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Bueso Ramos

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https://www.readbyqxmd.com/read/28596259/morphologic-and-molecular-characteristics-of-de-novo-aml-with-jak2-v617f-mutation
#1
Juliana E Hidalgo-López, Rashmi Kanagal-Shamanna, L Jeffrey Medeiros, Zeev Estrov, C Cameron Yin, Srdan Verstovsek, Sergej Konoplev, Jeffrey L Jorgensen, Mohammad M Mohammad, Roberto N Miranda, Chong Zhao, John Lee, Zhuang Zuo, Carlos E Bueso-Ramos
Background:JAK2 V617F mutation (mut) in acute myeloid leukemia (AML) is rare. We describe the clinicopathologic findings of a single-institution series of 11 de novo AML cases with JAK2 V617. Methods: We identified cases of de novo AML with JAK2 V617F over a 10-year period. We reviewed diagnostic peripheral blood and bone marrow (BM) morphologic, cytogenetic, and molecular studies, including next-generation sequencing. The control group consisted of 12 patients with JAK2 wild-type (wt) AML matched for age, sex, and diagnosis...
June 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28556489/factors-associated-with-risk-of-central-nervous-system-relapse-in-patients-with-non-core-binding-factor-acute-myeloid-leukemia
#2
Elias Jabbour, Naval Guastad Daver, Nicholas James Short, Xuelin Huang, Hsiang-Chun Chen, Abhishek Maiti, Farhad Ravandi, Jorge Cortes, Simon Abi Aad, Guillermo Garcia-Manero, Zeev Estrov, Tapan Kadia, Susan O'Brien, Bouthaina Dabaja, Carlos Bueso-Ramos, Paolo Strati, Carol Bivins, Sherry Pierce, Hagop Kantarjian
Central nervous system (CNS) relapse is uncommon in patients with acute myeloid leukemia (AML) with the use of high-dose cytarabine containing chemotherapy regimens. The clinical and molecular features associated with a higher risk of CNS relapse are not well defined. We assessed the incidence and outcome of CNS relapses among 1245 patients with relapsed/refractory AML referred to our institution between 2000 and 2014. CNS leukemia relapse was observed in 51 patients (4.1%). Using a multivariate regression model and after adjusting for age, FLT3-ITD mutation (OR=2...
May 27, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28551329/myeloproliferative-neoplasms-with-t-8-22-p11-2-q11-2-bcr-fgfr1-a-meta-analysis-of-20-cases-shows-cytogenetic-progression-with-b-lymphoid-blast-phase
#3
Ximena Montenegro-Garreaud, Roberto N Miranda, Alexandra Reynolds, Guilin Tang, Sa A Wang, Mariko Yabe, Wei Wang, Lianghua Fang, Carlos E Bueso-Ramos, Pei Lin, L Jeffrey Medeiros, Xinyan Lu
Rearrangements of FGFR1 result in the 8p11 myeloproliferative syndrome, a group of rare diseases that features a myeloproliferative neoplasm (MPN) that commonly progresses to lymphoblastic leukemia/lymphoma or acute myeloid leukemia. The most common partner of the FGFR1 is ZMYM2 and patients with the ZMYM2-FGFR1 fusion often present with MPN and T-lymphoblastic lymphoma. There are 14 other partners that can fuse with the FGFR1 and of interest is the BCR-FGFR1 fusion that results from t(8;22)(p11.2;q11.2). Patients with t(8;22) often show leukocytosis and present with an MPN resembling chronic myeloid leukemia (CML) or very rarely, with B lymphoblastic leukemia (B-ALL)...
May 24, 2017: Human Pathology
https://www.readbyqxmd.com/read/28548124/oligomonocytic-chronic-myelomonocytic-leukemia-chronic-myelomonocytic-leukemia-without-absolute-monocytosis-displays-a-similar-clinicopathologic-and-mutational-profile-to-classical-chronic-myelomonocytic-leukemia
#4
Julia T Geyer, Wayne Tam, Yen-Chun Liu, Zhengming Chen, Sa A Wang, Carlos Bueso-Ramos, Jean Oak, Daniel A Arber, Eric Hsi, Heesun J Rogers, Katherine Levinson, Adam Bagg, Duane C Hassane, Robert P Hasserjian, Attilio Orazi
Chronic myelomonocytic leukemia is characterized by persistent absolute monocytosis (≥1 × 10(9)/l) in the peripheral blood and dysplasia in ≥1 lineages. In the absence of dysplasia, an acquired clonal genetic abnormality is required or causes for reactive monocytosis have to be excluded. Oligomonocytic chronic myelomonocytic leukemia showing increased monocytes but no absolute monocytosis in the peripheral blood occurs occasionally. These cases are likely classified as myelodysplastic syndrome or myelodysplastic/myeloproliferative neoplasm, unclassifiable...
