Blastic plasmacytoid dendritic cell neoplasia
Case Report
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Copyright (c) 2021 Veronica Maribel Hurtado Hurtado, Andrea del Rocío Noboa Cercado, Lisette Elianna Moran Mosquera
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
DOI:
https://doi.org/10.33821/572Keywords:
Células dendríticas, Sarcoma de Células Dendríticas Interdigitantes, Reporte de caso, Neoplasia de células dendríticas, Linfoma blástico, Linfoma cutáneo, Leucemia de células dendríticasAbstract
Introduction: blast plasmacytoid dendritic cell neoplasia (BPDCN) is an aggressive pathology and represents less than 1% of hematological neoplasms, it is characterized by violaceous nodular skin lesions without evidence of adenopathy in most cases. Observational studies show that the Hyper-CVAD chemotherapy protocol and consolidation with transplantation of hematopoietic progenitor cells have been associated with greater overall survival.
Clinical case: a 82-year-old woman with a history of type Diabetes Mellitus with five months of hyperpigmented, raised, indurated, non-painful violaceous lesions on the cheek, arms, anterior and posterior thorax and legs.
Evolution: Flow cytometry determined a phenotype compatible with pathological cells (5.86%) with CD123 ++, HLADR +++, NG2 ++, CD56 +++, CD4 ++, which suggests a BPDCN. The bone marrow biopsy showed infiltration. PET CT after corticosteroid therapy: there is no evidence of metabolically active macroscopic tumor disease. Dexamethasone treatment was started, with which skin lesions decreased by 80%. The CHOP-like Chemotherapy Protocol was started, she has received 6 cycles until October 2021, currently in complete remission.
Conclusion: In the present case, the clinical course of NCDPB was not aggressive until the moment of closure of the case, presenting a decrease of 80% of the lesions.
