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https://www.readbyqxmd.com/read/28630775/a-rare-case-of-paraneoplastic-aortitis-associated-with-chronic-myelomonocytic-leukemia
#1
Sylwia Sasinowska, Pamela Traisak, Michael McCormack, Hala Eid
Aortitis is a broad term describing inflammation of the aorta. The most common causes of aortitis are the large-vessel vasculitides giant cell arteritis and Takayasu arteritis. Other etiologies include aortitis associated with other autoimmune disorders, infectious causes, and paraneoplastic and idiopathic cases. We describe a rare case of a large-vessel arteritis occurring in association with chronic myelomonocytic leukemia (CMML). A 68-year-old female with recent diagnosis of CMML presented to our office for evaluation of abnormal chest computed tomography (CT) that showed inflammation surrounding the entirety of thoracic and abdominal aorta, consistent with aortitis...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28621841/hypomethylating-agent-therapy-use-and-survival-in-older-patients-with-chronic-myelomonocytic-leukemia-in-the-united-states-a-large-population-based-study
#2
Amer M Zeidan, Xin Hu, Jessica B Long, Rong Wang, Xiaomei Ma, Nikolai A Podoltsev, Scott F Huntington, Steven D Gore, Amy J Davidoff
BACKGROUND: Despite the approval of azacitidine in 2004 and the approval of decitabine in 2006 in the United States for chronic myelomonocytic leukemia (CMML), the overall survival (OS) benefit with hypomethylating agent (HMA) therapy is unclear. METHODS: Older adults (age ≥ 66 years) who had been diagnosed with CMML from 2001 to 2011 were selected from the Surveillance, Epidemiology, and End Results-Medicare database, and propensity score matching was used to match patients who had been diagnosed after HMA approval (2007-2011) and had received HMA treatment with patients diagnosed before HMA approval (2001-2003)...
June 16, 2017: Cancer
https://www.readbyqxmd.com/read/28611023/accumulation-of-classical-monocytes-defines-a-subgroup-of-mds-that-frequently-evolve-into-cmml
#3
Dorothée Selimoglu-Buet, Bouchra Badaoui, Emmanuel Benayoun, Andréa Toma, Pierre Fenaux, Bruno Quesnel, Gabriel Etienne, Thorsten Braun, Nassera Abermil, Margot Morabito, Nathalie Droin, Eric Solary, Orianne Wagner-Ballon
No abstract text is available yet for this article.
June 13, 2017: Blood
https://www.readbyqxmd.com/read/28607470/a-phase-ii-multicentre-trial-of-decitabine-in-higher-risk-chronic-myelomonocytic-leukemia
#4
V Santini, B Allione, G Zini, D Gioia, M Lunghi, A Poloni, D Cilloni, A Sanna, E Masiera, M Ceccarelli, O Abdel-Wahab, A Terenzi, E Angelucci, C Finelli, F Onida, A M Pelizzari, D Ferrero, G Saglio, M Figueroa, A Levis
Chronic myelomonocytic leukemia (CMML) is a complex clonal hematological disorder classified among myelodysplastic/myeloproliferative neoplasms (MDS/MPNs). Prognosis is poor and there is a lack of effective treatments. The hypomethylating agent decitabine has shown activity against MDS and elderly acute myeloid leukemia, but there is little data focusing specifically on its efficacy in CMML. In this prospective, phase 2 Italian study, CMML patients received i.v. decitabine 20 mg/m(2)/day on Days 1 to 5 of a 28-day treatment cycle...
June 13, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28593791/eosinophil-rich-tissue-infiltrates-in-chronic-myelomonocytic-leukemia-patients
#5
Nathalie Droin, Nolwenn Lucas, Vincent Parinet, Dorothée Selimoglu-Buet, Marc Humbert, Véronique Saada, Olivier Lambotte, Eric Solary, Nicolas Noël
Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic disorder that associates dysplastic and proliferative features. Tissue inflammatory disorders occur in a fraction of CMML patients during the course of their disease. Here, we describe the occurrence of eosinophil-rich tissue inflammation, including eosinophilic pneumonia, chondritis, and cystitis, in CMML patients. Whole exome sequencing of leukemic cells did not identify a recurrent genetic abnormality among these three patients who were clinically improved by local or oral corticosteroids...
