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https://www.readbyqxmd.com/read/29064025/analysis-of-first-year-twitter-metrics-of-a-rare-disease-community-for-blastic-plasmacytoid-dendritic-cell-neoplasm-bpdcn-on-social-media-bpdcn
#1
REVIEW
Naveen Pemmaraju, Audun Utengen, Vikas Gupta, Michael A Thompson, Andrew A Lane
PURPOSE OF REVIEW: The use of Twitter, one of the most commonly engaged social media platforms in the world, is increasing among the general public. Notably, this trend has also been observed among those involved in the healthcare field. With its ability to readily connect diverse groups of stakeholders in a given area of interest, Twitter has become a focal point for those involved in increasing awareness and information exchange in orphan disease fields. Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematologic malignancy with generally poor long-term outcomes for adult patients and no standard therapeutic guidelines...
October 24, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/29064022/novel-pathways-and-potential-therapeutic-strategies-for-blastic-plasmacytoid-dendritic-cell-neoplasm-bpdcn-cd123-and-beyond
#2
REVIEW
Naveen Pemmaraju
No abstract text is available yet for this article.
October 24, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28980314/haematopoietic-cell-transplantation-for-blastic-plasmacytoid-dendritic-cell-neoplasm-a-north-american-multicentre-collaborative-study
#3
Mohamed A Kharfan-Dabaja, Monzr M Al Malki, Uday Deotare, Renju V Raj, Najla El-Jurdi, Navneet Majhail, Mohamad A Cherry, Qaiser Bashir, Justin Darrah, Taiga Nishihori, Hassan Sibai, Mehdi Hamadani, Marcos de Lima, Aaron T Gerds, George Selby, Muzaffar H Qazilbash, Stephen J Forman, Ernesto Ayala, Jeffrey H Lipton, Parameswaran N Hari, Tariq Muzzafar, Ling Zhang, Horatiu Olteanu, Janelle Perkins, Lubomir Sokol, Ambuj Kumar, Sairah Ahmed
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is incurable with conventional therapies. Limited retrospective data have shown durable remissions after haematopoietic cell transplantation (HCT) [allogeneic (allo) or autologous (auto)]. We conducted a multicentre retrospective study in BPDCN patients treated with allo-HCT and auto-HCT at 8 centres in the United States and Canada. Primary endpoint was overall survival (OS). The population consisted of 45 consecutive patients who received an allo-HCT (n = 37) or an auto-HCT (n = 8) regardless of age, pre-transplant therapies, or remission status at transplantation...
October 4, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28966462/blastic-plasmacytoid-dendritic-cell-neoplasm-following-acquired-erythropoietic-protoporphyria
#4
John R Krause, Laura Baugh, Alicia Swink, Micah Burch
A 56-year-old Texas rancher with a prior diagnosis of acquired erythropoietic protoporphyria secondary to an underlying myelodysplastic disorder developed an uncommon tumor, blastic plasmacytoid dendritic cell neoplasm (BPDCN). During his initial disease, analysis revealed a TET2 mutation, which is the most common mutation associated with BPDCN. This article discusses this unusual hematopoietic neoplasm, the possible evolution from erythropoietic protoporphyria, and the underlying myelodysplastic process.
October 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28906324/pediatric-blastic-plasmacytoid-dendritic-cell-neoplasm-a-systematic-literature-review
#5
REVIEW
Marie Jeong-Min Kim, Ahmed Nasr, Bilaal Kabir, Joseph de Nanassy, Ken Tang, Danielle Menzies-Toman, Donna Johnston, Dina El Demellawy
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy characterized by frequent skin involvement that most commonly affects older patients. BPDCN is known to have a poor prognosis. Our objective was to assess if outcome and disease prognosis were independently influenced by age when evaluated with clinical presentation, sex, and treatment regimens. We conducted a systematic review to identify BPDCN cases, to compare pediatric BPDCN cases with adult cases. A total of 125 publications were identified detailing 356 cases...
October 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28870016/clinical-features-and-treatment-outcomes-of-blastic-plasmacytoid-dendritic-cell-neoplasm-a-single-center-experience-in-korea
#6
Hae Su Kim, Hee-Jin Kim, Sun-Hee Kim, Joon Young Choi, Young Hyeh Ko, Won Seog Kim, Chul Won Jung, Seok Jin Kim
BACKGROUND/AIMS: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that typically presents in the form of skin manifestations with or without lymph node and bone marrow involvement. Given its rarity and recent recognition as a distinct pathological entity, no standard of treatment exists for this aggressive disease and its prognosis is particularly dismal. METHODS: We retrospectively analyzed clinical features and treatment outcomes of patients who were diagnosed with BPDCN between 2000 and 2014...
