas,Alejandro
title	Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos
title_short	Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos
title_full	Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos
title_fullStr	Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos
title_full_unstemmed	Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos
title_sort	neoplasia blástica de células dendríticas plasmocitoides. casos clínicos
publisher	Sociedad Médica de Santiago
publishDate	2017
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872017000901208
work_keys_str_mv	AT avilessalasalejandro neoplasiablasticadecelulasdendriticasplasmocitoidescasosclinicos AT candelariamyrna neoplasiablasticadecelulasdendriticasplasmocitoidescasosclinicos AT poncemayra neoplasiablasticadecelulasdendriticasplasmocitoidescasosclinicos AT canaveraconstantinoabraham neoplasiablasticadecelulasdendriticasplasmocitoidescasosclinicos AT burgossebastian neoplasiablasticadecelulasdendriticasplasmocitoidescasosclinicos
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Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos

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