Neoplasia blástica de células dendríticas plasmocitoides

Abstract

We report a case of a 26-year-old female with no significant past medical history, presented with nodular and squamous skin lesion in chest, later generalized with skin plaques in face and upper extremities accompanied by B symptoms, work-up with skin biopsy was made, in which poorly differentiated cells with nuclei of blastic aspect were reported, intensive staining for CD45, CD56, TdT and slightly for CD4, CD123, the bone marrow biopsy demonstrated 80% of blast cells; Based on this findings, the patient was diagnosed with a hematodermic neoplasm called plasmacytoid dendritic cell tumor, the patient was treated with HyperCVAD regimen, with suitable clinical answer. One is considered a rare entity, recently described, with little experience in its treatment, there has been a constant evolution in the disease's nomenclature because of its histological origin with tumorlike cells belong at the moment to the lineage of the dendritic cells, classifying itself for the WHO within acute leukemias. Histopathological examination with appropriate stain is the main issue for the diagnosis. The aggressiveness of this neoplasia and its treatment with schemes used in acute leukemias, because there is no defined treatment strategy, confers a poor prognosis with overall survival of approximately 17 months. We presented this case like experience of clinical, pathological, molecular behavior, as well as of the used treatment.