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Keiko Ono, Mikiko Ise, Dai Ikebe, Akiyasu Sato, Xiaofei Wang, Takeaki Sugawara, Hideki Tsujimura, Makiko Itami, Kyoya Kumagai
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematological malignancy derived from precursors of plasmacytoid dendritic cells. The majority of patients initially respond to multi-agent chemotherapy, though most relapse within a year and the prognosis is very poor. We report a 67-year-old man with erythema on the right chest and a nasopharyngeal mass. Histological examination revealed a mass of tumor cells expressing CD4, CD56, and CD123, but neither CD3 nor CD20. He was diagnosed with BPDCN...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Alexandar Tzankov, Konnie Hebeda, Markus Kremer, Roos Leguit, Attilio Orazi, Jon van der Walt, Umberto Gianelli
Two distinct forms of neoplasms derived from plasmacytoid dendritic cells (PDC) exist: mature PDC proliferations associated with myeloid neoplasms and blastic PDC neoplasms (BPDCN). Ten cases of PDC proliferations and neoplasms in the bone marrow have been submitted to the bone marrow workshop held at the 18th EAHP meeting. Based on observations from the submitted cases, scattered PDC (≤1% of cells) and PDC aggregates (≤10 PDC/HPF) reflect the normal bone marrow composition, while in myelodysplastic syndromes (MDS), there is a propensity for larger/more PDC aggregates (1-5% and 35 PDC/HPF)...
February 12, 2017: Annals of Hematology
Joan Montero, Jason Stephansky, Tianyu Cai, Gabriel K Griffin, Lucia Cabal-Hierro, Katsuhiro Togami, Leah J Hogdal, Ilene Galinsky, Elizabeth A Morgan, Jon C Aster, Matthew S Davids, Nicole R LeBoeuf, Richard M Stone, Marina Konopleva, Naveen Pemmaraju, Anthony Letai, Andrew A Lane
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive hematologic malignancy with dismal outcomes for which no standard therapy exists. We found that primary BPDCN cells were dependent on the antiapoptotic protein BCL2 and were uniformly sensitive to the BCL2 inhibitor venetoclax, as measured by direct cytotoxicity, apoptosis assays, and dynamic BH3 profiling. Animals bearing BPDCN patient-derived xenografts had disease responses and improved survival after venetoclax treatment in vivo Finally, we report on 2 patients with relapsed/refractory BPDCN who received venetoclax off-label and experienced significant disease responses...
February 2017: Cancer Discovery
Jill M Sullivan, David A Rizzieri
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare myeloid malignancy with no defined standard of care. BPDCN presents most commonly with skin lesions with or without extramedullary organ involvement before leukemic dissemination. As a result of its clinical ambiguity, differentiating BPDCN from benign skin lesions or those of acute myeloid leukemia with leukemia cutis is challenging. BPDCN is most easily defined by the phenotype CD4(+)CD56(+)CD123(+)lineage(-)MPO(-), although many patients will present with variable expression of CD4, CD56, or alternate plasmacytoid markers, which compounds the difficulty in differentiating BPDCN from other myeloid or lymphoid malignancies...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
(no author information available yet)
Blastic plasmacytoid dendritic cell neoplasms (BPDCN) depend on a TCF4/BRD4 transcriptional program.
January 2017: Cancer Discovery
Michele Ceribelli, Zhiying Esther Hou, Priscilla N Kelly, Da Wei Huang, George Wright, Karthik Ganapathi, Moses O Evbuomwan, Stefania Pittaluga, Arthur L Shaffer, Guido Marcucci, Stephen J Forman, Wenming Xiao, Rajarshi Guha, Xiaohu Zhang, Marc Ferrer, Laurence Chaperot, Joel Plumas, Elaine S Jaffe, Craig J Thomas, Boris Reizis, Louis M Staudt
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive and largely incurable hematologic malignancy originating from plasmacytoid dendritic cells (pDCs). Using RNAi screening, we identified the E-box transcription factor TCF4 as a master regulator of the BPDCN oncogenic program. TCF4 served as a faithful diagnostic marker of BPDCN, and its downregulation caused the loss of the BPDCN-specific gene expression program and apoptosis. High-throughput drug screening revealed that bromodomain and extra-terminal domain inhibitors (BETis) induced BPDCN apoptosis, which was attributable to disruption of a BPDCN-specific transcriptional network controlled by TCF4-dependent super-enhancers...
