Treatment of blastic plasmacytoid dendritic cell neoplasm.
Hematology Am Soc Hematol Educ Program
; 2016(1): 16-23, 2016 Dec 02.
Article
en En
| MEDLINE
| ID: mdl-27913457
ABSTRACT
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare myeloid malignancy with no defined standard of care. BPDCN presents most commonly with skin lesions with or without extramedullary organ involvement before leukemic dissemination. As a result of its clinical ambiguity, differentiating BPDCN from benign skin lesions or those of acute myeloid leukemia with leukemia cutis is challenging. BPDCN is most easily defined by the phenotype CD4+CD56+CD123+lineage-MPO-, although many patients will present with variable expression of CD4, CD56, or alternate plasmacytoid markers, which compounds the difficulty in differentiating BPDCN from other myeloid or lymphoid malignancies. Chromosomal aberrations are frequent, and the mutational landscape of BPDCN is being rapidly characterized although no obvious molecular target for chemoimmunotherapy has been identified. Chemotherapy regimens developed for acute myeloid leukemia, acute lymphoid leukemia, and myelodysplastic syndrome have all been used to treat BPDCN. Relapse is frequent, and overall survival is quite poor. Allogeneic transplantation offers a chance at prolonged remission and possible cure for those who are eligible; unfortunately, relapse remains high ranging from 30% to 40%. Novel therapies such as SL-401, a diphtheria toxin conjugated to interleukin-3 (IL-3) is commonly overexpressed in BPDCN and other aggressive myeloid malignancies and has shown considerable promise in ongoing clinical trials. Future work with SL-401 will define its place in treating relapsed or refractory disease as well as its role as a first-line therapy or bridge to transplantation.
Texto completo:
1
Colección:
01-internacional
Asunto principal:
Células Dendríticas
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Proteínas Recombinantes de Fusión
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Biomarcadores de Tumor
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Leucemia Mieloide
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Antígenos CD
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Infiltración Leucémica
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Trasplante de Células Madre
Tipo de estudio:
Prognostic_studies
Límite:
Humans
Idioma:
En
Revista:
Hematology Am Soc Hematol Educ Program
Asunto de la revista:
HEMATOLOGIA
Año:
2016
Tipo del documento:
Article
País de afiliación:
Nueva Caledonia