BLASTIC PLASMACYTOID DENDRITIC CELL NEOPLASM WITH COMPLETE CLINICAL REMISSION WITH CHEMOTHERAPY AND CENTRAL NERVOUS SYSTEM RELAPSE. REPORT OF ONE CASE
Neoplasia de células dendríticas plasmocitoides blásticas con respuesta inicial a quimioterapia y recaída en sistema nervioso central.
Author
Contreras, Loreto; Facultad de Medicina, Universidad de Concepción
Mercado, Loreto; Hospital Regional de Concepción "Dr. Guillermo Grant Benavente"
Delgado, Carolina; Hospital Regional de Concepción "Dr. Guillermo Grant Benavente"
Cabezas, Claudia; Hospital Regional de Concepción "Dr. Guillermo Grant Benavente"
Starke, Laksmi; Hospital Regional de Concepción "Dr. Guillermo Grant Benavente"
Romero, Mónica; Hospital Regional de Concepción "Dr. Guillermo Grant Benavente"
Ibieta, Fernando; Hospital Regional de Concepción "Dr. Guillermo Grant Benavente"
Henríquez, Mauricio; Hospital Regional de Concepción "Dr. Guillermo Grant Benavente"
Chandía, Mauricio; Hospital Regional de Concepción "Dr. Guillermo Grant Benavente"
Abstract
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination. The demonstration of tumor cells with the characteristic immunophenotype with expression of CD56, generally CD4 and dendritic cell antigens (CD123, cyTCL-1, HLA-DR), in the absence of myeloid or lymphoid lineage markers is required for the diagnosis. Responses to chemotherapy are initially satisfactory, with frequent systemic and central nervous system relapses. We report a 24 years old male with BPDCN, initially diagnosed and treated as non-Hodgkin CD4+ T-cell lymphoma, with initial complete remission who evolved with early central nervous system relapse. A second attempt of chemotherapy failed and the patient died two months later. Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination. The demonstration of tumor cells with the characteristic immunophenotype with expression of CD56, generally CD4 and dendritic cell antigens (CD123, cyTCL-1, HLA-DR), in the absence of myeloid or lymphoid lineage markers is required for the diagnosis. Responses to chemotherapy are initially satisfactory, with frequent systemic and central nervous system relapses. We report a 24 years old male with BPDCN, initially diagnosed and treated as non-Hodgkin CD4+ T-cell lymphoma, with initial complete remission who evolved with early central nervous system relapse. A second attempt of chemotherapy failed and the patient died two months later.
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