November 14, 2016: Cancer Cell
Maria Kleppe, Ross L Levine
In this issue of Cancer Cell, Ceribelli et al. use functional genomic and chemical screening to reveal the existence of a TCF4/BRD4-dependent oncogenic regulatory network in blastic plasmacytoid dendritic cell neoplasm (BPDCN) and demonstrate that BPDCN cells are highly sensitive to therapeutic targeting of this novel dependency.
November 14, 2016: Cancer Cell
Umberto Falcone, Hassan Sibai, Uday Deotare
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a clinically aggressive tumor derived from the precursors of plasmacytoid dendritic cells. It is a rare disease presenting across all ages with either skin or both skin and bone marrow involvement often conferring a poor prognosis. Though localized radiation has been used before, acute leukemia based regimens, remains the treatment of choice for induction of remission. Hematopoietic stem cell transplant, either autologous or allogeneic, is further required for attaining sustained remissions...
November 2016: Critical Reviews in Oncology/hematology
Ming Sheng Lim, Anoop K Enjeti, Karla Lemmert
No abstract text is available yet for this article.
February 2016: Pathology
Adam Ceroi, David Masson, Anne Roggy, Christophe Roumier, Cécile Chagué, Thierry Gauthier, Laure Philippe, Baptiste Lamarthée, Fanny Angelot-Delettre, Francis Bonnefoy, Sylvain Perruche, Sabeha Biichle, Claude Preudhomme, Elisabeth Macintyre, Laurent Lagrost, Francine Garnache-Ottou, Philippe Saas
Blastic plasmacytoid dendritic cell (PDC) neoplasm (BPDCN) is an aggressive hematological malignancy with a poor prognosis that derives from PDCs. No consensus for optimal treatment modalities is available today and the full characterization of this leukemia is still emerging. We identified here a BPDCN-specific transcriptomic profile when compared with those of acute myeloid leukemia and T-acute lymphoblastic leukemia, as well as the transcriptomic signature of primary PDCs. This BPDCN gene signature identified a dysregulation of genes involved in cholesterol homeostasis, some of them being liver X receptor (LXR) target genes...
December 8, 2016: Blood
Daniel Jeong, Jung W Choi, Katherine Jeong, Lubomir Sokol
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that is frequently misdiagnosed. We present a case of a 53-year-old man diagnosed with blastic plasmacytoid dendritic cell neoplasm with extensive computed tomography (CT) findings and provide an imaging focused review of this uncommon malignancy.
July 2016: Acta Radiologica Open
Naveen Pemmaraju, Vikas Gupta, Michael A Thompson, Andrew A Lane
The incorporation of Internet resources and the use of social media among patients, clinicians, advocates, and researchers in the field of hematology and oncology are growing in importance. Utilization of online information sharing is rising, especially among those involved in rare blood cancer fields, which have generally featured a paucity of reliable, updated information. In particular, blastic plasmacytoid dendritic cell neoplasm (BPDCN), an uncommon, but highly aggressive hematologic malignancy, is one example of a cancer with limited information readily available to the general public...
December 2016: Current Hematologic Malignancy Reports
Maiko Shimomura, Takaki Asano, Aya Furue, Mizuka Miki, Yasuhiko Sera, Hiroshi Kawaguchi, Kazuhiro Nakamura, Masao Kobayashi
Pediatric blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy that has an extremely poor prognosis despite the use of intensive chemotherapy. Recently, treatment of BPDCN with bone marrow transplantation (BMT) using myeloablative conditioning has been reported to increase survival in adults. We report a 9-year-old girl with cutaneous BPDCN who was successfully treated with combination chemotherapy followed by BMT using reduced intensity conditioning (RIC), without any adverse complications...
June 2016: Indian Journal of Hematology & Blood Transfusion
Yi-Wei Zhang, Ji-Hua Zhong, Xiao-Long Chen, Fei Xiao, Fang-Yuan Chen
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a clinically aggressive tumor, which frequently presents as cutaneous lesions and subsequently progresses to bone marrow (BM) involvement and leukemic dissemination. BPDCN is a rare entity that belongs in the same class as acute myeloid leukemia-associated precursor neoplasms, according to the 2008 World Health Organization classification. The present study reported the case of a 26-year-old female who presented with evident thrombocytopenia, leukocytosis and anemia, but without skin lesions...
July 2016: Experimental and Therapeutic Medicine
Livio Pagano, Caterina G Valentini, Sara Grammatico, Alessandro Pulsoni
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematological malignancy derived from the precursors of plamacytoid dendritic cells, with an aggressive clinical course and high frequency of cutaneous and bone marrow involvement. Neoplastic cells express CD4, CD43 (also termed SPN), CD45RA and CD56 (also termed NCAM1), as well as the plasmacytoid dendritic cell-associated antigens CD123 (also termed IL3RA), BDCA-2 (also termed CD303, CLEC4E) TCL1 and CTLA1 (also termed GZMB). The median survival is only a few months as the tumour exhibits a progressive course despite initial response to chemotherapy...
