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https://www.readbyqxmd.com/read/28742285/variable-expressivity-and-incomplete-penetrance-in-a-large-family-with-non-classical-diamond-blackfan-anemia-associated-with-ribosomal-protein-l11-splicing-variant
#1
Colleen M Carlston, Zeinab A Afify, Janice C Palumbos, Heidi Bagley, Carlos Barbagelata, Whitney L Wooderchak-Donahue, Rong Mao, John C Carey
Diamond-Blackfan anemia (DBA) is a group of clinically and genetically heterogeneous bone marrow failure disorders with or without congenital anomalies. Variable expressivity and incomplete penetrance have been observed within affected families. Diamond-Blackfan anemia-7 (DBA7), caused by heterozygous mutations in ribosomal protein L11 (RPL11), accounts for approximately 5% of DBA. DBA7 is usually characterized by early-onset bone marrow failure often accompanied by congenital malformations, especially thumb defects...
July 25, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28740534/primary-hyperparathyroidism-associated-to-thrombocytopenia-an-issue-to-consider
#2
Steven De Keukeleire, Kristoff Muylle, Georgios Tsoumalis, Stefan Vermeulen, Dirk Vogelaers
Primary hyperparathyroidism (PHPT) is probably the most common endocrine disorder of the parathyroid glands, causing hypercalcemia. It is diagnosed often in persons with elevated serum calcium levels. However, hematological manifestations, such as thrombocytopenia are less known. In this case we describe the possible association of PHPT with reversible thrombocytopenia after parathyroidectomy. This hematological abnormality can be included in the spectrum of possible causes, including seemingly non-specific symptoms, in the decision tree towards surgical assessment...
January 2017: Clinical Cases in Mineral and Bone Metabolism
https://www.readbyqxmd.com/read/28740434/a-global-quantitative-survey-of-hemostatic-assessment-in-postpartum-hemorrhage-and-experience-with-associated-bleeding-disorders
#3
Andra H James, David L Cooper, Michael J Paidas
PURPOSE: Coagulopathy may be a serious complicating or contributing factor to postpartum hemorrhage (PPH), and should be promptly recognized to ensure proper bleeding management. This study aims to evaluate the approaches of obstetrician-gynecologists worldwide towards assessing massive PPH caused by underlying bleeding disorders. METHODS: A quantitative survey was completed by 302 obstetrician-gynecologists from 6 countries (the UK, France, Germany, Italy, Spain, and Japan)...
2017: International Journal of Women's Health
https://www.readbyqxmd.com/read/28732370/silibinin-an-old-drug-for-hematological-disorders
#4
REVIEW
Hai Zou, Xing-Xing Zhu, Guo-Bing Zhang, Yuan Ma, Yi Wu, Dong-Sheng Huang
Silibinin (silybin), a non-toxic natural polyphenolic flavonoid, is the principal and the most biologically active component of silymarin. It is efficient in the treatment of acute and chronic liver disorders caused by toxins, drug, alcohol, hepatitis, and gall bladder disorders. Further, in our previous studies, we explored the anti-cancer efficacy in common cancers, such as lung, prostatic, colon, breast, bladder, as well as, hepatocellular carcinoma. Interestingly, silibinin is still not solely limited to the treatment of these diseases...
July 11, 2017: Oncotarget
https://www.readbyqxmd.com/read/28731904/monoclonal-gammopathy-of-undetermined-significance-after-kidney-transplantation-single-center-experience
#5
Gaetano Alfano, Francesco Fontana, Elisabetta Colaci, Andrea Messerotti, Francesca Bettelli, Leonardo Potenza, Mario Luppi, Gianni Cappelli
BACKGROUND: Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic premalignant plasma cell disorder. Prevalence and clinical outcomes of MGUS in kidney transplant (KT) recipients have been previously reported in few studies with conflicting results. METHODS: We conducted a retrospective study in a population of 548 KT recipients transplanted between 1998 and 2015. RESULTS: Thirty-nine subjects (8.1%) developed MGUS after KT...
July 21, 2017: Transplantation
https://www.readbyqxmd.com/read/28729034/prolonged-renal-survival-in-light-chain-amyloidosis-speed-and-magnitude-of-light-chain-reduction-is-the-crucial-factor
#6
Tamer Rezk, Helen J Lachmann, Marianna Fontana, Sajitha Sachchithanantham, Shameem Mahmood, Aviva Petrie, Carol J Whelan, Jennifer H Pinney, Darren Foard, Thirusha Lane, Taryn Youngstein, Ashutosh D Wechalekar, Paul Bass, Philip N Hawkins, Julian D Gillmore
Renal involvement causing progressive chronic kidney disease (CKD) is present in 70% of patients with systemic Ig light-chain (AL) amyloidosis at diagnosis. Chemotherapy that substantially suppresses free light chain production is associated with improved patient survival, but its benefit in delaying the onset of renal replacement therapy among patients who present with established advanced CKD has not been studied. To evaluate this, we studied 1000 patients enrolled in the prospective UK AL amyloidosis chemotherapy study (ALchemy)...