September 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28859047/childhood-blastic-plasmacytoid-dendritic-cell-neoplasm-mimicking-acute-rheumatic-fever-report-of-an-unusual-clinical-presentation-and-review-of-literature
#7
Banashree Devi Rajkumari, Vinay Munikoty, Sreejesh Sreedharanunni, Richa Jain, Man Updesh Singh Sachdeva, Neelam Varma
BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is very rarely diagnosed in children with less than 50 cases in the literature. OBSERVATION: We report a case of childhood BPDCN who mimicked acute rheumatic fever at presentation. Majority of the reported childhood BPDCN received acute lymphoblastic leukemia-like chemotherapy with/without stem cell therapy, whereas those who received acute myeloid leukemia-like therapy predominantly succumbed to disease or sepsis...
August 30, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28856005/blastic-plasmacytoid-dendritic-cell-neoplasm-in-children-a-review-of-two-cases
#8
Wenjun Deng, Minghua Yang, Feimei Kuang, Yingting Liu, Hui Zhang, Lizhi Cao, Min Xie, Liangchun Yang
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a newly characterized, rare malignant tumor of the skin and hematopoietic system. BPDCN occurs mainly in the elderly, whereas it is rarer among children, and has variable clinical manifestations. Optimal chemotherapeutic regimens for the treatment of BPDCN have not yet been determined and this tumor has a poor prognosis. In this study, two pediatric cases of BPDCN, including a 7-year-old female and a 9-year-old male patient, diagnosed at the Xiangya Hospital of Central South University over the past 2 years, were retrospectively reviewed...
October 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28795410/clinicopathological-analysis-of-46-cases-with-cd4-and-or-cd56-immature-haematolymphoid-malignancy-reappraisal-of-blastic-plasmacytoid-dendritic-cell-and-related-neoplasms
#9
Yuka Suzuki, Seiichi Kato, Kei Kohno, Akira Satou, Ahmed E Eladl, Naoko Asano, Michihiro Kono, Yuichi Kato, Masafumi Taniwaki, Masashi Akiyama, Shigeo Nakamura
AIMS: In this study, we aimed to investigate the clinicopathological features of CD4(+) and/or CD56(+) immature haematolymphoid malignancy (iHLM), including blastic plasmacytoid dendritic cell neoplasm (BPDCN). METHODS AND RESULTS: We analysed the clinicopathological features of 46 patients diagnosed consecutively with CD4(+) /CD56(+) iHLM. These cases were categorised into three groups based on their immunohistochemical expression of three plasmacytoid dendritic cell (pDC) markers [CD123, CD303 and T cell leukaemia/lymphoma (TCL1)]: cutaneous BPDCN (n = 35), non-cutaneous BPDCN (n = 6) and non-BPDCN-type CD56(+) neoplasms (n = 5)...
August 10, 2017: Histopathology
https://www.readbyqxmd.com/read/28656936/blastic-plasmacytoid-dendritic-cell-leukemia-in-a-black-malian
#10
Y L Diallo, J Cucherousset, O Samassekou, M Doumbere, Y Cissoko, D T Menta, N Ouologuem, M Ouologuem, M Konate, A T Sidibe
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy, that most commonly manifests as cutaneous lesions. A 19-year-old Malian female was admitted to the Unit of Medicine of Hopital du Mali with anemia, fever, weakness, and weight loss. On physical examination she was wasted, pale, febrile (37.4°C), and had inguinal and axillary lymphadenopathies. The complete blood count found pancytopenia with Hemoglobin level of 4.8 g/dL, Leucocytes count of 1900/μL (neutrophil: 300/μL), and platelets count of 56 000/μL...
June 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/28635683/blastic-plasmacytoid-dendritic-cell-neoplasm-with-pulmonary-involvement-and-atypical-skin-lesion
#11
Cyndi Myrelle da Silva Barros Romão, Cláudio José Dos Santos Júnior, Luiz Arthur Calheiros Leite, Maria Jordana Rocha Gomes Alves, Nathalia Silva Araújo, Anderson F L Castro, Muriel Silva Moura, Vitória Mikaelly da Silva Gomes, Arthur Moacir Costa Sampaio Batinga, João Antonio S Queiroz, Natanael Barbosa Dos Santos
BACKGROUND Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematodermic malignancy neoplasm with highly aggressive course and poor prognosis. This disease typically presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and peripheral blood.  CASE REPORT Here, we report the case of a 51-year-old woman who presented a violaceus skin lesion on the lateral region of the right thigh, weight loss, fever, and lymphadenopathies. Computed tomography (CT) displayed thoracic and abdominal lymph node and alveolar bleeding...
