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https://www.readbyqxmd.com/read/29331736/dysfunctional-telomeres-and-hematological-disorders
#1
REVIEW
Elena Fiorini, Andrea Santoni, Simona Colla
Telomere biology disorders, which are characterized by telomerase activity haploinsufficiency and accelerated telomere shortening, most commonly manifest as degenerative diseases. Tissues with high rates of cell turnover, such as those in the hematopoietic system, are particularly vulnerable to defects in telomere maintenance genes that eventually culminate in bone marrow (BM) failure syndromes, in which the BM cannot produce sufficient new blood cells. Here, we review how telomere defects induce degenerative phenotypes across multiple organs, with particular focus on how they impact the hematopoietic stem and progenitor compartment and affect hematopoietic stem cell (HSC) self-renewal and differentiation...
January 4, 2018: Differentiation; Research in Biological Diversity
https://www.readbyqxmd.com/read/29328013/-a-woman-with-a-painful-and-enlarged-tongue
#2
B P van Lieshout, K H Karagozoglu, E A J M Schulten
A 53-year-old woman presented with painful macroglossia and periorbital papules. Based on this clinical features and biopsies the diagnosis of nodular amyloidosis was established. Further analysis revealed that multiple myeloma was the underlying hematological disorder.
2018: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/29327082/evaluation-of-committed-and-primitive-cord-blood-progenitors-after-expansion-on-adipose-stromal-cells
#3
E R Andreeva, I V Andrianova, A N Gornostaeva, B Sh Gogiya, L B Buravkova
Umbilical cord blood mononuclear fraction is a valuable source of hematopoietic stem and progenitor cells (CB HSPCs). The rarity of this population is a serious limitation of its application in cell therapy. Ex vivo expansion enables to significantly amplify the number of hematopoietic precursors of different commitment. Here, we expand CB MNCs in co-culture with human adipose tissue-derived stromal cells (ASCs) to enrich HSPCs and describe phenotypic features of newly formed hematopoietic populations. The CD34+-HSPCs demonstrated 6-fold enrichment with 9000 CFUs per 50 × 103 HSPCs on average...
January 11, 2018: Cell and Tissue Research
https://www.readbyqxmd.com/read/29322218/-paraneoplasms-of-the-skin
#4
REVIEW
C Gießen-Jung, A Wollenberg, M Reinholz
The cutaneous manifestations of malignancies include nonmalignant skin disorders that occur in association with malignancies (facultative paraneoplastic dermatoses) and skin disorders that are always associated with hematologic diseases or solid tumors (obligate paraneoplastic dermatoses). Paraneoplastic increase of growth factors or immunological reactions lead to a variety of inflammatory, hyperkeratotic or proliferative skin reactions. When paraneoplastic dermatoses develop before cancer is diagnosed, recognition of these skin diseases can accelerate both the diagnosis and treatment...
January 10, 2018: Der Internist
https://www.readbyqxmd.com/read/29320898/did-i-miss-it-discovering-hidden-coexisting-hematological-neoplasms-a-single-institutional-review-of-100-collision-tumors
#5
Evan Himchak, Etan Marks, Yang Shi, Yanhua Wang
A collision tumor is defined as two histologically distinct tumor types identified at the same anatomic site. Hematolymphoid proliferative disorders (HLPDs), which coincide with non-hematological neoplasms, can mimic an immune response and can easily be overlooked as an immune reaction to a solid organ neoplasm, especially when low grade. In order to avoid a delay in the diagnosis of a HLPD during the workup for a non-hematological neoplasm, we identified a cohort of 100 cases with a HLPD diagnosis during the initial workup and treatment of a non-hematological neoplasm, or vice versa...
January 1, 2018: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29318955/case-report-of-igm-multiple-myeloma-diagnosing-a-rare-hematologic-entity
#6
Fernando Javier Bonilla-Valentín, Javier Cerra, William Cáceres-Perkins, Melissa Alsina
IgM multiple myeloma is an exceedingly rare hematologic entity comprising only less than 0.5% of multiple myeloma cases. Given the rarity of this disorder, it makes it a challenge to differentiate from other more prevalent hematologic disorders like Waldenstrom macroglobulinemia. These 2 diseases have the common finding of an IgM monoclonal gammopathy and distinguishing between these 2 diagnoses is of great importance given that therapy and prognosis differ significantly. This report illustrates the case of a 64-year-old man who presented with IgM lambda monoclonal gammopathy in whom signs, symptoms, laboratories, and imaging were initially thought to be consistent with Waldenstrom macroglobulinemia...
