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"titulo" => "[[Artículo traducido]]Neoplasia blástica de células dendríticas plasmocitoides: serie unicéntrica. Caracterización clínica, estudio mutacional y repuesta al tratamiento intensivo con trasplante alogénico de progenitores hematopoyéticos"
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"titulo" => "Abstract"
"resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematodermic neoplasm usually involving the skin. In this retrospective case series, 10 cases of BPDCN were identified, 90% of which had skin involvement and exhibited predominantly violaceous nodules and/or bruise-like plaques. Skin lesions showed diffuse or nodular dermal-based infiltrates of intermediate sized blasts with a grenz zone. Tumor immunophenotyping was CD4(+), CD56(+), CD123(+) and CD303(+). The most frequently mutated genes according to targeted next-generation sequencing were TET2 (3/7) and NRAS (2/7). Multiagent chemotherapy (CT) was administered as first-line therapy, and a total of 5 patients underwent allogenic hematopoietic stem cell transplantation (allo-HSCT). Better outcomes were observed in younger patients and those treated with acute lymphoblastic leukemia (ALL)-like CT followed by allo-HSCT. This study shows the clinical range of cutaneous lesions of BPDCN. Despite the absence of a gold standard therapy, patients treated with myeloablative intensive regimens and allo-HSCT seem to have a more favorable prognosis.</p></span>"
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"resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La neoplasia blástica de células dendríticas plasmocitoides (NBCDP), es una neoplasia hematodérmica poco frecuente y agresiva. En esta serie de casos retrospectiva, se identificaron 10 casos de NBCDP, con un 90 % de afectación de la piel, presentándose predominantemente como nódulos violáceos y/o placas hematoma-like. Las lesiones cutáneas mostraban infiltrados dérmicos difusos o nodulares de blastos de tamaño intermedio con zona de grenz. El inmunofenotipado fue CD4(+), CD56(+), CD123(+) y CD303(+). Los genes mutados más frecuentes fuerton TET2 (3/7) y NRAS (2/7). Se administró multi-quimioterapia (QT) como tratamiento de primera línea, y cinco pacientes se sometieron a trasplante alogénico de progenitores hematopoyéticas (alo-TPH). Se observaron mejores resultados en pacientes más jóvenes y aquellos tratados con QT similar a la leucemia linfoblástica aguda (LLA) seguida de alo-TPH. Este estudio muestra el rango clínico de las lesiones cutáneas de NBCDP. A pesar de no haber un <span class="elsevierStyleItalic">gold-standard</span> terapéutico, los regímenes de QT mieloablativos y alo-TPH parecen tener un pronóstico más favorable.</p></span>"
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