June 21, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28620927/bendamustine-for-relapsed-blastic-plasmacytoid-dendritic-cell-leukaemia
#12
Sarah Bétrian, Sarah Guenounou, Isabelle Luquet, Cécile Demur, Anne Huynh, Loïc Ysebaert, Christian Recher, Françoise Huguet
Optimal treatment of blastic plasmacytoid dendritic cell neoplasm (BPDCN), a rare entity of dismal prognosis previously described as CD4+/CD56+ hematodermic malignancies, is not defined. We report five cases of relapsed BPDCN treated with bendamustine hydrochloride, a well-tolerated bifunctional drug acting as an alkylating and antimetabolite agent. All patients were above the age of 50 years and in advanced disease (early first relapse in two, subsequent relapse in three; multi-organ involvement in four; previous intensive chemotherapy in five; and stem cell transplantation in four)...
June 2017: Hematological Oncology
https://www.readbyqxmd.com/read/28419186/cd4-negative-variant-of-cutaneous-blastic-plasmacytoid-dendritic-cell-neoplasm-with-a-novel-pbrm1-mutation-in-an-11-year-old-girl
#13
Nuri Yigit, Luisa Fernanda Suarez, Lisa Giulino Roth, Attilio Orazi, Wayne Tam
Objectives: We report a rare case of CD4- cutaneous blastic plasmacytoid dendritic cell neoplasm (BPDCN) with a novel PBRM1 mutation. Methods: An 11-year-old girl presented with an enlarged mass on her left arm and underwent an incisional biopsy. Results: Histopathologic examination and immunohistochemistry studies showed a monotonous proliferation of blasts that were CD4-, CD56+, and CD123+. There was no evidence of leukemic dissemination...
May 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28409040/a-case-of-blastic-plasmacytoid-dendritic-cell-neoplasm-extensively-studied-by-flow-cytometry-and-immunohistochemistry
#14
Martina Pennisi, Clara Cesana, Micol Giulia Cittone, Laura Bandiera, Barbara Scarpati, Valentina Mancini, Silvia Soriani, Silvio Veronese, Mauro Truini, Silvano Rossini, Roberto Cairoli
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(+), CD123(high), 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 remission after acute lymphoblastic leukemia like chemotherapy and allogeneic hematopoietic stem cell transplantation...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28394841/blastic-plasmacytoid-dendritic-cell-neoplasm-evaluated-by-fdg-pet-ct
#15
Zu-Gui Li, Hai-Yu Mu
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematological malignancy. A 34-year-old man with a biopsy-proven BPDCN underwent FDG PET/CT for staging. FDG PET/CT revealed multiple mild FDG-avid cutaneous lesions on the chest and back, involvement of left inguinal lymph node, and a markedly increased FDG-avid subcutaneous mass in the left lower leg.
July 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28393977/-blastic-plasmacytoid-dendritic-cell-neoplasm-with-complete-clinical-remission-with-chemotherapy-and-central-nervous-system-relapse-report-of-one-case
#16
Loreto Contreras, Loreto Mercado, Carolina Delgado, Claudia Cabezas, Laksmi Starke, Mónica Romero, Fernando Ibieta, Mauricio Henríquez, Mauricio Chandia
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...
January 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/28321093/successful-treatment-with-biweekly-chop-for-bone-marrow-relapse-of-blastic-plasmacytoid-dendritic-cell-neoplasm
#17
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
https://www.readbyqxmd.com/read/28191591/plasmacytoid-dendritic-cell-proliferations-and-neoplasms-involving-the-bone-marrow-summary-of-the-workshop-cases-submitted-to-the-18th-meeting-of-the-european-association-for-haematopathology-eahp-organized-by-the-european-bone-marrow-working-group-basel-2016
#18
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)...
May 2017: Annals of Hematology
https://www.readbyqxmd.com/read/27986708/blastic-plasmacytoid-dendritic-cell-neoplasm-is-dependent-on-bcl2-and-sensitive-to-venetoclax
#19
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
https://www.readbyqxmd.com/read/27913457/treatment-of-blastic-plasmacytoid-dendritic-cell-neoplasm
#20
REVIEW
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
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