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https://www.readbyqxmd.com/read/28495918/bone-marrow-morphology-is-a-strong-discriminator-between-chronic-eosinophilic-leukemia-not-otherwise-specified-from-reactive-idiopathic-hypereosinophilic-syndrome
#1
Sa A Wang, Robert P Hasserjian, Wayne Tam, Albert G Tsai, Julia T Geyer, Tracy I George, Kathryn Foucar, Heesun J Rogers, Eric D Hsi, Bryan A Rea, Adam Bagg, Carlos Bueso-Ramos C, Daniel A Arber, Srdan Verstovsek, Attilio Orazi
Chronic eosinophilic leukemia, not otherwise specified can be difficult to distinguish from idiopathic hypereosinophilic syndrome according to the current World Health Organization guideline. To examine if BM morphological features might aid in the differential diagnosis of these two entities, we studied a total of 139 patients with a diagnosis of chronic eosinophilic leukemia, not otherwise specified (n=17) or idiopathic hypereosinophilic syndrome (n=122). As a group, abnormal BM morphological features, resembling myelodysplastic syndromes, myeloproliferative neoplasm or myelodysplastic/myeloproliferative neoplasm were identified in 40/139 (27%) patients, 16(94%) chronic eosinophilic leukemia and 24 (20%) hypereosinophilic syndrome...
May 11, 2017: Haematologica
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
#2
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...
April 15, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28375434/most-myeloid-neoplasms-with-deletion-of-chromosome-16q-are-distinct-from-acute-myeloid-leukemia-with-inv-16-p13-1q22-a-bone-marrow-pathology-group-multicenter-study
#3
MULTICENTER STUDY
Heesun J Rogers, Eric D Hsi, Guilin Tang, Sa A Wang, Carlos E Bueso-Ramos, Daniel Lubin, Jennifer J D Morrissette, Adam Bagg, Durga P Cherukuri, Tracy I George, LoAnn Peterson, Yen-Chun Liu, Susan Mathew, Attilio Orazi, Robert P Hasserjian
Objectives: Isolated deletion of the long arm of chromosome 16 (del(16q)) is rare in myeloid neoplasms (MNs) and was historically considered a variant of inv(16)(p13.1q22) (inv(16)), a subtype of acute myeloid leukemia (AML) associated with CBFB-MYH11 rearrangement and favorable prognosis. This study aims to determine clinicopathologic characteristics of patients with isolated del(16q) in MNs in comparison to AMLs with isolated inv(16). Methods: Clinicopathologic features were retrospectively reviewed in 18 MNs with del(16q) and 34 AMLs with inv(16) patients from seven institutions...
April 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28317298/giant-appendicolith-causing-severe-lameness-in-a-child
#4
Ottavio Adorisio, Massimiliano Silveri, Emanuela Ceriati, Paola Marchetti, Cinzia Orazi, Mauro Colajacomo, Lorenzo Gregori, Francesco De Peppo
No abstract text is available yet for this article.
March 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
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
#5
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/28059092/bone-marrow-morphology-and-disease-progression-in-congenital-thrombocytopenia-a-detailed-clinicopathologic-and-genetic-study-of-eight-cases
#6
Hamilton C Tsang, James B Bussel, Susan Mathew, Yen-Chun Liu, Allison A Imahiyerobo, Attilio Orazi, Julia T Geyer
Patients with congenital thrombocytopenia have an increased risk of developing myeloid neoplasms. In these cases, the morphologic distinction between disease at baseline and at progression is challenging. This report analyzes clinicopathologic features of congenital thrombocytopenia with long-term follow-up at one referral center. Records from the last 20 years were searched for cases of congenital thrombocytopenia with bone marrow biopsies and peripheral blood smears. The clinical, morphologic, immunophenotypic, and molecular features were analyzed...
April 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28006767/a-2-year-longitudinal-prospective-study-of-the-effects-of-eltrombopag-on-bone-marrow-in-patients-with-chronic-immune-thrombocytopenia
#7
MULTICENTER STUDY
Russell K Brynes, Raymond S M Wong, Maung M Thein, Kalpana K Bakshi, Paul Burgess, Dickens Theodore, Attilio Orazi
BACKGROUND: The long-term effects of eltrombopag on bone marrow (BM) reticulin and/or collagen deposition in previously treated adults with chronic immune thrombocytopenia (ITP) were assessed. METHODS: Three BM biopsies were collected at baseline and after 1 and 2 years of eltrombopag treatment. Specimens were centrally processed, stained for reticulin and collagen, independently reviewed by 2 hematopathologists, and rated according to the European Consensus 0-3 scale of marrow fibrosis (MF)...