January 2018: Cancer Control: Journal of the Moffitt Cancer Center
https://www.readbyqxmd.com/read/29316837/ruxolitinib-for-the-treatment-of-patients-with-steroid-refractory-gvhd-an-introduction-to-the-reach-trials
#7
Madan Jagasia, Robert Zeiser, Michael Arbushites, Patricia Delaite, Brian Gadbaw, Nikolas von Bubnoff
For patients with hematologic malignancies and disorders, allogeneic hematopoietic stem cell transplantation offers a potentially curative treatment option. Many patients develop graft-versus-host disease (GVHD), a serious complication and leading cause of nonrelapse mortality. Corticosteroids are the standard first-line treatment for GVHD; however, patients often become steroid-refractory or remain corticosteroid-dependent. New second-line treatment options are needed to improve patient outcomes. Here we review the role of JAK1 and JAK2 in acute and chronic GVHD...
January 10, 2018: Immunotherapy
https://www.readbyqxmd.com/read/29314691/mastocytosis-pathogenesis-clinical-manifestation-and-treatment
#8
Nicola Wagner, Petra Staubach
The term mastocytosis designates a group of rare disorders characterized by typical skin lesions, frequently associated episodes of anaphylaxis, and clinical symptoms related to the release of various mediators. Dermatologists/allergists are frequently the first to establish the diagnosis. The condition is based on clonal mast cell proliferation, usually in the skin or bone marrow and only rarely in the gastrointestinal tract or other tissues. In general, mastocytosis has a good prognosis in terms of life expectancy...
January 2018: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/29305723/image-guided-ommaya-reservoir-insertion-for-intraventricular-chemotherapy-a-retrospective-series
#9
Jonathan C Lau, Suzanne E Kosteniuk, David R Macdonald, Joseph F Megyesi
BACKGROUND: Ayub Ommaya proposed a surgical technique for subcutaneous reservoir and pump placement in 1963 to allow access to intraventricular cerebrospinal fluid (CSF). Currently, the most common indication for Ommaya reservoir insertion (ORI) in adults is for patients with hematologic or leptomeningeal disorders requiring repeated injection of chemotherapy into the CSF space. Historically, the intraventricular catheter has been inserted blindly based on anatomical landmarks. The purpose of this study was to examine short-term complication rates with ORI with image guidance (IG) and without image guidance (non-IG)...
January 5, 2018: Acta Neurochirurgica
https://www.readbyqxmd.com/read/29304902/laboratory-assessment-of-anemia
#10
Sirisha Kundrapu, Jaime Noguez
Anemia is one of the most common health problems in both industrialized and developing countries. It has been recognized by the World Health Organization as an important disorder leading to significant health care burden. Laboratory testing plays a significant role in the diagnosis of most types of anemia since the clinical diagnosis may not always be straightforward, especially with multiple underlying conditions. Once the existence of anemia is established, the cause must be determined to enable selection of a specific and effective therapy...
2018: Advances in Clinical Chemistry
https://www.readbyqxmd.com/read/29302561/low-backache-in-adults-as-an-initial-presentation-of-acute-lymphoblastic-leukemia
#11
Gunjan Garg, Naveen Chawla, Atul Gogia, Atul Kakar
Low backache as an initial manifestation of acute lymphoblastic leukemia (ALL) in adults has been rarely reported. In this hematological disorder, although bone marrow is replaced by malignant cells, not many cases of low backache as an initial presentation of ALL are reported. We present a series of clinical cases with low backache, which on evaluation found to have ALL.
April 2017: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/29301129/amegakaryocytic-thrombocytopenia-and-subsequent-aplastic-anemia-associated-with-apparent-epstein-barr-virus-infection
#12
Ilana Levy, Ruth Laor, Nizar Jiries, Jacob Bejar, Aaron Polliack, Tamar Tadmor
Acquired amegakaryocytic thrombocytopenia (AAT), a rare entity characterized by severe thrombocytopenia and the absence of megakaryocytes in the bone marrow, may mimic or precede the diagnosis of aplastic anemia (AA). Here, we describe a patient who presented with apparent Epstein-Barr virus (EBV)-associated immune thrombocytopenia resistant to several lines of therapies, which was in fact a form of AAT with some features of AA. He eventually responded to therapy with eltrombopag, cyclosporine A (CSA), and antithymocyte globulin (ATG) and recovered completely...