July 2016: British Journal of Haematology
Anouk Emadali, Neda Hoghoughi, Samuel Duley, Azadeh Hajmirza, Els Verhoeyen, Francois-Loic Cosset, Philippe Bertrand, Christophe Roumier, Anne Roggy, Céline Suchaud-Martin, Martine Chauvet, Sarah Bertrand, Sieme Hamaidia, Sophie Rousseaux, Véronique Josserand, Julie Charles, Isabelle Templier, Takahiro Maeda, Juliana Bruder-Costa, Laurence Chaperot, Joel Plumas, Marie-Christine Jacob, Thierry Bonnefoix, Sophie Park, Remy Gressin, Cornelis P Tensen, Cristina Mecucci, Elizabeth Macintyre, Dominique Leroux, Elisabeth Brambilla, Florence Nguyen-Khac, Isabelle Luquet, Dominique Penther, Christian Bastard, Fabrice Jardin, Christine Lefebvre, Francine Garnache, Mary B Callanan
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive leukemia for which knowledge on disease mechanisms and effective therapies are currently lacking. Only a handful of recurring genetic mutations have been identified and none is specific to BPDCN. In this study, through molecular cloning in an index case that presented a balanced t(3;5)(q21;q31) and molecular cytogenetic analyses in a further 46 cases, we identify monoallelic deletion of NR3C1 (5q31), encoding the glucocorticoid receptor (GCR), in 13 of 47 (28%) BPDCN patients...
June 16, 2016: Blood
Kamel Laribi, Nathalie Denizon, Anne Besançon, Jonathan Farhi, Pierre Lemaire, Jeremy Sandrini, Catherine Truong, Habib Ghnaya, Alix Baugier de Materre
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy with an aggressive clinical course. It is grouped with acute myeloid leukemia-related precursor neoplasms in the 2008 World Health Organization classification. Most patients with BPDCN have skin lesions at diagnosis and subsequent or simultaneous involvement of the bone marrow, peripheral blood, and lymph nodes. Patients usually respond to initial chemotherapy but often relapse. Stem cell transplantation may improve survival...
August 2016: Biology of Blood and Marrow Transplantation
Zhenya Tang, Guilin Tang, Sa A Wang, Xinyan Lu, Ken H Young, Carlos E Bueso-Ramos, Yesid Alvarado, L Jeffrey Medeiros, Joseph D Khoury
BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy. Based on literature reports of limited cases, over 50 % of BPDCN have chromosomal abnormalities, but no single chromosomal change has been identified as diagnostic of this entity. CASE PRESENTATION: In this report, we present a case of BPDCN with complicated chromosomal abnormalities involving chromosomes 12 and 22 and resulting in a simultaneous partial deletion of ETV6 and EWSR1...
2016: Molecular Cytogenetics
Lourdes Martín-Martín, Julia Almeida, Helena Pomares, Eva González-Barca, Pilar Bravo, Teresa Giménez, Cecilia Heras, José-Antonio Queizán, Elena Pérez-Ceballos, Violeta Martínez, Natalia Alonso, Carlota Calvo, Rodolfo Álvarez, María Dolores Caballero, Alberto Orfao
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive myeloid neoplasm which shows a high rate of central nervous system (CNS) recurrence and overall survival (OS) of <1 year. Despite this, screening for CNS involvement is not routinely performed at diagnosis and intrathecal (IT) prophylaxis is not regularly administered in BPDCN. Here, we prospectively evaluated 13 consecutive BPDCN patients for the presence of CNS involvement by flow cytometry. Despite none of the patients presented with neurological symptoms, occult CNS involvement was detected in 6/10 cases evaluated at diagnosis and 3/3 studied at relapse/progression...
March 1, 2016: Oncotarget
Ryan C Johnson, Jinah Kim, Yasodha Natkunam, Uma Sundram, Aharon G Freud, Bryan Gammon, Michael J Cascio
Myeloid neoplasms constitute one of the most common malignancies in adults. In most cases these proliferations initially manifest in the blood and marrow; however, extramedullary involvement may precede blood or marrow involvement in a subset of cases, making a definitive diagnosis challenging by morphologic and immunohistochemical assessment alone. Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive entity that frequently presents in extramedullary sites and can show morphologic and immunophenotypic overlap with myeloid neoplasms...
April 2016: American Journal of Surgical Pathology
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