2017: Acta Haematologica
https://www.readbyqxmd.com/read/27941215/rela-nf-%C3%AE%C2%BAb-subunit-activation-as-a-therapeutic-target-in-diffuse-large-b-cell-lymphoma
#8
Mingzhi Zhang, Zijun Y Xu-Monette, Ling Li, Ganiraju C Manyam, Carlo Visco, Alexandar Tzankov, Jing Wang, Santiago Montes-Moreno, Karen Dybkaer, April Chiu, Attilio Orazi, Youli Zu, Govind Bhagat, Kristy L Richards, Eric D Hsi, William W L Choi, J Han van Krieken, Jooryung Huh, Maurilio Ponzoni, Andrés J M Ferreri, Michael B Møller, Ben M Parsons, Jane N Winter, Miguel A Piris, L Jeffrey Medeiros, Lan V Pham, Ken H Young
It has been well established that nuclear factor kappa-B (NF-κB) activation is important for tumor cell growth and survival. RelA/p65 and p50 are the most common NF-kB subunits and involved in the classical NF-kB pathway. However, the prognostic and biological significance of RelA/p65 is equivocal in the field. In this study, we assessed RelA/p65 nuclear expression by immunohistochemistry in 487 patients with de novo diffuse large B-cell lymphoma (DLBCL), and studied the effects of molecular and pharmacological inhibition of NF-kB on cell viability...
December 8, 2016: Aging
https://www.readbyqxmd.com/read/27933465/nano-mechanical-characterization-of-ataxia-telangiectasia-cells-treated-with-dexamethasone
#9
Michele Menotta, Sara Biagiotti, Giulia Bartolini, Bianchi Marzia, Sara Orazi, Aldo Germani, Luciana Chessa, Mauro Magnani
Ataxia telangiectasia is a rare genetic disease and no therapy is currently available. Glucocorticoid analogues have been shown to improve the neurological symptoms of treated patients. In the present study ataxia telangiectasia and wild type cells were used as a cellular model and treated with dexamethasone. The cells were subsequently investigated for membrane and whole cell mechanical properties by atomic force microscopy. In addition, cytoskeleton protein dynamics and nuclear shapes were assayed by fluorescence microscopy, while western blots were used to assess actin and tubulin content...
March 2017: Cell Biochemistry and Biophysics
https://www.readbyqxmd.com/read/27760757/assessment-of-cd37-b-cell-antigen-and-cell-of-origin-significantly-improves-risk-prediction-in-diffuse-large-b-cell-lymphoma
#10
Zijun Y Xu-Monette, Ling Li, John C Byrd, Kausar J Jabbar, Ganiraju C Manyam, Charlotte Maria de Winde, Michiel van den Brand, Alexandar Tzankov, Carlo Visco, Jing Wang, Karen Dybkaer, April Chiu, Attilio Orazi, Youli Zu, Govind Bhagat, Kristy L Richards, Eric D Hsi, William W L Choi, Jooryung Huh, Maurilio Ponzoni, Andrés J M Ferreri, Michael B Møller, Ben M Parsons, Jane N Winter, Michael Wang, Frederick B Hagemeister, Miguel A Piris, J Han van Krieken, L Jeffrey Medeiros, Yong Li, Annemiek B van Spriel, Ken H Young
CD37 (tetraspanin TSPAN26) is a B-cell surface antigen widely expressed on mature B cells. CD37 is involved in immune regulation and tumor suppression but its function has not been fully elucidated. We assessed CD37 expression in de novo diffuse large B-cell lymphoma (DLBCL), and investigated its clinical and biologic significance in 773 patients treated with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) and 231 patients treated with CHOP. We found that CD37 loss (CD37(-)) in ∼60% of DLBCL patients showed significantly decreased survival after R-CHOP treatment, independent of the International Prognostic Index (IPI), germinal center B-cell-like (GCB)/activated B-cell-like (ABC) cell of origin, nodal/extranodal primary origin, and the prognostic factors associated with CD37(-), including TP53 mutation, NF-κB(high), Myc(high), phosphorylated STAT3(high), survivin(high), p63(-), and BCL6 translocation...