May 26, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
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
#5
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/28495642/feasibility-of-lenalidomide-therapy-for-persistent-chronic-lymphocytic-leukemia-after-allogeneic-transplantation
#6
Maria R Khouri, Elias J Jabbour, Alison M Gulbis, Francesco Turturro, Celina Ledesma, Martin Korbling, Barry I Samuels, Sairah Ahmed, Amin M Alousi, Stefan O Ciurea, David Marin, Krina K Patel, Uday R Popat, Carlos E Bueso-Ramos, Roland L Bassett, Issa F Khouri
In patients with chronic lymphocytic leukemia (CLL), persistence of disease after allogeneic stem cell transplantation (alloSCT) can result in poor outcomes. In an effort to improve these outcomes, patients with persistent CLL who were 90 to 100 days beyond alloSCT with no evidence of graft-versus-host-disease (GVHD) were randomized to receive lenalidomide or standard care (withdrawal of immunosuppression followed by donor lymphocyte infusion). Lenalidomide was initiated at 5 mg every other day and increased to 10 mg daily, if tolerated, in each patient...
May 8, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28473622/characteristics-and-clinical-significance-of-cytogenetic-abnormalities-in-polycythemia-vera
#7
Guilin Tang, Juliana E Hidalgo Lopez, Sa A Wang, Shimin Hu, Junsheng Ma, Sherry Pierce, Wenli Zuo, Adrian Alejandro Carballo-Zarate, C Cameron Yin, Zhenya Tang, Shaoying Li, L Jeffrey Medeiros, Srdan Verstovsek, Carlos E Bueso-Ramos
UP to 20% of patients with polycythemia vera have karyotypic abnormalities at time of initial diagnosis. However, the cytogenetic abnormalities in polycythemia vera have not been well-characterized and their prognostic impact is largely unknown. In this study, we aimed to address these questions using a large cohort of polycythemia vera patients with cytogenetic information available. The study included 422 patients, 271 in polycythaemic phase, 112 post-polycythaemic myelofibrosis, 11 accelerated phase, and 28 blast phase...
May 4, 2017: Haematologica
https://www.readbyqxmd.com/read/28428103/characteristics-and-clinical-implications-of-reactive-germinal-centers-in-the-bone-marrow
#8
Rose Lou Marie C Agbay, L Jeffrey Medeiros, Joseph D Khoury, Alireza Salem, Carlos E Bueso-Ramos, Sanam Loghavi
Reactive germinal centers (GCs) in the bone marrow (BM) have been described in patients with autoimmune disorders, infections, malignancies and following certain drug therapies, or as an isolated finding without obvious underlying disease. In this study, we describe the clinical conditions in which reactive GCs occur in BM samples, and their topography and accompanying laboratory and ancillary findings in the setting of a cancer center. We identified 32 BM specimens with reactive GCs with an estimated frequency of <0...
April 17, 2017: Human Pathology
https://www.readbyqxmd.com/read/28382648/characteristics-management-and-outcomes-of-patients-with-follicular-dendritic-cell-sarcoma
#9
Preetesh Jain, Sarah A Milgrom, Keyur P Patel, Loretta Nastoupil, Luis Fayad, Michael Wang, Chelsea C Pinnix, Bouthaina S Dabaja, Grace L Smith, Jun Yu, Shimin Hu, Carlos E Bueso Ramos, Rashmi Kanagal-Shamanna, L Jeffrey Medeiros, Yasuhiro Oki, Nathan Fowler
Dendritic cell sarcomas are rare tumours of antigen presenting cells. Data regarding their biology, management and outcomes are sparse. We analysed 66 patients with follicular dendritic cell sarcoma (FDCS). Six patients also had Castleman disease, 9 had another malignancy and 13 had an autoimmune disease. Fifty-four per cent of patients presented with localized disease and 46% with systemic involvement. The median progression-free (PFS) and overall survival (OS) following frontline therapy was 21 and 50 months, respectively...
