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Bueso Ramos

Elias Jabbour, Stefan Faderl, Koji Sasaki, Tapan Kadia, Naval Daver, Naveen Pemmaraju, Keyur Patel, Joseph D Khoury, Carlos Bueso-Ramos, Zachary Bohannan, Farhad Ravandi, Gautam Borthakur, Srdan Verstovsek, Darla Miller, Rita Maduike, Chitra Hosing, Hagop M Kantarjian, Guillermo Garcia-Manero
BACKGROUND: The outcome of patients with higher risk myelodysplastic syndromes (MDS) after hypomethylating agent (HMA) failure is poor. This study evaluated the safety and activity of a combination of low-dose clofarabine and cytarabine for these patients. METHODS: Seventy patients with higher risk MDS who had no response, progressed, or relapsed after at least 4 cycles of HMA therapy were treated. RESULTS: The median age was 72 years. Thirty-nine percent of the patients had high-risk disease according to the International Prognostic Scoring System, and 50% of the patients had poor-risk cytogenetics...
October 14, 2016: Cancer
Lanshan Huang, Sa A Wang, Sergej Konoplev, Carlos E Bueso-Ramos, Beenu Thakral, Roberto N Miranda, Elias Jabbour, L Jeffrey Medeiros, Rashmi Kanagal-Shamanna
INTRODUCTION: Well-differentiated systemic mastocytosis (WDSM) is a rare, recently recognized provisional subvariant of systemic mastocytosis (SM). We report a case of WDSM that showed excellent clinical and cutaneous response to imatinib in the absence of known molecular genetic abnormalities. CLINICAL FINDINGS/DIAGNOSES: We present a 24-year-old woman with childhood onset of skin manifestations that progressed to mediator-related systemic events, and a gastrointestinal tract mastocytoma...
October 2016: Medicine (Baltimore)
Xuan J Wang, L Jeffrey Medeiros, Carlos E Bueso-Ramos, Guilin Tang, Sa Wang, Yasuhiro Oki, Parth Desai, Joseph D Khoury, Roberto N Miranda, Zhenya Tang, Nishitha Reddy, Shaoying Li
In patients with diffuse large B-cell lymphoma, MYC rearrangement (MYC-R), MYC expression, or concurrent expression of MYC and BCL2 is associated with a poorer prognosis. P53 expression also has been shown to confer inferior survival in diffuse large B-cell lymphoma patients, but less is known about the role of P53 expression in those with MYC-R, MYC expression (MYC+), or MYC&BCL2 co-expression (MYC+/BCL2+). We studied P53 expression in 201 patients with untreated de novo diffuse large B-cell lymphoma. Sixty-seven (33%) cases were P53 positive, 56 (28%) had MYC-R (including 17 MYC/BCL2 double hit lymphoma), 86 (45%) were MYC+/BCL2+, and 47 (24%) were positive for both MYC and P53...
October 14, 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Daniela Hoehn, Jorge E Cortes, L Jeffrey Medeiros, Elias J Jabbour, Juliana E Hidalgo, Rashmi Kanagal-Shamanna, Carlos E Bueso-Ramos
BACKGROUND: We assessed patients with chronic myelogenous leukemia (CML) for serum calcium (Ca), phosphate (PO4), bone alkaline phosphatase, N-telopeptide (NTx), osteoprotegerin (OPG) levels, and trabecular bone area (TBA) in bone marrow (BM) specimens before and after treatment with dasatinib. We identified a significant increase in percentage of TBA in postdasatinib BM (P = .022). This suggests that dasatinib therapy can increase TBA without significant changes in bone and mineral metabolism...