December 29, 2016: Blood
https://www.readbyqxmd.com/read/27739437/the-myeloproliferative-neoplasms-unclassifiable-clinical-and-pathological-considerations
#11
Umberto Gianelli, Daniele Cattaneo, Anna Bossi, Ivan Cortinovis, Leonardo Boiocchi, Yen-Chun Liu, Claudia Augello, Arturo Bonometti, Stefano Fiori, Nicola Orofino, Francesca Guidotti, Attilio Orazi, Alessandra Iurlo
In this study, we investigate in detail the morphological, clinical and molecular features of 71 consecutive patients with a diagnosis of myeloproliferative neoplasms, unclassifiable. We performed a meticulous morphological analysis and found that most of the cases displayed a hypercellular bone marrow (70%) with normal erythropoiesis without left-shifting (59%), increased granulopoiesis with left-shifting (73%) and increased megakaryocytes with loose clustering (96%). Megakaryocytes displayed frequent giant forms with hyperlobulated or bulbous nuclei and/or other maturation defects...
February 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27636396/dexamethasone-improves-redox-state-in-ataxia-telangiectasia-cells-by-promoting-an-nrf2-mediated-antioxidant-response
#12
Sara Biagiotti, Michele Menotta, Sara Orazi, Chiara Spapperi, Serena Brundu, Alessandra Fraternale, Marzia Bianchi, Luigia Rossi, Luciana Chessa, Mauro Magnani
Ataxia telangiectasia (A-T) is a rare incurable neurodegenerative disease caused by biallelic mutations in the gene for ataxia-telangiectasia mutated (ATM). The lack of a functional ATM kinase leads to a pleiotropic phenotype, and oxidative stress is considered to have a crucial role in the complex physiopathology. Recently, steroids have been shown to reduce the neurological symptoms of the disease, although the molecular mechanism of this effect is largely unknown. In the present study, we have demonstrated that dexamethasone treatment of A-T lymphoblastoid cells increases the content of two of the most abundant antioxidants [glutathione (GSH) and NADPH] by up to 30%...
November 2016: FEBS Journal
https://www.readbyqxmd.com/read/27568520/loss-of-prdm1-blimp-1-function-contributes-to-poor-prognosis-of-activated-b-cell-like-diffuse-large-b-cell-lymphoma
#13
Y Xia, Z Y Xu-Monette, A Tzankov, X Li, G C Manyam, V Murty, G Bhagat, S Zhang, L Pasqualucci, C Visco, K Dybkaer, A Chiu, A Orazi, Y Zu, K L Richards, E D Hsi, W W L Choi, J H van Krieken, J Huh, M Ponzoni, A J M Ferreri, M B Møller, B M Parsons, J N Winter, M A Piris, J Westin, N Fowler, R N Miranda, C Y Ok, Y Li, J Li, L J Medeiros, K H Young
PRDM1/BLIMP-1, a master regulator of plasma-cell differentiation, is frequently inactivated in activated B-cell-like (ABC) diffuse large B-cell lymphoma (DLBCL) patients. Little is known about its genetic aberrations and relevant clinical implications. A large series of patients with de novo DLBCL was effectively evaluated for PRDM1/BLIMP-1 deletion, mutation, and protein expression. BLIMP-1 expression was frequently associated with the ABC phenotype and plasmablastic morphologic subtype of DLBCL, yet 63% of the ABC-DLBCL patients were negative for BLIMP-1 protein expression...
March 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/27521149/the-effects-of-hematopoietic-stem-cell-transplant-on-splenic-extramedullary-hematopoiesis-in-patients-with-myeloproliferative-neoplasm-associated-myelofibrosis
#14
Marco Pizzi, Usama Gergis, Felicia Chaviano, Attilio Orazi
BACKGROUND/OBJECTIVE: Hematopoietic stem cell transplant (HSCT) is the only curative treatment for myeloproliferative neoplasm-associated myelofibrosis (MPN-MF). The main clinical manifestation of MPN-MF is splenomegaly secondary to extramedullary hematopoiesis (EMH). The effects of HSCT on splenic EMH and associated vascular and stromal changes are unknown. This study compares the findings seen in spleens following HSCT with those of nontransplanted patients, normal controls, and matched bone marrow (BM) samples...
September 2016: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/27395325/myeloid-sarcoma-presenting-as-low-back-pain-in-the-pediatric-emergency-department
#15
Michela Massoud, Francesca Del Bufalo, Anna Maria Caterina Musolino, Paolo Maria Schingo, Stefania Gaspari, Mara Pisani, Cinzia Orazi, Antonino Reale, Umberto Raucci
BACKGROUND: Low back pain is a common symptom in the pediatric population; approximately half of all children present with at least one episode of low back pain. The majority of cases are due to nonspecific causes such as musculoskeletal trauma with spontaneous regression. On some occasions, however, life-threatening diseases have to be considered. CASE REPORT: A 15-year-old girl presented to the Pediatric Emergency Department for a history of continuous 2-day duration of low back pain and transient paresthesia of the right gluteal area...