June 8, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28576879/robust-patient-derived-xenografts-of-mds-mpn-overlap-syndromes-capture-the-unique-characteristics-of-cmml-and-jmml
#6
Akihide Yoshimi, Maria E Balasis, Alexis Vedder, Kira Feldman, Yan Ma, Hailing Zhang, Stanley Chun-Wei Lee, Christopher Letson, Sandrine Niyongere, Sydney X Lu, Markus Ball, Justin Taylor, Qing Zhang, Yulong Zhao, Salma Youssef, Young Rock Chung, Xiao Jing Zhang, Benjamin H Durham, Wendy Yang, Alan F List, Mignon L Loh, Virginia Klimek, Michael F Berger, Elliot Stieglitz, Eric Padron, Omar Abdel-Wahab
Chronic myelomonocytic leukemia (CMML) and juvenile myelomonocytic leukemia (JMML) are characterized by monocytosis, myelodysplasia, and a characteristic hypersensitivity to GM-CSF. Currently, there are no available disease-modifying therapies for CMML, nor are there preclinical models that fully recapitulate the unique features of CMML. Through use of immunocompromised mice with transgenic expression of human GM-CSF, IL-3, and SCF in a NOD/SCID-IL2Rγ(null) background (NSGS mice), we demonstrate remarkable engraftment of CMML and JMML providing the first examples of serially transplantable and genetically accurate models of CMML...
June 2, 2017: Blood
https://www.readbyqxmd.com/read/28572287/how-i-treat-chronic-myelomonocytic-leukemia
#7
Eric Solary, Raphael Itzykson
Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic malignancy that may deserve specific management. Defined by a persistent peripheral blood monocytosis ≥1 × 10(9)/L and monocytes accounting for ≥10% of the white blood cells, this ageing-associated disease combines cell proliferation as a consequence of myeloid progenitor hypersensitivity to granulocyte-macrophage colony stimulating factor (GM-CSF) with myeloid cell dysplasia and ineffective hematopoiesis. The only curative option for CMML remains allogeneic stem cell transplantation...
June 1, 2017: Blood
https://www.readbyqxmd.com/read/28555081/genomic-determinants-of-chronic-myelomonocytic-leukemia
#8
B Patel, B Przychodzen, S Thota, T Radivoyevitch, V Visconte, T Kuzmanovic, M Clemente, C Hirsch, A Morawski, R Souaid, C Saygin, A Nazha, B Demarest, T LaFramboise, H Sakaguchi, S Kojima, H Carraway, S Ogawa, H Makishima, M Sekeres, J Maciejewski
The biology, clinical phenotype and progression rate of chronic myelomonocytic leukemia (CMML) are highly variable due to diverse initiating and secondary clonal genetic events. To determine the effects of molecular features including clonal hierarchy in CMML, we studied whole exome and targeted next generation sequencing data from 150 patients with robust clinical and molecular annotation assessed cross-sectionally and at serial time points of disease evolution. To identify molecular lesions unique to CMML we compared it to related myeloid neoplasms (N=586), including JMML, MDS, and primary monocytic AML and discerned distinct molecular profiles despite similar patho-morphologic features...
May 30, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28554891/dna-repair-gene-expressions-are-related-to-bone-marrow-cellularity-in-myelodysplastic-syndrome
#9
Howard L Ribeiro, Allan Rodrigo S Maia, Roberta Taiane G de Oliveira, Marília Braga Costa, Izabelle Rocha Farias, Daniela de Paula Borges, Juliana Cordeiro de Sousa, Silvia Maria M Magalhães, Ronald F Pinheiro
OBJECTIVE: To evaluate the expression of genes related to nuclear excision (ERCC8, XPA and XPC), homologous recombination and non-homologous end-joining (ATM, BRCA1, BRCA2 and LIG4) repair mechanisms, using quantitative PCR methodologies, and it relation with bone marrow cellularity in myelodysplastic syndrome (MDS). METHODS AND RESULTS: A total of 51 adult de novo patients with MDS (3 refractory anaemia (RA), 11 refractory anaemia with ringed sideroblasts (RARS), 28 refractory cytopenia with multilineage dysplasia (RCMD), 3 refractory anaemia with excess blasts type I (RAEB-I), 5 refractory anaemia with excess blasts type II (RAEB-II), and 1 chronic myelomonocytic leukaemia (CMML) were evaluated...
