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2017
DOI: 10.1155/2017/4984951
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A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm Extensively Studied by Flow Cytometry and Immunohistochemistry

Abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with aggressive clinical course and poor prognosis. Diagnosis is based on detection of CD4+ CD56+, CD123high, TCL-1+, and blood dendritic cell antigen-2/CD303+ blasts, together with the absence of lineage specific antigens on tumour cells. In this report we present a case of BPDCN presenting with extramedullary and bone marrow involvement, extensively studied by flow cytometry and immunohistochemistry, who achieved complete r… Show more

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Cited by 2 publications
(2 citation statements)
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“…Although histopathology showing lymphoblasts or small immunoblots supports the presence of a neoplastic reaction, it requires IHC staining to verify the diagnosis and distinguish this malignancy from its differential diagnoses [ 17 , 18 ]. The differential diagnoses of BPDCN include mature T-cell lymphoma, myeloid sarcoma, acute myeloid leukemia, and T-cell lymphoblastic lymphoma [ 19 ].…”
Section: Discussionmentioning
confidence: 99%
“…Although histopathology showing lymphoblasts or small immunoblots supports the presence of a neoplastic reaction, it requires IHC staining to verify the diagnosis and distinguish this malignancy from its differential diagnoses [ 17 , 18 ]. The differential diagnoses of BPDCN include mature T-cell lymphoma, myeloid sarcoma, acute myeloid leukemia, and T-cell lymphoblastic lymphoma [ 19 ].…”
Section: Discussionmentioning
confidence: 99%
“…9 Chromosomal losses and deletions in 5q, 12p13, 13q21, 6q23-ter, and 9 have also been observed in BPDCN cells. 10,11 Inactivation of the tumour suppressors retinoblastoma-associated protein (RB1), tumour protein 53 (TP53) and cyclindependent kinase inhibitor 2A (CDKN2A), and activation of the oncogenes NRAS proto-oncogene, GTPase (NRAS), and KRAS proto-oncogene, GTPase (KRAS) has also been observed in many patients with BPDCN. 12 The primary therapy that most patients with BPDCN receive is an acute lymphocytic leukaemia (ALL)-based or acute myeloid leukaemia (AML)-based chemotherapy regimen, after which, patients tend to rapidly achieve complete remission.…”
Section: Discussionmentioning
confidence: 99%