August 2016: Clinical Lymphoma, Myeloma & Leukemia
Nicholas J Short, Hagop M Kantarjian, Koji Sasaki, Jorge E Cortes, Farhad Ravandi, Deborah A Thomas, Guillermo Garcia-Manero, Issa Khouri, Partow Kebriaei, Richard E Champlin, Sherry Pierce, Ghayas C Issa, Marina Konopleva, Tapan M Kadia, Carlos Bueso-Ramos, Joseph D Khoury, Nitin Jain, Susan M O'Brien, Elias Jabbour
BACKGROUND: The role of day 14 (D14) bone marrow (BM) assessment in detecting increased blasts in patients undergoing induction for acute lymphoblastic leukemia (ALL) is not well defined. METHODS: This study evaluated 389 adolescent and adult patients with previously untreated Philadelphia chromosome-negative ALL who received frontline induction chemotherapy and for whom a D14 BM assessment was performed. RESULTS: A D14 BM blast proportion < 10% (including blast-free aplastic BM) was observed in 319 patients (82%), 10% to 29% was observed in 31 patients (8%), and ≥30% was observed in 39 patients (10%)...
August 10, 2016: Cancer
Juliana E Hidalgo Lopez, Mariko Yabe, Adrian A Carballo-Zarate, Sa A Wang, Jeffrey L Jorgensen, Sairah Ahmed, John Lee, Shaoying Li, Ellen Schlette, Timothy McDonnell, Roberto N Miranda, L Jeffrey Medeiros, Carlos E Bueso-Ramos, C Cameron Yin
T-cell large granular lymphocytic (T-LGL) leukemia after hematopoietic stem cell transplantation (SCT) is rare and its natural history and clinical outcome have not been well described. We report the clinical, morphologic, immunophenotypic, and molecular features of a case of donor-derived T-LGL leukemia in a 16-year-old man who received allogeneic SCT for peripheral T-cell lymphoma not otherwise specified (PTCL-NOS). The patient presented with persistent neutropenia and splenomegaly 9 months after SCT when the chimerism study showed a 100% donor pattern...
August 2016: Journal of the National Comprehensive Cancer Network: JNCCN
Yang O Huh, Guilin Tang, Sameer S Talwalkar, Joseph D Khoury, Maro Ohanian, Carlos E Bueso-Ramos, Lynne V Abruzzo
Double minute chromosomes (dmin) are small, paired chromatin bodies that lack a centromere and represent a form of extrachromosomal gene amplification. Dmin are rare in myeloid neoplasms and are generally associated with a poor prognosis. Most studies of dmin in myeloid neoplasms are case reports or small series. In the current study, we present the clinicopathologic and cytogenetic features of 22 patients with myeloid neoplasms harboring dmin. These neoplasms included acute myeloid leukemia (AML) (n = 18), myelodysplastic syndrome (MDS) (n = 3), and chronic myelomonocytic leukemia (CMML) (n = 1)...
July 2016: Cancer Genetics
Mir Alikhan, Joo Y Song, Aliyah R Sohani, Julien Moroch, Anne Plonquet, Amy S Duffield, Michael J Borowitz, Liuyan Jiang, Carlos Bueso-Ramos, Kedar Inamdar, Madhu P Menon, Sandeep Gurbuxani, Ernest Chan, Sonali M Smith, Alina Nicolae, Elaine S Jaffe, Philippe Gaulard, Girish Venkataraman
Nodal follicular helper T-cell-derived lymphoproliferations (specifically the less common peripheral T-cell lymphomas of follicular type) exhibit a spectrum of histologic features that may mimic reactive hyperplasia or Hodgkin lymphoma. Even though angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma of follicular type share a common biologic origin from follicular helper T-cells and their morphology has been well characterized, flow cytometry of peripheral T-cell lymphomas of follicular type has not been widely discussed as a tool for identifying this reactive hyperplasia/Hodgkin lymphoma mimic...
