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myelodysplastic syndrome review

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https://www.readbyqxmd.com/read/28822050/nuances-of-morphology-in-myelodysplastic-diseases-in-the-age-of-molecular-diagnostics
#1
REVIEW
Aaron C Shaver, Adam C Seegmiller
Morphologic dysplasia is an important factor in diagnosis of myelodysplastic syndrome (MDS). However, the role of dysplasia is changing as new molecular genetic and genomic technologies take a more prominent place in diagnosis. This review discusses the role of morphology in the diagnosis of MDS and its interactions with cytogenetic and molecular testing. Recent changes in diagnostic criteria have attempted to standardize approaches to morphologic diagnosis of MDS, recognizing significant inter-observer variability in assessment of dysplasia...
August 18, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28801374/myeloid-sarcoma-in-a-patient-with-myelodysplastic-syndrome-associated-with-del-5q-case-report-and-literature-review
#2
Josh A Showalter, Nidhi Tandon, Bihong Zhao, Guilin Tang, Nghia D Nguyen, L Jeffrey Medeiros
OBJECTIVE: Myeloid sarcoma (MS) is defined in the World Health Organization classification as a tumor mass consisting of myeloblasts with or without maturation and involving any anatomic site other than the bone marrow. We present a case of MS developing in a patient with 5q- myelodysplastic syndrome (MDS) and review the relevant literature. METHODS: A 77-year-old woman with recent diagnosis of MDS associated with del(5q) presented with symptoms and signs attributable to a mass involving the T8 vertebra...
August 2017: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/28797620/extended-follow-up-of-patients-treated-with-bendamustine-for-lymphoid-malignancies
#3
Mara Penne, Maryam Sarraf Yazdy, Kruti Sheth Nair, Bruce D Cheson
INTRODUCTION: Bendamustine, typically in combination with rituximab, is an effective treatment for chronic lymphocytic leukemia (CLL) and B-cell non-Hodgkin lymphoma. Despite its acceptable short-term toxicity profile, long-term toxicities are less well established. This study investigated the long-term adverse effects of bendamustine and responses to subsequent treatments. PATIENTS AND METHODS: Charts of 194 patients were retrospectively reviewed; 54% had received prior treatment (76% attained complete response [CR] or partial response [PR])...
June 30, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28796431/acute-kidney-injury-in-a-postpartum-woman-with-paroxysmal-nocturnal-hemoglobinuria-a-case-report-and-literature-review
#4
Masatoshi Nishimoto, Masaru Matsui, Hideo Tsushima, Kaori Tanabe, Miho Tagawa, Ken-Ich Samejima, Yasuhiro Akai, Yoshihiko Saito
Paroxysmal nocturnal hemoglobinuria is a rare clonal hematopoietic stem cell disorder characterized by intravascular hemolysis, hemoglobinuria, and inflammatory thrombotic state. Intravascular hemolysis in paroxysmal nocturnal hemoglobinuria (PNH) can lead to acute and chronic renal injury through hemoglobin-mediated toxicity. A 32-year-old pregnant woman with myelodysplastic syndrome was admitted to our hospital with severe preeclampsia. Shortly after an urgent caesarean section, she became obtunded and showed signs of acute kidney injury (AKI) with anuria, severe intravascular hemolysis, and hypermagnesemia...
August 10, 2017: Hemodialysis International
https://www.readbyqxmd.com/read/28779902/-erythropoiesis-stimulating-agents-literature-review-of-uses-and-indications-in-advanced-oncological-and-non-oncological-disease-in-the-elderly
#5
Nieves Fernandez Letamendi, Teresa Fernandez Letamendi, M Angeles Montañes Gracia, Valle Recasens Flores
The aim of this article is to review possible indications and controversies about the most frequent uses of ESAs in the treatment of anaemia in elderly patients with oncological and non-oncological diseases. Using PubMed a systematic review was carried out on articles published from 1985 to September 2016, as well as a review of the main Spanish, European, and American consensus guidelines on each of the following diseases in which could pose the treatment of anaemia associated with ESA. A review was also carried out on the main Spanish, European and American consensus guidelines regarding the management of anaemia related to the diseases outlined in this article...
