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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 neoplasm (BPDCN) is an aggressive hematological malignancy with a poor prognosis that derives from plasmacytoid dendritic cells (PDC). 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 to those of acute myeloid leukemia and T-acute lymphoblastic leukemia, as well as the transcriptomic signature of primary PDC. This BPDCN gene signature identified a dysregulation of genes involved in cholesterol homeostasis, some of them being liver X receptor (LXR) target genes...
October 4, 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...
August 4, 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
Ming Sheng Lim, Karla Lemmert, Anoop Enjeti
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive haematological malignancy in the elderly, with a high frequency of cutaneous and bone marrow involvement and poor prognosis. We report a case of BPDCN with classic presentation and discuss its treatment and the value of different investigation tools used in diagnosis and response assessment.
January 20, 2016: BMJ Case Reports
Uday Deotare, Karen W L Yee, Lisa W Le, Anna Porwit, Anne Tierens, Rumina Musani, David Barth, Emina Torlakovic, Aaron Schimmer, Andre C Schuh, Matthew Seftel, Mark D Minden, Vikas Gupta, Elizabeth Hyjek
Few studies describe the comprehensive immunophenotypic pattern of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in the bone marrow and its treatment. This retrospective analysis evaluates the diagnostic flow cytometry (FCM) pattern and outcome of nine patients diagnosed with BPDCN. A four-tube 10-color FCM panel used for diagnosis of acute leukemia (AL), showed cells in the blast gate (CD45dim/low SSC) and were positive for CD4(bright), CD33(dim), CD56(heterogenous), CD123(bright), CD36, CD38, HLA-DR, CD71...
March 2016: American Journal of Hematology
Joana Ferreira, Maria Gabriela Gasparinho, Ricardo Fonseca
BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic tumor that was once believed to be derived from natural killer cells and is now recognized as originating from precursors of plasmacytoid dendritic cells. It generally involves the skin and has an aggressive clinical course. Due to its highly malignant behavior, a fast and accurate diagnosis of this condition is of the utmost importance. METHODS: Six cytology specimens from 5 patients diagnosed with BPDCN were reviewed as well as their clinical records...
March 2016: Cancer Cytopathology
Catherine M Nguyen, Lauren Stuart, Hadas Skupsky, Yun-Sun Lee, Arline Tsuchiya, David S Cassarino
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy primarily found in adults, often carrying a poor prognosis. There are only 33 reported pediatric cases of BPDCN in the literature. Although standard treatment is not yet established for children, current literature recommends the use of high-risk acute lymphoblastic leukemia (ALL)-type chemotherapy. Recent studies, however, have explored the benefits of combining chemotherapy with stem-cell transplantation. Here, the authors present 2 cases of pediatric BPDCN treated with different modalities...
December 2015: American Journal of Dermatopathology
Sumaira Qayoom, Garima Durga, Seena George, Khaliqur Rahman
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive neoplasm classified under "acute myeloid leukemia (AML) and related precursor neoplasm" by current WHO classification. Elderly male are commonly affected with cutaneous lesion being the hallmark of disease presentation. The disease progresses rapidly and sooner or later involves bone marrow and peripheral blood. Cases presenting primarily as leukemia without cutaneous involvement is a rarity with about 29 cases reported in literature till date...
July 2015: Indian Journal of Pathology & Microbiology
Naoya Ishibashi, Toshiya Maebayashi, Takuya Aizawa, Masakuni Sakaguchi, Osamu Abe, Katsuhiro Miura, Yoshihiro Hatta, Masahiko Sugitani
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare tumor that usually arises in the skin. Most patients develop skin lesions, which may be isolated and subsequently spread to affect the whole body. The prognosis is poor. Although BPDCN is usually treated by chemotherapy, radiation therapy is used in some cases (e.g., isolated lesions, elderly patients, or patients with comorbidities). The overall therapeutic efficacy and dose of radiation therapy remain unknown. We herein present a case of successful radiation treatment for BPDCN in a 77-year-old Japanese patient and describe the results of the first literature review on BPDCN of the skin initially treated with radiation therapy...
2015: International Journal of Clinical and Experimental Medicine
Elena Andrese, Laura Gheucă Solovăstru, G Dimofte, D Ferariu, V Porumb, D Vâţă, Luminita Smaranda Iancul
Blastic plasmacytoid dendritic cell neoplasm (BPDCN), CD4+/CD56+hematodermic neoplasm was formally known as blastic NK-cell lymphoma. It is in fact a form of acute myeloid leukemia notable for highly aggressive behavior with cutaneous, lymph node and bone marrow involvement. This entity is derived from plasmocytoid dendritic cells and has a predilection for extranodal sites, especially the skin. Elderly male patients are the most affected and the prognostic is poor. The first case was reported in 1994 and sice then, single cases and a few small series have been published...
April 2015: Revista Medico-chirurgicală̆ a Societă̆ţ̜ii de Medici ş̧i Naturaliş̧ti Din Iaş̧i
Naery Yang, Jungwon Huh, Wha Soon Chung, Min-Sun Cho, Kyung-Ha Ryu, Hae-Sun Chung
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy characterized by CD4 and CD56 coexpression without apparent lineage commitment. The molecular pathogenesis of BPDCN has been studied in only a limited number of cases, and specific chromosomal aberrations are lacking thus far. KMT2A (MLL) rearrangements are observed in various types of pediatric and adult leukemia, but only one adult case report has so far showed KMT2A (MLL)-MLLT1 gene rearrangements in BPDCN. We present the first pediatric case of BPDCN with a KMT2A (MLL)-MLLT1 rearrangement confirmed by molecular study...
September 2015: Cancer Genetics
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