April 6, 2017: British Journal of Haematology
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
#10
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/28370157/an-exploratory-clinical-trial-of-bortezomib-in-patients-with-lower-risk-myelodysplastic-syndromes
#11
May Daher, Juliana Elisa Hidalgo Lopez, Jasleen K Randhawa, Kausar Jabeen Jabbar, Yue Wei, Naveen Pemmaraju, Gautam Borthakur, Tapan Kadia, Marina Konopleva, Hagop M Kantarjian, Katherine Hearn, Zeev Estrov, Steven Reyes, Carlos E Bueso-Ramos, Guillermo Garcia-Manero
Myelodysplastic syndromes (MDSs) are characterized by ineffective hematopoiesis and an increased risk of transformation. Few effective therapies are available for lower risk MDS patients, especially after the failure of hypomethylating agents. MDS progenitor cells are dependent on the nuclear factor-κB (NF-κB) for survival, which makes it an attractive therapeutic target. As a proteosomal inhibitor, bortezomib is thought to have inhibitory activity against NF-κB. We designed a proof-of-principle study of subcutaneous (SC) bortezomib in lower risk MDS patients with evidence of NF-κB activation in their bone marrow...
March 31, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28370097/natural-history-of-chronic-myelomonocytic-leukemia-treated-with-hypomethylating-agents
#12
Ana Alfonso, Guillermo Montalban-Bravo, Koichi Takahashi, Elias J Jabbour, Tapan Kadia, Farhad Ravandi, Jorge Cortes, Zeev Estrov, Gautam Borthakur, Naveen Pemmaraju, Marina Konopleva, Carlos Bueso-Ramos, Sherry Pierce, Hagop Kantarjian, Guillermo Garcia-Manero
Hypomethylating agents (HMA) are the most commonly used therapeutic intervention in chronic myelomonocytic leukemia (CMML). Due to the lack of CMML-specific clinical trials, the impact of these agents in the natural history of CMML is not fully understood. We present the largest retrospective series of CMML (n = 151) treated with HMA. Mean age at diagnosis was 69 years (range 50-88). According to the CMML-specific prognostic scoring system (CPSS): 17 (15%) were low-risk, 45 (39%) intermediate-1 risk, 42 (36%) intermediate-2, and 12 (10%) high-risk...
March 28, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28340183/crlf2-positive-b-cell-acute-lymphoblastic-leukemia-in-adult-patients-a-single-institution-experience
#13
Sergej Konoplev, Xinyan Lu, Marina Konopleva, Nitin Jain, Juan Ouyang, Maitrayee Goswami, Kathryn G Roberts, Marc Valentine, Charles G Mullighan, Carlos Bueso-Ramos, Patrick A Zweidler-McKay, Jeffrey L Jorgensen, Sa A Wang
Objectives: Cytokine receptor-like factor 2 ( CRLF2 ) rearrangement is found in approximately 50% of pediatric Ph-like B-cell acute lymphoblastic leukemia (B-ALL), and around 50% of CRLF2 + cases harbor JAK mutations. We analyzed CRLF2 expression and studied its correlation with CRLF2 rearrangement in adult patients with B-ALL. Methods: Multiparameter flow cytometry (MFC) was performed consecutively in 126 patients. Results: CRLF2 overexpression was detected in 30 (27%) patients, 28 (41%) of 69 patients with B-ALL not otherwise specified, 14 (21%) of 67 untreated patients, and 16 (27%) of 59 patients with relapsed B-ALL, with the highest among Hispanic patients (25/55, 45%)...
April 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28338082/a-clinical-trial-for-patients-with-acute-myeloid-leukemia-or-myelodysplastic-syndromes-not-eligible-for-standard-clinical-trials
#14
G Montalban-Bravo, X Huang, K Naqvi, E Jabbour, G Borthakur, C D DiNardo, N Pemmaraju, J Cortes, S Verstovsek, T Kadia, N Daver, W Wierda, Y Alvarado, M Konopleva, F Ravandi, Z Estrov, N Jain, A Alfonso, M Brandt, T Sneed, H C Chen, H Yang, C Bueso-Ramos, S Pierce, E Estey, Z Bohannan, H M Kantarjian, G Garcia-Manero
No abstract text is available yet for this article.
March 24, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28264600/quality-and-cost-comparison-of-powered-versus-manual-bone-marrow-biopsy-devices-in-patients-with-myelofibrosis
#15
Abhishek Maiti, Nicholas J Short, Srdan Verstovsek, Cynthia A Powers, Cheryl A Fullmer, Steven R Reyes, Carlos E Bueso-Ramos
No abstract text is available yet for this article.
February 28, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28247057/novel-hematological-parameters-for-the-evaluation-of-patients-with-myeloproliferative-neoplasms-the-immature-platelet-and-reticulocyte-fractions
#16
Paolo Strati, Prithviraj Bose, Lindsey Lyle, Katie Gaw, Lingsha Zhou, Sherry A Pierce, Julie Huynh-Lu, Cheryl F Hirsch-Ginsberg, Daniel E Bueso-Mendoza, Carlos E Bueso-Ramos, Srdan Verstovsek
New automated hematology analyzers have led to the availability of novel hematological parameters, including the immature platelet fraction (IPF) and the immature reticulocyte fraction (IRF), both of potential interest in patients with myeloproliferative neoplasms (MPNs). We performed a prospective analysis of 217 patients with MPN, including 32 (15%) with essential thrombocythemia (ET), 43 (20%) with polycythemia vera (PV), and 142 (65%) with myelofibrosis (MF); the IPF and IRF were measured by the Sysmex XN analyzer...