August 2, 2017: Revista Española de Geriatría y Gerontología
https://www.readbyqxmd.com/read/28760304/diagnostic-prognostic-and-predictive-utility-of-recurrent-somatic-mutations-in-myeloid-neoplasms
#6
REVIEW
Umang Patel, Rajyalakshmi Luthra, L Jeffrey Medeiros, Keyur P Patel
The classification and risk stratification of myeloid neoplasms, including acute myeloid leukemia, myelodysplastic syndromes, myelodysplastic syndromes/myeloproliferative neoplasms, and myeloproliferative neoplasms, have increasingly been guided by molecular genetic abnormalities. Gene expression analysis and next-generation sequencing have led to the ever increasing discovery of somatic gene mutations in myeloid neoplasms. Mutations have been identified in genes involved in epigenetic modification, RNA splicing, transcription factors, DNA repair, and the cohesin complex...
July 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28759108/a-call-for-action-increasing-enrollment-of-untreated-patients-with-higher-risk-myelodysplastic-syndromes-in-first-line-clinical-trials
#7
REVIEW
Amer M Zeidan, Maximilian Stahl, Mikkael A Sekeres, David P Steensma, Rami S Komrokji, Steven D Gore
Hypomethylating agents (HMAs) have changed the landscape of the management of patients with higher-risk myelodysplastic syndromes (HR-MDS). HMAs have improved hematopoiesis and quality of life and, in the case of azacitidine, prolonged survival in a large randomized trial. However, multiple real-life and registry analyses have demonstrated minimal survival gains at the population level after the approval of HMAs. Furthermore, the 24-month median survival observed with azacitidine in the landmark AZA-001 trial has not been replicated in population-based studies or in other clinical trials using azacitidine monotherapy arms...
July 31, 2017: Cancer
https://www.readbyqxmd.com/read/28724539/racial-and-ethnic-disparities-in-hematologic-malignancies
#8
Kedar Kirtane, Stephanie J Lee
Racial and ethnic disparities in patients with solid malignancies have been well documented. Less is known about these disparities in patients with hematologic malignancies. With the advent of novel chemotherapeutics and targeted molecular, cellular, and immunologic therapies, it is important to identify differences in care that may lead to disparate outcomes. This review provides a critical appraisal of the empirical research on racial and ethnic disparities in incidence, survival, and outcomes in patients with hematologic malignancies...
July 19, 2017: Blood
https://www.readbyqxmd.com/read/28713749/mucormycosis-of-the-frontal-sinus-a-rare-case-report-and-review
#9
Nanda Kishore Sahoo, Vishal Kulkarni, Amit K Bhandari, Arun Kumar
Mucormycosis of the frontal sinus are rarely seen in day to day clinical practice. Although this fungus is commonly found in the environment, the disease is usually prevented by the immune system and is hence rare. Well-recognized risk factors for the disease include diabetes mellitus, leukemia, aplastic anemia, myelodysplastic syndrome, blood dyscrasias, and immunosuppressive therapy in organ transplantation, renal disease, sepsis, and severe burns. The disease is primarily found in those who are immunocompromised, but it may also manifest in immuno competent persons...
January 2017: Annals of Maxillofacial Surgery
https://www.readbyqxmd.com/read/28708320/monosomy-7-del-7q-in-inherited-bone-marrow-failure-syndromes-a-systematic-review
#10
REVIEW
Alex Pezeshki, Shreya Podder, Ralph Kamel, Seth J Corey
Inherited bone marrow failure syndromes (IBMFS) are rare cancer predisposition syndromes with an especially high risk of transformation to myelodysplastic syndrome (MDS) and/or acute myeloid leukemia (AML). We performed a retrospective systematic review of reported MDS/AML arising in the eight most common IBMFS to determine the frequency and outcome of chromosome 7 abnormalities. We identified 738 MDS/AML cases of 4,293 individuals. Monosomy 7 or del (7q) occurred in ∼17%. Greater understanding of the roles played by sequential acquisition of genetic and cytogenetic changes will provide insights into myeloid leukemogenesis and improve the surveillance and hopefully outcomes for individuals with IBMFS...