October 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Héctor M Ramos-Zaldívar, Daniel G Martínez-Irías, Nelson A Espinoza-Moreno, José S Napky-Rajo, Tulio A Bueso-Aguilar, Karla G Reyes-Perdomo, Jimena A Montes-Gambarelli, Isis M Euceda, Aldo F Ponce-Barahona, Carlos A Gámez-Fernández, Wilberg A Moncada-Arita, Victoria A Palomo-Bermúdez, Julia E Jiménez-Faraj, Amanda G Hernández-Padilla, Denys A Olivera, Kevin J Robertson, Luis A Leiva-Sanchez, Edwin Francisco Herrera-Paz
BACKGROUND: Chromosomal region 7q21.3 comprises approximately 5.2 mega base pairs that include genes DLX5/6, SHFM1, and DYNC1I1 associated with split hand/split foot malformation 1. So far, there are reports of eight families with deletion of DYNC1I1 and preserved DLX5/6 associated with ectrodactyly. From these families, only three patients did not present ectrodactyly and, unlike our patient, no other cases have been described as having craniofacial dysmorphology, mitral valve prolapse, kyphoscoliosis, inguinal herniae, or personality disorder...
2016: Journal of Medical Case Reports
Shaoying Li, Annapurna Saksena, Parth Desai, Jie Xu, Zhuang Zuo, Pei Lin, Guilin Tang, C Cameron Yin, Adam Seegmiller, Jeffrey L Jorgensen, Roberto N Miranda, Nishitha M Reddy, Carlos Bueso-Ramos, L Jeffrey Medeiros
MYC/BCL2 double hit lymphoma (DHL) has been the subject of many studies; however, no study has systemically compared the clinicopathologic features and prognostic factors between patients with de novo disease versus those with a history of follicular lymphoma (FL). In addition, the prognostic importance of several other issues remains controversial in these patients. In this retrospective study, we assess 157 patients with MYC/BCL2 DHL including 108 patients with de novo disease and 49 patients with a history of FL or rarely other types of low-grade B-cell lymphoma...
May 19, 2016: Oncotarget
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
Xin Han, L Jeffrey Medeiros, Yu Helen Zhang, M James You, Michael Andreeff, Marina Konopleva, Carlos E Bueso-Ramos
The human homologue of murine double minute 2 (HDM2) and HDM4 negatively regulate p53. HDM4 has not been assessed in acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS). We examined the expression of HDM4 and the short splicing variant, HDM4-S, in bone marrow samples obtained from 85 and 23 patients with AML and MDS, respectively, and 18 negative tumor staging bone marrow samples (used as the control). Immunohistochemical staining showed that HDM4 was overexpressed in 78 AML cases (92%) and 12 MDS cases (52%) compared with 1 stressed bone marrow sample (6%)...
August 2016: Clinical Lymphoma, Myeloma & Leukemia
Yi Zhou, Rashmi Kanagal-Shamanna, Zhuang Zuo, Guilin Tang, L Jeffrey Medeiros, Carlos E Bueso-Ramos
No abstract text is available yet for this article.
August 2016: Annals of Diagnostic Pathology
Sanam Loghavi, Carlos E Bueso-Ramos, Rashmi Kanagal-Shamanna, Chi Young Ok, Alaa A Salim, Mark J Routbort, Meenakshi Mehrotra, Srdan Verstovsek, L Jeffrey Medeiros, Rajyalakshmi Luthra, Keyur P Patel
OBJECTIVES: Calreticulin (CALR) mutations are present in 50% to 85% of JAK2/MPL wild-type (wt) myeloproliferative neoplasms (MPNs). The histopathologic features of CALR-mutated MPNs are unknown. METHODS: We identified 71 patients with essential thrombocythemia (ET), primary myelofibrosis (PMF), and post-essential thrombocythemia myelofibrosis (post-ET MF) with available CALR status. CALR was assessed using capillary electrophoresis followed by Sanger sequencing confirmation...
March 2016: American Journal of Clinical Pathology
Miguel Gallardo, Marisa J Hornbaker, Xiaorui Zhang, Peter Hu, Carlos Bueso-Ramos, Sean M Post
The classification of a gene as an oncogene or a tumor suppressor has been a staple of cancer biology for decades. However, as we delve deeper into the biology of these genes, this simple classification has become increasingly difficult for some. In the case of heterogeneous nuclear ribonuclear protein K (hnRNP K), its role as a tumor suppressor has recently been described in acute myeloid leukemia and demonstrated in a haploinsufficient mouse model. In contrast, data from other clinical correlation studies suggest that hnRNP K may be more fittingly described as an oncogene, due to its increased levels in a variety of malignancies...