May 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28246192/more-than-1-tp53-abnormality-is-a-dominant-characteristic-of-pure-erythroid-leukemia
#17
LETTER
Guillermo Montalban-Bravo, Christopher B Benton, Sa A Wang, Farhad Ravandi, Tapan Kadia, Jorge Cortes, Naval Daver, Koichi Takahashi, Courtney DiNardo, Elias Jabbour, Gautam Borthakur, Marina Konopleva, Sherry Pierce, Carlos Bueso-Ramos, Keyur Patel, Steven Kornblau, Hagop Kantarjian, Ken H Young, Guillermo Garcia-Manero, Michael Andreeff
No abstract text is available yet for this article.
May 4, 2017: Blood
https://www.readbyqxmd.com/read/28076892/impact-of-achievement-of-complete-cytogenetic-response-on-outcome-in-patients-with-myelodysplastic-syndromes-treated-with-hypomethylating-agents
#18
Elias Jabbour, Paolo Strati, Monica Cabrero, Susan O'Brien, Farhad Ravandi, Carlos Bueso-Ramos, Qiao Wei, Jianhua Hu, Simon Abi Aad, Nicholas J Short, Courtney Dinardo, Naval Daver, Tapan Kadia, William Wierda, Yue Wei, Simona Colla, Gautam Borthakur, Jorge Cortes, Zeev Estrov, Hagop Kantarjian, Guillermo Garcia-Manero
Two hundred and sixteen consecutive patients with MDS and abnormal karyotype treated with hypomethylating agents between 4/04 and 10/12 were reviewed. Median follow-up was 17 months. Using IWG criteria, best responses were complete response (CR) in 79 patients (37%), partial response (PR) in 4 (2%), and hematologic improvement (HI) in 10 (5%). Cytogenetic response (CyR) was achieved in 78 patients (36%): complete (CCyR) in 62 (29%) and partial in 16 (7%). CyR was achieved in 48 of 79 patients (61%) with CR, 1 of 14 (7%) with PR/HI, and in 29 of the 123 (24%) with no morphologic response...
April 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28074068/a-cpg-island-methylator-phenotype-in-acute-myeloid-leukemia-independent-of-idh-mutations-and-associated-with-a-favorable-outcome
#19
A D Kelly, H Kroeger, J Yamazaki, R Taby, F Neumann, S Yu, J T Lee, B Patel, Y Li, R He, S Liang, Y Lu, M Cesaroni, S A Pierce, S M Kornblau, C E Bueso-Ramos, F Ravandi, H M Kantarjian, J Jelinek, J-Pj Issa
Genetic changes are infrequent in acute myeloid leukemia (AML) compared with other malignancies and often involve epigenetic regulators, suggesting that an altered epigenome may underlie AML biology and outcomes. In 96 AML cases including 65 pilot samples selected for cured/not-cured, we found higher CpG island (CGI) promoter methylation in cured patients. Expanded genome-wide digital restriction enzyme analysis of methylation data revealed a CGI methylator phenotype independent of IDH1/2 mutations we term AML-CGI methylator phenotype (CIMP) (A-CIMP(+))...
January 31, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28038706/hepatosplenic-t-cell-lymphoma-arising-in-patients-with-immunodysregulatory-disorders-a-study-of-7-patients-who-did-not-receive-tumor-necrosis-factor-%C3%AE-inhibitor-therapy-and-literature-review
#20
Mariko Yabe, L Jeffrey Medeiros, Yahya Daneshbod, Masoud Davanlou, Carlos E Bueso-Ramos, Elisa J Moran, Ken H Young, Roberto N Miranda
Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive extranodal T-cell lymphoma that can arise in patients with underlying immune disorders. Others have suggested that tumor necrosis factor (TNF)-α inhibitor therapy for immune disorders increases the risk of HSTCL. To assess for a potential relationship between HSTCL and the use of TNF-α inhibitors, we searched for patients with HSTCL and underlying immune disorders at our institution. We identified 7 patients with a median age of 38 years. Five patients had Crohn disease, 1 ulcerative colitis, and 1 rheumatoid arthritis...
February 2017: Annals of Diagnostic Pathology
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