July 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28706555/advances-in-iron-chelation-therapy-transitioning-to-a-new-oral-formulation
#11
Nirmish R Shah
Iron overload is a concern for patients who require repeated red-blood-cell transfusions due to conditions such as sickle cell disease, thalassemia, or myelodysplastic syndromes. The recommended treatment for removing excess iron in these patients is iron chelation therapy. Currently available iron chelators include deferoxamine, which is administered by injection, and deferasirox and deferiprone, both of which are administered orally. Adherence to iron chelator therapy is an important consideration and may be affected by side effects...
2017: Drugs in Context
https://www.readbyqxmd.com/read/28685840/european-leukemianet-study-on-the-reproducibility-of-bone-marrow-features-in-masked-polycythemia-vera-and-differentiation-from-essential-thrombocythemia
#12
Hans Michael Kvasnicka, Attilio Orazi, Juergen Thiele, Giovanni Barosi, Carlos E Bueso-Ramos, Alessandro M Vannucchi, Robert P Hasserjian, Jean-Jacques Kiladjian, Umberto Gianelli, Richard Silver, Tariq I Mughal, Tiziano Barbui
The purpose of the study was to assess consensus and interobserver agreement among an international panel of six hematopathologists regarding characterization and reproducibility of bone marrow (BM) histologic features used to diagnose early stage myeloproliferative neoplasms, in particular differentiation of so-called masked/prodromal polycythemia vera (mPV) from JAK2-mutated essential thrombocythemia (ET). The six members of the hematopathology panel evaluated 98 BM specimens independently and in a blinded fashion without knowledge of clinical data...
July 7, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28685821/pd-1-pd-l1-inhibitors-in-haematological-malignancies-update-2017
#13
REVIEW
Tomas Jelinek, Jana Mihalyova, Michal Kascak, Juraj Duras, Roman Hajek
The introduction of PD-1/PD-L1 pathway inhibitors is an important landmark in solid oncology with unprecedented practice-changing activity in various types of solid tumours. Among haematological malignancies, PD-1/PD-L1 inhibitors have been successful, so far, only in the treatment of classical Hodgkin lymphoma, which typically exhibits an over-expression of PD-1 ligands (PD-L1, PD-L2) due to alterations in chromosome 9p24.1. Such positive outcomes led to the US Food and Drug Administration approval of nivolumab use in relapsed Hodgkin lymphoma in 2016 as the first haematological indication...
July 6, 2017: Immunology
https://www.readbyqxmd.com/read/28683583/successful-controlled-ovarian-stimulation-and-vitrification-of-oocytes-in-an-adolescent-diagnosed-with-myelodysplastic-pre-malignant-clone-with-monosomy-7
#14
Valerie Laura Peddie, Abha Maheshwari
An improvement in long-term outcomes for malignancies and non-malignant conditions, together with a review of the NICE Guideline, has led to a need to provide clinical services to deal with the sequelae of disease, its treatment, and subsequent survival of young people diagnosed with cancer. In this article, we describe fertility preservation in an adolescent female diagnosed with Myelodysplastic/pre-malignant Clone with Monosomy 7 with pathophysiology like that of chronic myeloid leukaemia (CML) with known genetic markers in the tumour cells...
July 7, 2017: Human Fertility: Journal of the British Fertility Society
https://www.readbyqxmd.com/read/28680340/successful-treatment-of-pulmonary-candidiasis-and-aspergillosis-in-patient-with-refractory-hodgkin-lymphoma-using-micafungin-case-study-and-brief-literature-review
#15
Marta Barańska, Renata Kroll-Balcerzak, Lidia Gil, Joanna Rupa-Matysek, Mieczysław Komarnicki
The number of patients with hematological malignancies who develop invasive fungal disease (IFD) has increased dramatically in recent decades. This increase is attributed to impairment of the host immune system due to intensive cytotoxic chemotherapies, use of corticosteroids and profound immunosuppression after hematopoietic stem cell transplantation (HSCT). Additionally, the increasing prevalence of fungal infections caused by emerging and rare pathogens, IFD of mixed etiology or of atypical localization is observed...