January 5, 2018: Acta Haematologica
https://www.readbyqxmd.com/read/29299089/hamartoma-of-the-spleen-splenoma-with-calcifications-in-a-child-with-beta-thalassemia-a-case-report
#13
Dario Giambelluca, Dario Picone, Giuseppe Lo Re, Salvatore Pappalardo, Placido Romeo
Splenic hamartoma (or splenoma) is a rare, benign, vascular tumor, often incidentally found at imaging, surgery or autopsy. Albeit usually asymptomatic and rare in children, when it occurs in the pediatric population it is more commonly symptomatic. We report a case of a 15-year-old girl with iron-deficiency anemia and beta-thalassemia, who had a large (10 × 8 × 7 cm) splenic lesion with calcifications, incidentally found during follow-up for splenomegaly and histologically characterized as hamartoma with calcified areas...
May 2017: Journal of Radiology Case Reports
https://www.readbyqxmd.com/read/29296947/detectable-clonal-mosaicism-in-blood-as-a-biomarker-of-cancer-risk-in-fanconi-anemia
#14
Judith Reina-Castillón, Roser Pujol, Marcos López-Sánchez, Benjamín Rodríguez-Santiago, Miriam Aza-Carmona, Juan Ramón González, José Antonio Casado, Juan Antonio Bueren, Julián Sevilla, Isabel Badel, Albert Català, Cristina Beléndez, María Ángeles Dasí, Cristina Díaz de Heredia, Jean Soulier, Detlev Schindler, Luis Alberto Pérez-Jurado, Jordi Surrallés
Detectable clonal mosaicism for large chromosomal events has been associated with aging and an increased risk of hematological and some solid cancers. We hypothesized that genetic cancer predisposition disorders, such as Fanconi anemia (FA), could manifest a high rate of chromosomal mosaic events (CMEs) in peripheral blood, which could be used as early biomarkers of cancer risk. We studied the prevalence of CMEs by single-nucleotide polymorphism (SNP) array in 130 FA patients' blood DNA and their impact on cancer risk...
January 24, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296892/allogeneic-bone-marrow-transplant-in-the-absence-of-cytoreductive-conditioning-rescues-mice-with-%C3%AE-thalassemia-major
#15
Yongliang Huo, Jonathan R Lockhart, Shanrun Liu, Suean Fontenard, Mike Berlett, Thomas M Ryan
β-thalassemia is a group of inherited blood disorders that result in defects in β-globin chain production. Cooley anemia (CA), or β-thalassemia major, is the most severe form of the disease and occurs when an individual has mutations in both copies of the adult β-globin gene. Patients with CA fail to make adult hemoglobin, exhibit ineffective erythropoiesis, experience severe anemia, and are transfusion dependent for life. Currently, allogeneic bone marrow transplantation (BMT) is the only cure; however, few patients have suitable donors for this procedure, which has significant morbidity and mortality...
November 28, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296802/patient-centered-care-coordination-in-hematopoietic-cell-transplantation
#16
REVIEW
Nandita Khera, Patricia Martin, Kristen Edsall, Anthony Bonagura, Linda J Burns, Mark Juckett, Olivia King, C Frederick LeMaistre, Navneet S Majhail
Hematopoietic cell transplantation (HCT) is an expensive, resource-intensive, and medically complicated modality for treatment of many hematologic disorders. A well-defined care coordination model through the continuum can help improve health care delivery for this high-cost, high-risk medical technology. In addition to the patients and their families, key stakeholders include not only the transplantation physicians and care teams (including subspecialists), but also hematologists/oncologists in private and academic-affiliated practices...