May 29, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28546581/a-phase-1b-2b-multicenter-study-of-oral-panobinostat-plus-azacitidine-in-adults-with-mds-cmml-or-aml-with-%C3%A2-30-blasts
#10
G Garcia-Manero, M A Sekeres, M Egyed, M Breccia, C Graux, J D Cavenagh, H Salman, A Illes, P Fenaux, D J DeAngelo, R Stauder, K Yee, N Zhu, J-H Lee, D Valcarcel, A MacWhannell, Z Borbenyi, L Gazi, S Acharyya, S Ide, M Marker, O G Ottmann
Treatment with azacitidine (AZA), a demethylating agent, prolonged overall survival (OS) vs conventional care in patients with higher-risk myelodysplastic syndromes (MDS). As median survival with monotherapy is <2 years, novel agents are needed to improve outcomes. This phase 1b/2b trial (n=113) was designed to determine the maximum tolerated dose (MTD) or recommended phase 2 dose (RP2D) of panobinostat (PAN) + AZA (phase 1b) and evaluate early efficacy and safety of PAN+AZA vs AZA monotherapy (phase 2b) in patients with higher-risk MDS, chronic myelomonocytic leukemia, or oligoblastic acute myeloid leukemia with <30% blasts...
May 26, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28535394/red-blood-cell-alloimmunization-in-184-patients-with-myeloid-neoplasms-treated-with-azacitidine-a-retrospective-single-center-experience
#11
M Leisch, L Weiss, N Lindlbauer, C Jungbauer, A Egle, E Rohde, R Greil, C Grabmer, L Pleyer
Alloimmunization to Red Blood Cell (RBC) antigens frequently occurs in patients with myeloid neoplasms (AML, MDS and CMML) and potentially poses the patient at risk for delayed hemolytic transfusion reactions and limited supply of compatible RBC-units. However, there is comparatively little data on transfusion associated characteristics in this patient cohort. We therefore retrospectively analyzed transfusion requirements and clinical outcomes of 184 patients with myloid neoplasms treated with azacitidine at the Paracelsus Medical University Salzburg, which were included in the Austrian Registry of Hypomethylating Agents...
May 9, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28526957/cytomorphology-review-of-100-newly-diagnosed-lower-risk-mds-patients-in-the-european-leukemianet-mds-eumds-registry-reveals-a-high-inter-observer-concordance
#12
Louise de Swart, Alex Smith, Marius MacKenzie, Argiris Symeonidis, Judith Neukirchen, Dana Mikulenková, Teresa Vallespí, Gina Zini, Malgorzata Paszkowska-Kowalewska, Anton Kruger, Leonie Saft, Pierre Fenaux, David Bowen, Eva Hellström-Lindberg, Jaroslav Čermák, Reinhard Stauder, Aurelia Tatic, Mette Skov Holm, Luca Malcovati, Krzysztof Mądry, Jackie Droste, Nicole Blijlevens, Theo de Witte, Ulrich Germing
The European LeukemiaNet MDS (EUMDS) registry is collecting data of myelodysplastic syndrome (MDS) patients belonging to the IPSS low or intermediate-1 category, newly diagnosed by local cytologists. The diagnosis of MDS can be challenging, and some data report inter-observer variability with regard to the assessment of the MDS subtype. In order to ensure that correct diagnoses were made by the participating centres, blood and bone marrow slides of 10% of the first 1000 patients were reviewed by an 11-person panel of cytomorphologists...
July 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28486043/randomized-phase-ii-study-of-azacitidine-alone-or-in-combination-with-lenalidomide-or-with-vorinostat-in-higher-risk-myelodysplastic-syndromes-and-chronic-myelomonocytic-leukemia-north-american-intergroup-study-swog-s1117
#13
Mikkael A Sekeres, Megan Othus, Alan F List, Olatoyosi Odenike, Richard M Stone, Steven D Gore, Mark R Litzow, Rena Buckstein, Min Fang, Diane Roulston, Clara D Bloomfield, Anna Moseley, Aziz Nazha, Yanming Zhang, Mario R Velasco, Rakesh Gaur, Ehab Atallah, Eyal C Attar, Elina K Cook, Alyssa H Cull, Michael J Rauh, Frederick R Appelbaum, Harry P Erba
Purpose Azacitidine is standard, first-line therapy in higher-risk myelodysplastic syndromes (MDS). Whether azacitidine-based combinations with lenalidomide or vorinostat produce superior overall response rates (ORRs) to azacitidine is not known. Patients and Methods North American Intergroup Study S1117 is a phase II/III trial that randomly assigned patients with higher-risk MDS and chronic myelomonocytic leukemia (CMML) 1:1:1 to azacitidine (75 mg/m(2)/day on days 1 to 7 of a 28-day cycle); azacitidine plus lenalidomide (10 mg/day on days 1 to 21); or azacitidine plus vorinostat (300 mg twice daily on days 3 to 9)...
