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Blastic plasmacytoid dendritic cell neoplasm in a pediatric patient
Blastic plasmacytoid dendritic cell neoplasm in a pediatric patient
Vanessa Terán-Cerqueda 1 
, Luis E. Bernabé-Gaspar 1, 2 , Rubí E. Romo-Rodríguez 2 , M.Guadalupe Pérez-Dorantes 1 , Juan C. Solís-Poblano 1 , Patricia Zagoya-Martínez 1 , Andrea Huerta-Moreno 1 , Amara Sequeira-Colonnier 1 , Arturo García-Galicia 3 , Susana Barrera-Hernández 3 , M. José Borja-Núñez 4 , Jorge Loría-Castellanos 5
1 Servicio de Hematología, Unidad Médica de Alta Especialidad Hospital de Especialidades de Puebla, Centro Médico Nacional General de División Manuel Ávila Camacho, Instituto Mexicano del Seguro Social (IMSS), Puebla de Zaragoza, México; 2 Centro de Investigación Biomédica de Oriente, Órgano Operador de Administración Descentralizada Puebla, IMSS, Metepec, Puebla, México; 3 División de Investigación, Unidad Médica de Alta Especialidad Hospital de Especialidades de Puebla, Centro Médico Nacional General de División Manuel Ávila Camacho, IMSS, Puebla de Zaragoza, México; 4 Facultad de Medicina, Universidad Popular Autónoma del Estado de Puebla, Puebla. Pue., México; 5 Centro de Simulación, Universidad Anáhuac, Ciudad de México. México
*Correspondence: Luis E. Bernabé-Gaspar. Email: luisedu.bernabe@hotmail.com
Abstract
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an uncommon (0.44%), clinically aggressive hematolymphoid neoplasm. It arises from clonal plasmacytoid dendritic cells or their precursors. It often affects multiple anatomical compartments, including tissue, bone marrow, lymph nodes, and peripheral blood. We present the case of a previously healthy 7-year-old female patient with osteoarticular pain, persistent fever, weight loss, and right-sided exophthalmos. On admission, the laboratory findings revealed severe leukocytosis (271,600/μL) with 80% blasts in peripheral blood, anemia, and thrombocytopenia. Erythematous violaceous skin lesions appeared on the thorax, abdomen, and lower extremities. After 7 days, bone marrow flow cytometry found a blast population expressing CD4+, CD56+, CD123++, low CD45, and HLA-DR+, consistent with the diagnosis of BPDCN. During the induction, the patient experienced a torpid clinical course, resulting in death 19 days after diagnosis. We conclude that the pediatric BPDCN is an oncohematologic emergency that requires prompt and exact diagnosis as well as an integrated therapeutic approach guided by multiparametric diagnostic tools.
Keywords: Blastic plasmacytoid dendritic cell neoplasm. Hematodermic neoplasm. CD4+ -CD5.
