Neoplasia de Células Dendríticas Plasmocitoides blásticas: reporte del primer caso en Ecuador

Abstract

Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare, aggressive, difficult-to-diagnose haematological neoplasm with high mortality. The first case in Ecuador of a young patient with no relevant pathological history, admitted to the internal medicine service of the Enrique Garcés Hospital for presenting cutaneous macula, arthralgias and myalgias, which is complicated with pleural effusion type exudative and poor respiratory mechanics. Extension tests revealed: Acute myeloid leukemia type M2, which is why he was referred to a reference cancer center to complete study and management. Cytogenetic and phenotypic studies corroborated the diagnosis of BPDCN, Treatment with Hyper-CVAD protocol was instituted, however, the patient presented respiratory, renal and haematological commitment that progresses to refractory and death shock. The aggressive nature of this rare leukemia is a limitation in time to institute targeted treatment, most often determining high mortality.