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https://www.readbyqxmd.com/read/28331130/cytopenias-and-clonal-expansion-of-gamma-delta-t-cells-in-a-patient-with-anaplasmosis-a-potential-diagnostic-pitfall
#1
Daniel Marko, Anamarija M Perry, Arjuna Ponnampalam, Michel R Nasr
Human granulocytic anaplasmosis is a rare, tick-borne infectious disease caused by Anaplasma phagocytophilum. Herein, we report a rare case of human granulocytic anaplasmosis associated with cytopenias and clonal expansion of gamma/delta T-cells in the bone marrow. A 77-year old man presented multiple times to the emergency department complaining of muscle weakness. Complete blood count detected cytopenias and peripheral blood smear showed pseudo Pelger-Huet neutrophils. These findings prompted bone marrow evaluation with ancillary studies including flow cytometry, karyotyping and T-cell rearrangement studies...
2017: Journal of Clinical and Experimental Hematopathology: JCEH
https://www.readbyqxmd.com/read/28329668/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-in-us-adults
#2
Derek Y Hsu
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening disorders. The incidences, mortality and cost of SJS and TEN in US adults are not well-characterized. We analyzed data from the 2009-2012 Nationwide Inpatient Sample, containing a representative 20% sample of all hospitalizations in the US. SJS, SJS/TEN and TEN were identified by a validated algorithm using ICD-9- CM codes. The mean incidences of SJS, SJS/TEN and TEN were 9.3, 1.6, and 1.9 per million people per year, respectively...
September 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28328595/hemorrhage-and-infarction-of-the-conjunctiva-and-orbit-in-essential-thrombocythemia
#3
Jaehoon Na, Soo Youn Choi, Sehyun Baek, Hwa Lee
An 82-year-old woman who had a history of essential thrombocythemia presented with ocular pain, bleeding, and decreased visual acuity of the left eye. Orbital computed tomography revealed a relatively well-defined homogenous mass-like lesion in the left subconjunctival and intraconal space. Conjunctival biopsy showed acute inflammation with necrosis, vascular ectasia with thrombosis and hemorrhage. After the treatment with hydroxyurea and anagrelide, her symptom and lesion were markedly improved. Hematologic disorders like essential thrombocythemia should be considered in patients with severe spontaneous bleeding around the eye...
March 21, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28327806/invasive-fungal-infection-in-patients-with-hematologic-disorders-in-a-brazilian-tertiary-care-hospital
#4
Milton Camplesi, Hildene Meneses Silva, Adriano Moraes Arantes, Carolina Rodrigues Costa, Fábio Silvestre Ataides, Thaisa Cristina Silva, Maysa de Paula Costa Dos Reis, Maria do Rosário Rodrigues Silva
INTRODUCTION: Invasive fungal infections (IFIs) are an important complication in immunocompromised individuals, particularly neutropenic patients with hematological malignancies. In this study, we aimed to verify the epidemiology and diagnosis of IFIs in patients with hematologic problems at a tertiary hospital in Goiânia-GO, Brazil. METHODS: Data from 117 patients, involving 19 cases of IFIs, were collected. The collected data included diagnosis methods, demographics, clinical characteristics, and in vitro susceptibility to different antifungal agents...
January 2017: Revista da Sociedade Brasileira de Medicina Tropical
https://www.readbyqxmd.com/read/28326424/total-knee-arthroplasty-in-a-patient-with-hypofibrinogenemia
#5
Christopher R Nacca, Kalpit N Shah, Jeremy N Truntzer, Lee E Rubin
Patients with afibrinogenemia or hypofibrinogenemia present a unique challenge to the arthroplasty surgeon as fibrinogen is a key contributor to hemostasis. Patients with these disorders are known to have a higher risk for postsurgical bleeding complications. We present the case of a patient with hypofibrinogenemia who underwent an elective total knee arthroplasty. Our colleagues in hematology-oncology guided us initially to achieve and maintain appropriate fibrinogen levels in the early perioperative period...
December 2016: Arthroplasty Today
https://www.readbyqxmd.com/read/28325300/one-step-biallelic-and-scarless-correction-of-a-%C3%AE-thalassemia-mutation-in-patient-specific-ipscs-without-drug-selection
#6
Yali Liu, Yi Yang, Xiangjin Kang, Bin Lin, Qian Yu, Bing Song, Ge Gao, Yaoyong Chen, Xiaofang Sun, Xiaoping Li, Lei Bu, Yong Fan
Monogenic disorders (MGDs), which are caused by single gene mutations, have a serious effect on human health. Among these, β-thalassemia (β-thal) represents one of the most common hereditary hematological diseases caused by mutations in the human hemoglobin β (HBB) gene. The technologies of induced pluripotent stem cells (iPSCs) and genetic correction provide insights into the treatments for MGDs, including β-thal. However, traditional approaches for correcting mutations have a low efficiency and leave a residual footprint, which leads to some safety concerns in clinical applications...