July 17, 2017: Kidney International
https://www.readbyqxmd.com/read/28728162/realistic-lenalidomide-dose-adjustment-strategy-for-transplant-ineligible-elderly-patients-with-relapsed-refractory-multiple-myeloma-japanese-real-world-experience
#7
Aya Nakaya, Shinya Fujita, Atsushi Satake, Takahisa Nakanishi, Yoshiko Azuma, Yukie Tsubokura, Masaaki Hotta, Hideaki Yoshimura, Kazuyoshi Ishii, Tomoki Ito, Shosaku Nomura
Lenalidomide is an immunomodulatory drug administered orally in the treatment of multiple myeloma. Some elderly patients require a reduced lenalidomide dose because of comorbidities and/or adverse events. This study investigated the actual dose of lenalidomide in elderly patients, finding that most received reduced (5-10 mg) doses. The most common reasons for dose reduction were renal dysfunction (54% of patients), fatigue (grade ≥3; 20%), hematologic disorder (grade ≥3; 14%), and rash (grade ≥3; 9%)...
July 21, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28727131/evaluation-of-the-hematologic-system-as-a-marker-of-subclinical-inflammation-in-hyperemesis-gravidarum-a-case-control-study
#8
Fatma Beyazit, Filiz Halici Öztürk, Eren Pek, Mesut Abdülkerim Ünsal
OBJECTIVES: Current evidence suggests that subclinical inflammation plays a significant role in the development of hyperemesis gravidarum (HEG). Simple hematological markers, such as mean platelet volume (MPV), neutrophil-to-lymphocyte ratio (NLR), and platelet-to-lymphocyte ratio (PLR), have been shown to reflect inflammatory burden and disease activity in several disorders. This study aimed to determine the diagnostic value of these hematological parameters for HEG. MATERIAL AND METHODS: A total of 54 HEG patients and 58 age- and gestational-age-matched control subjects were studied...
2017: Ginekologia Polska
https://www.readbyqxmd.com/read/28725969/mastocytosis-from-a-molecular-point-of-view
#9
REVIEW
Daniel Elieh Ali Komi, Todd Rambasek, Stefan Wöhrl
Mast cells (MCs) are physiologically activated by binding of stem cell factor (SCF) to the extracellular domains of the Kit receptor. This binding increases the proliferation and prolongs the survival of normal mature MCs, as well as intensifies the release of mediators. In mastocytosis, somatic mutations of the coding Kit gene cause autocrine dysregulation and lead to constitutive KIT activation even in the absence of its ligand SCF. Clinical symptoms are caused by MC-mediator release and/or infiltration of MCs into tissues...
July 19, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28723802/tocilizumab-for-uncontrollable-systemic-inflammatory-response-syndrome-complicating-adult-onset-still-disease-case-report-and-review-of-literature
#10
Asami Masui-Ito, Ryuji Okamoto, Kaoru Ikejiri, Mika Fujimoto, Muneyoshi Tanimura, Shiro Nakamori, Tomohiro Murata, Eiji Ishikawa, Norikazu Yamada, Hiroshi Imai, Masaaki Ito
RATIONALE: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, fever spikes, arthralgia, and lymphadenopathy. AOSD usually has a good prognosis, but it can sometimes be fatal, especially when it is complicated by systemic inflammatory response syndrome (SIRS) and multiple organ failure. PATIENT CONCERNS: A previously healthy 26-year-old woman was referred to our hospital for persistent high fever and mild systemic edema...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28720126/a-small-molecule-cytokine-combination-enhances-hematopoietic-stem-cell-proliferation-via-inhibition-of-cell-differentiation
#11
Lan Wang, Xin Guan, Huihui Wang, Bin Shen, Yu Zhang, Zhihua Ren, Yupo Ma, Xinxin Ding, Yongping Jiang
BACKGROUND: Accumulated evidence supports the potent stimulating effects of multiple small molecules on the expansion of hematopoietic stem cells (HSCs) which are important for the therapy of various hematological disorders. Here, we report a novel, optimized formula, named the SC cocktail, which contains a combination of three such small molecules and four cytokines. METHODS: Small-molecule candidates were individually screened and then combined at their optimal concentration with the presence of cytokines to achieve maximum capacity for stimulating the human CD34(+) cell expansion ex vivo...
July 18, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/28719386/is-there-a-standard-of-care-for-transfusion-therapy-in-thalassemia
#12
Massimo Franchini, Gian L Forni, Giancarlo M Liumbruno
PURPOSE OF REVIEW: Thalassemia is the most common form of inherited anemia, characterized by variable clinical phenotypes. The purpose of this review is to summarize the transfusion support in thalassemia patients and the management of transfusion-related iron overload. RECENT FINDINGS: The most recent evidence on transfusion strategy and iron chelation therapy in thalassemia arising from clinical trials as well as from recommendation guidelines are critically discussed...