2017: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/28679999/bone-marrow-failure-in-childhood-central-pathology-review-of-a-nationwide-registry
#16
Masafumi Ito
Refractory cytopenia of childhood (RCC) was proposed as a provisional entity in the 2008 WHO classification of myelodysplastic syndromes (MDS). It is defined as a childhood MDS featuring persistent cytopenia without increase blasts in bone marrow (BM) or peripheral blood (PB). Because the majority of RCC cases feature hypocellularity and pancytopenia, it is quite challenging to differentiate RCC from acquired aplastic anemia (AA) and many kinds of inherited bone marrow failure syndromes (IBMFS). Diagnosis of RCC requires BM histology of characteristic features such as isolated erythroid islet with left shift, abnormal localization and micromegakaryocytes...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28679998/deregulated-polycomb-complex-function-in-the-pathogenesis-of-mds
#17
Atsushi Iwama
Epigenetic regulation holds a key role in gene expression due to its modulation of the structure and function of chromatin. Notably, epigenetic dysregulation is deeply involved in the pathogenesis of hematological malignancies. Polycomb group (PcG) genes encoding histone modifier proteins are representative epigenetic genes that regulate a variety of cellular functions, including self-renewal and multi-lineage differentiation of stem cells. Surprisingly, many PcG genes are targeted by deletions or somatic mutations or both in a number of hematological malignancies, such as myelodysplastic syndrome (MDS)...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28675065/hypomethylating-agents-hma-treatment-for-myelodysplastic-syndromes-alternatives-in-the-frontline-and-relapse-settings
#18
Natalie Uy, Abhay Singh, Steven D Gore, Thomas Prebet
Hypomethylating agents (HMA) have played a pivotal role for treating myelodysplastic syndromes (MDS) over the past decade, inducing sustained hematological responses and delaying progression to leukemia. However, a vast majority of patients will experience treatment failure within 2 years, with poor prognoses and limited options, and management of this growing patient population remains unclear. Areas covered: With the introduction of new agents in the MDS field, a better understanding of the biology of MDS, and updated information on standard of care options (including allogeneic transplantation), we re-evaluate the global treatment strategy in MDS via novel agents, focusing in particular on investigational approaches for patients who fail to respond to HMA when applicable...
August 1, 2017: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/28668372/-two-cases-of-myeloid-sarcomas-with-review-of-literature
#19
P Mottaz, F Becot-Bretez, R Jeanneau, G Vignon, A Bertin, F Carrere, P F Augereau, P Aucher, F Lellouche
INTRODUCTION: Myeloid sarcomas are uncommon proliferations of immature myeloid cells occurring in any extramedullary organ. We report here two cases of myeloid sarcomas in patients with, respectively, a polycythemia vera and a myelodysplastic syndrome. CASE REPORTS: The first is an 81-year-old woman who presented with osteolytic lesions. Diagnosis has been highlighted using anatomopathological study after bone marrow biopsy, but it was delayed because of a very localized basin lesion and few positive myeloid markers...
June 28, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28645262/acute-myeloid-leukemia-and-pregnancy-clinical-experience-from-a-single-center-and-a-review-of-the-literature
#20
Nicola Stefano Fracchiolla, Mariarita Sciumè, Francesco Dambrosi, Francesca Guidotti, Manuela Wally Ossola, Giovanna Chidini, Umberto Gianelli, Daniela Merlo, Agostino Cortelezzi
BACKGROUND: Acute myeloid leukemia (AML) accounts for more than two thirds of leukemia during pregnancy and has an incidence of 1 in 75,000 to 100,000. Its clinical management remains a challenging therapeutic task both for patient and medical team, given to the therapy-attributable risks for mother and fetus and the connected counseling regarding pregnancy continuation. METHODS: We provided a review of updated literature and a comprehensive description of five maternal/fetal outcomes of AML cases diagnosed concomitantly to pregnancy and treated at our Institution from 2006 to 2012...
June 23, 2017: BMC Cancer
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