June 17, 2016: Cell Cycle
Le Le Aye, Sanam Loghavi, Ken H Young, Imran Siddiqi, C Cameron Yin, Mark J Routbort, Mei Liang, Keith Eilerman, L Jeffrey Medeiros, Russell K Brynes, Carlos Bueso-Ramos
Patients with chronic myelogenous leukemia (CML) present typically with an elevated white blood cell count (WBC) and cytogenetic or molecular genetic evidence of t(9;22)/BCR-ABL1 fusion gene. Rarely, CML patients may present with a normal or mildly elevated WBC and are asymptomatic, and we describe 7 patients in this study. The WBC in these patients ranged from 3.6 to 14.3 K/mm(3) with 50% to 73% granulocytes and 0% blasts. In all patients, t(9;22)(q34;q11.2) was detected by conventional cytogenetics, and BCR-ABL1 fusion was shown, supporting the diagnosis of preleukemic CML (pre-CML)...
April 2016: Annals of Diagnostic Pathology
Wei Wang, Jorge E Cortes, Guilin Tang, Joseph D Khoury, Sa Wang, Carlos E Bueso-Ramos, Joseph A DiGiuseppe, Zi Chen, Hagop M Kantarjian, L Jeffrey Medeiros, Shimin Hu
Clonal cytogenetic evolution with additional chromosomal abnormalities (ACAs) in chronic myelogenous leukemia (CML) is generally associated with decreased response to tyrosine kinase inhibitor (TKI) therapy and adverse survival. Although ACAs are considered as a sign of disease progression and have been used as one of the criteria for accelerated phase, the differential prognostic impact of individual ACAs in CML is unknown, and a classification system to reflect such prognostic impact is lacking. In this study, we aimed to address these questions using a large cohort of CML patients treated in the era of TKIs...
June 2, 2016: Blood
Mariko Yabe, L Jeffrey Medeiros, Guilin Tang, Sa A Wang, Keyur P Patel, Mark Routbort, Govind Bhagat, Carlos E Bueso-Ramos, Jeffrey L Jorgensen, Rajyalakshmi Luthra, Weina Chen, Tariq Muzzafar, Rashmi Kanagal-Shamanna, Joseph D Khoury, Yahya Daneshbod, Masoud Davanlou, Shaoying Li, Ken H Young, Roberto N Miranda
Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell lymphoma commonly associated with cytopenias. The pathogenesis of cytopenias in patients with HSTCL is not well defined, although the presence of dyspoietic hematopoietic cells and the common association with trisomy 8 raise the possibility of an associated myelodysplastic syndrome (MDS). In 25 bone marrow specimens involved by HSTCL, we systematically assessed for morphologic features of dyspoiesis and correlated the findings with peripheral cytopenia(s), cytogenetic findings, and detection of chromosome 8 by fluorescence in situ hybridization...
April 2016: Human Pathology
Monica Cabrero, Yue Wei, Hui Yang, Irene Ganan-Gomez, Zach Bohannan, Simona Colla, Matteo Marchesini, Guillermo Montalban Bravo, Koichi Takahashi, Carlos Bueso-Ramos, Guillermo Garcia-Manero
EZH2 genetic mutations are common in myelodysplastic syndrome (MDS), which implies that this gene has a pathophysiological role in the disease. To further characterize molecular alterations of EZH2, and their potential prognostic impact in MDS, we assessed EZH2 RNA expression in primary bone marrow CD34+ cells from 78 patients. We found that 47% of patients have reduced EZH2 expression compared to normal controls. Further analyses revealed that EZH2 is significantly underexpressed in patients bearing chromosome 7 or 7q deletions (7-alt) when compared to controls, diploid patients, and patients with other cytogenetic alterations (p<0...
May 2016: Leukemia Research
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
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