July 14, 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28718199/the-clec12a-receptor-marks-human-basophils-potential-implications-for-minimal-residual-disease-detection-in-myeloid-malignancies
#13
Marie Toft-Petersen, Anne Stidsholt Roug, Trine Plesner, Lene Ebbesen, Gordon D Brown, Line Nederby
BACKGROUND: The transmembrane receptor C-type lectin domain family 12, member A (CLEC12A) is known to be highly expressed on monocytes and neutrophils and is a reliable leukemia associated marker in acute myeloid leukemia. Consequently, detailed knowledge of the various normal cell types expressing this receptor is essential. We have observed CLEC12A to be expressed on CD45lowSSClowCD14-CD123+ basophils in peripheral blood (PB) and in the present study, we aimed at verifying this observation and further delineate the CD45lowSSClowCD14-CD123+CLEC12A+ subpopulation...
July 17, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/28716109/clinical-characteristics-and-outcomes-of-pseudomonas-aeruginosa-bacteremia-in-febrile-neutropenic-children-and-adolescents-with-the-impact-of-antibiotic-resistance-a-retrospective-study
#14
Hyo Sup Kim, Bo Kyoung Park, Seong Koo Kim, Seung Beom Han, Jae Wook Lee, Dong-Gun Lee, Nack-Gyun Chung, Bin Cho, Dae Chul Jeong, Jin Han Kang
BACKGROUND: Although the proportion of Pseudomonas aeruginosa infections has reduced after the introduction of antibiotics with anti-pseudomonal effects, P. aeruginosa bacteremia still causes high mortality in immunocompromised patients. This study determined the clinical characteristics and outcomes of P. aeruginosa bacteremia and the antibiotic susceptibilities of strains isolated from febrile neutropenic patients. METHODS: Thirty-one febrile neutropenic children and adolescents with underlying hematologic/oncologic disorders diagnosed with P...
July 17, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28706388/comparison-of-bone-marrow-aspiration-cytology-with-bone-marrow-trephine-biopsy-histopathology-an-observational-study
#15
Meenu Gilotra, Monika Gupta, Sunita Singh, Rajeev Sen
INTRODUCTION: Bone marrow examination is a useful investigative tool for the diagnosis of many hematological and nonhematological disorders. Bone marrow aspiration (BMA) provides information about the numerical and cytological features of marrow cells, whereas bone marrow trephine biopsies (BMB) provide excellent appreciation of spatial relationships between cells and of overall bone marrow structure. We conducted this study with the objective of comparing the accuracy of BMA with BMB in the diagnosis of various hematological disorders...
July 2017: Journal of Laboratory Physicians
https://www.readbyqxmd.com/read/28704210/factor-xiii-deficiency-in-south-of-tunisia
#16
Ines Maaloul, Moez Medhaffer, Nacim Louhichi, Imen Krichen, Sofien Alibi, Sana Kmiha, Hajer Aloulou, Faiza Fakhfakh, Moez Elloumi, Choumous Kallel, Mongia Hachicha
: Factor XIII deficiency is a rare autosomal recessive disorder of hemostasis characterized by a plasmatic factor XIII level less than 1% in homozygote and bleeding as of the youth. The aim of the study is to describe the clinical features and the outcome of the patients and to determine molecular characteristics. A retrospective study, was conducted on seven patients with factor XIII deficiency in the department of hematology and pediatrics, Hedi Chaker Hospital, Sfax, Tunisia during the period of 14 years (2001-2014)...
July 12, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28703446/paroxysmal-dyskinesia-in-border-terriers-clinical-epidemiological-and-genetic-investigations
#17
Q E M Stassen, L L E Koskinen, F G van Steenbeek, E H Seppälä, T S Jokinen, P G M Prins, H G J Bok, M M J M Zandvliet, M Vos-Loohuis, P A J Leegwater, H Lohi
BACKGROUND: In the last decade, a disorder characterized by episodes of involuntary movements and dystonia has been recognized in Border Terriers. OBJECTIVES: To define clinical features of paroxysmal dyskinesia (PD) in a large number of Border Terriers and to study the genetics of the disease. ANIMALS: 110 affected and 128 unaffected client-owned Border Terriers. METHODS: A questionnaire regarding clinical characteristics of PD was designed at Utrecht University and the University of Helsinki...
July 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28702158/hematologic-disorder-a-manifestation-of-helicobacter-pylori-infection
#18
Mohammad Zamani, Jila Masrour-Roudsari, Vahid Zamani
No abstract text is available yet for this article.
2017: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/28701183/ruxolitinib-found-to-cause-eyelash-growth-a-case-report
#19
Julia Song, Alice Song, Trisa Palmares, Michael Song, Harold Song
BACKGROUND: Hypereosinophilic syndrome is a hematologic disorder in which the eosinophils proliferate. Oral Janus kinase inhibitors are known to be effective treating hypereosinophilic syndrome. Janus kinase inhibitors have also demonstrated efficacy in alopecia. Madarosis is a condition in which the eyelashes are missing or absent and can been seen in alopecia patients. CASE PRESENTATION: We present the case of a 77-year-old Asian man who was diagnosed with hypereosinophilic syndrome, refractive to all medications except ruxolitinib...
July 12, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28699030/dna-damage-response-and-disorders-with-hematology-oncology-and-immunology
#20
EDITORIAL
Shuki Mizutani
No abstract text is available yet for this article.
July 11, 2017: International Journal of Hematology
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