May 9, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28476038/prognostic-role-of-tet2-deficiency-in-myelodysplastic-syndromes-a-meta-analysis
#14
Yun Lin, Zhijuan Lin, Kun Cheng, Zhihong Fang, Zhifeng Li, Yiming Luo, Bing Xu
Tet methylcytosine dioxygenase2 gene (TET2) is one of the most frequently mutated gene in myeloid neoplasm, but the prognostic role of TET2 aberrations in myelodysplastic syndromes (MDS) remains unclear. Therefore, we performed a meta-analysis. Fourteen eligible studies with 1983 patients were included in this meta-analysis. Among these, 2 studies evaluated the impact that the TET2 expression level had on the prognosis. The combined hazard ratios (HR) estimated for overall survival (OS) was 1.00 (95%CI: 0.74 to 1...
April 18, 2017: Oncotarget
https://www.readbyqxmd.com/read/28455647/cmml-clinical-and-molecular-aspects
#15
REVIEW
Raphael Itzykson, Matthieu Duchmann, Nolwenn Lucas, Eric Solary
Chronic Myelomonocytic Leukemia is a chronic myeloid neoplasm occurring mostly in the elderly with overlapping features of myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN) characterized by chronic monocytosis. Recent progresses in the molecular and cellular pathogenesis of CMML have stirred a renewed interest in this clinically heterogeneous disorder. Here, we review the recent progresses in the biology of CMML and how it affects its current and future clinical management.
June 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28450577/the-thin-line-between-cml-and-cmml
#16
Megan Parilla, Girish Venkataraman
No abstract text is available yet for this article.
April 27, 2017: Blood
https://www.readbyqxmd.com/read/28442268/generalized-palisaded-neutrophilic-and-granulomatous-dermatitis-a-cutaneous-manifestation-of-chronic-myelomonocytic-leukemia-a-clinical-histopathological-and-molecular-study-of-three-cases
#17
Birgit Federmann, Irina Bonzheim, Amir S Yazdi, Janine Schmidt, Falko Fend, Gisela Metzler
Palisaded neutrophilic and granulomatous dermatitis (PNGD) is characterized by erythematous papules or plaques on trunk or limbs and is frequently associated with rheumatological, autoimmune or hematological malignancies. Histopathology shows interstitial granulomas composed of epitheloid histiocytes in the reticular dermis with surrounding foci of collagen degeneration and variable neutrophilic inflammation. We report three cases of generalized PNGD associated with chronic myelomonocytic leukemia (CMML), a myelodysplastic/myeloproliferative neoplasm, which may show a variety of cutaneous manifestations...
April 22, 2017: Human Pathology
https://www.readbyqxmd.com/read/28370365/monocytosis-in-polycythemia-vera-clinical-and-molecular-correlates
#18
Daniela Barraco, Sonia Cerquozzi, Naseema Gangat, Mrinal M Patnaik, Terra Lasho, Christy Finke, Curtis A Hanson, Rhett P Ketterling, Animesh Pardanani, Ayalew Tefferi
Monocytosis (absolute monocyte count, AMC ≥ 1 × 10(9) /L) might accompany a spectrum of myeloid neoplasms, other than chronic myelomonocytic leukemia (CMML). In the current study, we examined the prevalence, laboratory and molecular correlates, and prognostic relevance of monocytosis in polycythemia vera (PV). Among 267 consecutive patients with World Health Organization (WHO)-defined PV, 55 (21%) patients displayed an AMC of ≥1 × 10(9) /L and 18 (7%) an AMC of ≥1.5 × 10(9) /L. In general, PV patients with monocytosis were significantly older and displayed higher frequencies of leukocytosis (81% vs...
March 28, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28370097/natural-history-of-chronic-myelomonocytic-leukemia-treated-with-hypomethylating-agents
#19
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/28368509/in-higher-risk-myelodysplastic-syndromes-and-low-blast-count-acute-myeloid-leukemia-results-of-the-bmt-aza-prospective-study
#20
M T Voso, G Leone, A Piciocchi, L Fianchi, S Santarone, A Candoni, M Criscuolo, A Masciulli, E Cerqui, A Molteni, C Finelli, M Parma, A Poloni, A M Carella, F Spina, A Cortelezzi, F Salvi, E P Alessandrino, A Rambaldi, S Sica
Background: Allogeneic stem cell transplantation (HSCT) is the only curative treatment in myelodysplastic syndromes (MDS). Azacitidine (AZA) is increasingly used prior to HSCT, however in Europe it is only approved for patients who are not eligible for HSCT. Patients and Methods: We conducted a phase II multicenter study to prospectively evaluate the feasibility of HSCT after treatment with AZA in 70 patients with a myelodysplastic syndrome (MDS), 19 with acute myeloid leukemia (AML), and 8 with chronic myelomonocytic leukemia (CMML)...
March 29, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
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