August 22, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296774/treated-secondary-acute-myeloid-leukemia-a-distinct-high-risk-subset-of-aml-with-adverse-prognosis
#17
Prajwal Boddu, Hagop M Kantarjian, Guillermo Garcia-Manero, Farhad Ravandi, Srdan Verstovsek, Elias Jabbour, Gautam Borthakur, Marina Konopleva, Kapil N Bhalla, Naval Daver, Courtney D DiNardo, Christopher B Benton, Koichi Takahashi, Zeev Estrov, Sherry R Pierce, Michael Andreeff, Jorge E Cortes, Tapan M Kadia
Secondary acute myeloid leukemia (s-AML) includes therapy-related AML and AML evolving from antecedent hematological disorder (AHD). s-AML arising after treating AHD likely represents a prognostically distinct, high-risk disease category. In this study, treated s-AML (ts-AML) was defined by: (1) prior diagnosis of myelodysplasia, myeloproliferative neoplasm, or aplastic anemia and (2) at least 1 therapy for that diagnosis. ts-AML was categorized by age (< or ≥60 years), and each cohort assessed for response rates and overall survival (OS) on various treatment regimens...
July 25, 2017: Blood Advances
https://www.readbyqxmd.com/read/29289266/is-myc-an-important-biomarker-myc-expression-in-immune-disorders-and-cancer
#18
REVIEW
Shivtia Trop-Steinberg, Yehudit Azar
The proto-oncogene Myc serves as a paradigm for understanding the dynamics of transcriptional regulation. Myc protein has been linked to immune dysfunction, cancer development and neoplastic transformation. We review recent research regarding functions of Myc as an important modulator in immune disorders, postallogeneic hematopoietic stem cell transplantation (HSCT) and several cancers. Myc overexpression has been repeatedly linked to immune disorders and specific cancers, such as myasthenia gravis, psoriasis, pemphigus vulgaris, atherosclerosis, long-term allogeneic survival among HSCT patients, (primary) inflammatory breast cancer, (primary) ovarian carcinoma and hematological malignancies: acute myeloid leukemia, chronic myelogenous leukemia, Hodgkin's lymphoma and diffuse large B-cell lymphoma...
January 2018: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29288719/safety-assessment-of-methanolic-extract-of-terminalia-chebula-fruit-terminalia-arjuna-bark-and-its-bioactive-constituent-7-methyl-gallic-acid-in-vitro-and-in-vivo-studies
#19
N Suganthy, S Muniasamy, G Archunan
Terminalia chebula and Terminalia arjuna were widely used in traditional medicine for the treatment of memory impairment, inflammatory disorders and as an anti-aging agent. However, reports regarding their safety aspects are lacking. Hence, the present study was carried out to investigate the toxicity of methanolic extracts of Terminalia chebula fruit (TCF), Terminalia arjuna bark (TAB) and its bioactive constituent 7- Methyl gallic acid (7MG) under in vitro and in vivo conditions. In vitro toxicity profile of TCF, TAB and 7MG (250-2000 μg/ml) were assessed through cytotoxicity, hemolytic activity, mutagenicity and genotoxicity assays...
December 27, 2017: Regulatory Toxicology and Pharmacology: RTP
https://www.readbyqxmd.com/read/29285951/seifem-2017-from-real-life-to-an-agreement-on-the-use-of-granulocyte-transfusions-and-colony-stimulating-factors-for-prophylaxis-and-treatment-of-infectious-complications-in-patients-with-hematologic-malignant-disorders
#20
Alessandro Busca, Simone Cesaro, Luciana Teofili, Mario Delia, Chiara Cattaneo, Marianna Criscuolo, Francesco Marchesi, Nicola Stefano Fracchiolla, Caterina Giovanna Valentini, Francesca Farina, Roberta Di Blasi, Lucia Prezioso, Angelica Spolzino, Anna Candoni, Maria Ilaria Del Principe, Luisa Verga, Annamaria Nosari, Franco Aversa, Livio Pagano
The rapid spread of severe infections mainly due to resistant pathogens, justifies the search for therapies aiming to restore immune functions severely compromised in patients with hematologic malignancies. Areas covered: The present review summarizes the current knowledge on the role of granulocyte transfusions and colony-stimulating factors as treatment strategy for hematologic patients with serious infectious complications. In addition, a survey among 21 hematologic centers, to evaluate the clinical practice for the use of G-CSF originator and biosimilars was performed...
December 29, 2017: Expert Review of Hematology
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