September 2016: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27389864/clinicopathologic-evaluation-of-cytopenic-patients-with-isolated-trisomy-8-a-detailed-comparison-between-idiopathic-cytopenia-of-unknown-significance-and-low-grade-myelodysplastic-syndrome
#16
Kseniya Petrova-Drus, Robert Hasserjian, Olga Pozdnyakova, Paola Dal Cin, Susan Mathew, Elizabeth Margolskee, Attilio Orazi, Julia T Geyer
The significance of an isolated trisomy 8 (+8) in the diagnosis of myelodysplastic syndrome (MDS) is not well established. It is common in MDS, but is not considered as an MDS-defining abnormality in the absence of morphologic dysplasia. We evaluated two groups of patients with isolated +8 and either low-grade MDS (LG-MDS) or idiopathic cytopenia of undetermined significance (ICUS). At presentation, ICUS patients had a lower platelet count (85.0 vs 163.5 × 10(9) cells/L; p = 0.02), while MDS patients had more frequent incidence of isolated anemia (64% vs 0%, p = 0...
July 7, 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/27174585/targeted-next-generation-sequencing-identifies-a-subset-of-idiopathic-hypereosinophilic-syndrome-with-features-similar-to-chronic-eosinophilic-leukemia-not-otherwise-specified
#17
Sa A Wang, Wayne Tam, Albert G Tsai, Daniel A Arber, Robert P Hasserjian, Julia T Geyer, Tracy I George, David R Czuchlewski, Kathryn Foucar, Heesun J Rogers, Eric D Hsi, B Bryan Rea, Adam Bagg, Paola Dal Cin, Chong Zhao, Todd W Kelley, Srdan Verstovsek, Carlos Bueso-Ramos, Attilio Orazi
The distinction between chronic eosinophilic leukemia, not otherwise specified and idiopathic hypereosinophilic syndrome largely relies on clonality assessment. Prior to the advent of next-generation sequencing, clonality was usually determined by cytogenetic analysis. We applied targeted next-generation sequencing panels designed for myeloid neoplasms to bone marrow specimens from a cohort of idiopathic hypereosinophilic syndrome patients (n=51), and assessed the significance of mutations in conjunction with clinicopathological features...
August 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27161873/myeloproliferative-neoplasms-bcr-abl1-negative-and-myelodysplastic-myeloproliferative-neoplasms-current-diagnostic%C3%A2-principles-and-upcoming-updates
#18
REVIEW
J T Geyer, A Orazi
Since the publication of the latest World Health Organization (WHO) classification in 2008, there has been a significant effort for clarification of unresolved questions, especially with the help of the rapidly developing field of molecular genetic studies, next-generation sequencing in particular. Numerous entities within the WHO categories of myeloproliferative neoplasms (MPNs) and myelodysplastic (MDS)/MPNs have been extensively studied, with large published series attempting to characterize and better define their morphologic and molecular genetic features...
May 2016: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/27114465/pilz-domain-protein-flgz-mediates-cyclic-di-gmp-dependent-swarming-motility-control-in-pseudomonas-aeruginosa
#19
Amy E Baker, Andreas Diepold, Sherry L Kuchma, Jessie E Scott, Dae Gon Ha, Giulia Orazi, Judith P Armitage, George A O'Toole
UNLABELLED: The second messenger cyclic diguanylate (c-di-GMP) is an important regulator of motility in many bacterial species. In Pseudomonas aeruginosa, elevated levels of c-di-GMP promote biofilm formation and repress flagellum-driven swarming motility. The rotation of P. aeruginosa's polar flagellum is controlled by two distinct stator complexes, MotAB, which cannot support swarming motility, and MotCD, which promotes swarming motility. Here we show that when c-di-GMP levels are elevated, swarming motility is repressed by the PilZ domain-containing protein FlgZ and by Pel polysaccharide production...
July 1, 2016: Journal of Bacteriology
https://www.readbyqxmd.com/read/27069254/the-2016-revision-to-the-world-health-organization-classification-of-myeloid-neoplasms-and-acute-leukemia
#20
REVIEW
Daniel A Arber, Attilio Orazi, Robert Hasserjian, Jürgen Thiele, Michael J Borowitz, Michelle M Le Beau, Clara D Bloomfield, Mario Cazzola, James W Vardiman
The World Health Organization (WHO) classification of tumors of the hematopoietic and lymphoid tissues was last updated in 2008. Since then, there have been numerous advances in the identification of unique biomarkers associated with some myeloid neoplasms and acute leukemias, largely derived from gene expression analysis and next-generation sequencing that can significantly improve the diagnostic criteria as well as the prognostic relevance of entities currently included in the WHO classification and that also suggest new entities that should be added...
May 19, 2016: Blood
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