Localized skin-limited blastic plasmacytoid dendritic cell neoplasm: A subset with possible durable remission without transplantation

Amitay‐Laish, Iris; Sundram, Uma; Hoppe, Richard T.; Hodak, Emmilia; Medeiros, Bruno C.; Kim, Youn H.

doi:10.1016/j.jdcr.2017.03.015

Cited by 16 publications

(8 citation statements)

References 12 publications

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“…El Protocolo Hyper-CVAD produce una remisión completa (RC) en 90% de pacientes con una mediana de SG de 29 meses [20], otro estudio informó RC del 83% y una mediana de SG de 24.3 meses [21]. En reporte de casos aislados dos pacientes recibieron Hiper-CVAD más radioterapia sobre lesiones cutáneas con RC hasta 9 años [22]. En pacientes frágiles se puede considerar la monoterapia con azatidina con respuesta moderada [23].…”

Section: Discussionunclassified

Neoplasia de células dendríticas plasmocitoides blásticas

2021

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Introducción: La neoplasia de células dendríticas plasmocitoides blásticas (NCDPB) es una patología agresiva y representa menos del 1% de neoplasias hematológicas, se caracteriza por lesiones cutáneas nodulares violáceas sin evidencia de adenopatías en la mayoría de casos. Estudios observacionales demuestran que el Protocolo de quimioterapia Hyper-CVAD y la consolidación con trasplante de células progenitoras hematopoyéticas se han asociado con una mayor supervivencia general. Caso Clínico: mujer de 82 años con antecedentes de Diabetes Mellitus tipo con cinco meses de lesiones hiperpigmentadas, elevadas, induradas, violáceas no dolorosas en mejilla, brazos, tórax anterior y posterior y piernas. Evolución: En citometría de flujo se determinó un fenotipo compatible con células patológicas (5.86%) con CD123++, HLADR+++, NG2++, CD56+++, CD4++, que sugiere una NCDPB. La biopsia de médula ósea presentó infiltración. PET CT posterior a terapia corticoide: no evidencia enfermedad tumoral macroscópica metabólicamente activa. Se inicia tratamiento con Dexametasona, con lo que las lesiones cutáneas disminuyeron en un 80%. Se inició Quimioterapia Protocolo CHOP like, ha recibido 6 ciclos hasta octubre del 2021, actualmente en remisión completa. Conclusión: En el presente caso el curso clínico de la NCDPB no fue agresivo hasta el momento del cierre del caso presentando disminución del 80% de las lesiones.

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Section: Discussionunclassified

Neoplasia de células dendríticas plasmocitoides blásticas

2021

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Section: Discussionmentioning

confidence: 99%

“…14 In a recent case report, two patients with skin located BPDCN who received combination treatment of an ALL-like (hyper-CVAD [cyclophosphamide, vincristine, doxorubicin, dexamethasone], A1B) chemotherapy protocol and consolidative localized radiotherapy without stem cell transplantation or other therapy, were reported to have achieved complete remission that was sustained for up to 9 years. 15 To extend the overall survival of patients with BPDCN, many have been given hematopoietic stem cell transplantation after the first complete remission. In a retrospective study of patients with BPDCN, treatment with allo-haematopoietic cell transplantation or auto-haematopoietic cell transplantation during the first complete remission was shown to significantly prolong progress free survival and overall survival times.…”

Section: Discussionmentioning

confidence: 99%

A new palliative treatment for blastic plasmacytoid dendritic cell neoplasm: a case report and review of the literature

Yang

Nie

et al. 2019

J Int Med Res

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematological malignancy that derives from plasmacytoid dendritic cells. The cancer is characterized by aggressive development and dismal prognosis, and due to limited prospective study, there is currently no established standard treatment. In this study, the case of a 77-year old female patient with BPDCN, who presented with a cutaneous lesion on the right of her face, is described. The lesion developed into a serious ulcer due to rapid disease progress, thus, a surgical excision was performed. As the patient refused to receive radiotherapy or haematopoietic stem cell transplantation following surgery, a new palliative combination chemotherapy was administered, comprising gemcitabine, nedaplatin and bleomycin. The therapy gave satisfactory results in terms of short-term treatment response and was well-tolerated. Published literature regarding BPDCN is also reviewed.

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“…Since the most recently revised nomenclature by consensus in 2008 by the World Health Organization (WHO), 21 cases of BPDCN have been reported in the literature. Clinically, most cases of BPDCN have been described as violaceous, bruise‐like, infiltrated plaques or tumors . Herein, we describe BPDCN clinically simulating a melanocytic nevus and with centrocyte‐like histomorphology mimicking follicle center lymphoma (FCL).…”

Section: Introductionmentioning

confidence: 96%