March 17, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28322165/anti-inflammatory-and-anti-arthritic-activity-of-different-milk-based-formulation-of-curcumin-in-rat-model
#7
Sumeet Gupta
Inflammation is the key mediator for arthritis. Plant based products are most useful for treating various disorders, but at the same time drug absorbtion is utmost important for effective therapy. The aim of the study was to find out the therapeutic concern in pharmacokinetic and pharmacodynamic parameters in an arthritis induced rat model. Carrageenan and complete Freud's adjuvant, both were used for an arthritis induction as an animal model. Five different formulations of curcumin were prepared in single toned milk, double toned milk, full fat milk, full fat milk with ghee and in an aqueous suspension...
March 20, 2017: Current Drug Delivery
https://www.readbyqxmd.com/read/28321281/effects-of-maternal-hypertension-on-the-neonatal-haemogram-in-southern-nigeria-a-case-control-study
#8
Helen C Okoye, Lisa I Eweputanna, Kaladada I Korubo, Oseikhuemen A Ejele
BACKGROUND: Hypertension in pregnancy is a leading cause of maternal and neonatal morbidity and mortality. This study aimed to compare the hematological parameters in neonates of hypertensive mothers with those of normotensive mothers, and also to compare the incidence of polycythaemia, neutropenia and thrombocytopenia in both groups. METHODS: This was a hospital-based case control study. Three milliliters of cord blood from neonates of women with hypertension in pregnancy and those of normotensive pregnant women were sampled for haemogram parameters using a 3-part autoanalyser...
December 2016: Malawi Medical Journal: the Journal of Medical Association of Malawi
https://www.readbyqxmd.com/read/28319054/association-of-attention-deficit-hyperactivity-disorder-and-autism-spectrum-disorders-with-mean-platelet-volume-and-vitamin-d
#9
Mesut Garipardic, Murat Doğan, Keziban Asli Bala, Tuba Mutluer, Sultan Kaba, Oktay Aslan, Lokman Üstyol
BACKGROUND The purpose of this study was to assess the values of the mean platelet volume (MPV) in children with attention deficit hyperactivity disorder (ADHD) and with autism spectrum disorders (ASDs) to determine the risk of cardiovascular disease in these 2 disorder groups. MATERIAL AND METHODS The study included a total of 79 patients with ADHD or ASDs and controls in the Van region of Turkey. The control group included subjects of matching age and sex with no ADHD, ASDs, or chronic disease and taking no vitamins...
March 20, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28316434/comparison-of-deferasirox-and-deferoxamine-effects-on-iron-overload-and-immunological-changes-in-patients-with-blood-transfusion-dependent-%C3%AE-thalassemia
#10
Hayder M Al-Kuraishy, Ali I Al-Gareeb
INTRODUCTION: Beta-thalassemias are a cluster of inherited (autosomal recessive) hematological disorders prevalent in the Mediterranean area due to defects in synthesis of β chains of hemoglobin. The aim of present study was to compare the effects of deferasirox and deferoxamine on iron overload and immunological changes in patients with blood transfusion-dependent β-thalassemia major and intermedia. PATIENTS AND METHODS: This study involved 64 patients with known cases of β-thalassemia major or intermedia that has been treated with blood transfusion and iron chelators...
January 2017: Asian Journal of Transfusion Science
https://www.readbyqxmd.com/read/28315032/novel-indications-for-fecal-microbial-transplantation-update-and-review-of-the-literature
#11
REVIEW
Nathaniel Aviv Cohen, Nitsan Maharshak
BACKGROUND AND AIMS: Fecal microbial transplantation (FMT) is an established successful treatment modality for recurrent Clostridium difficile infection (CDI). The safety profile and potential therapeutic advantages of FMT for diseases associated with dysbiosis and immune dysfunction have led to many publications, mainly case series, and while many studies and reviews have been published on the use of FMT for inflammatory bowel disease (IBD), its potential use for other disease conditions has not been thoroughly reviewed...
March 17, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28300669/enhanced-plasma-protein-carbonylation-in-patients-with-myelodysplastic-syndromes
#12
Alžběta Hlaváčková, Jana Štikarová, Kristýna Pimková, Leona Chrastinová, Pavel Májek, Roman Kotlín, Jaroslav Čermák, Jiří Suttnar, Jan Evangelista Dyr
Myelodysplastic syndromes (MDS) represent a heterogeneous group of pre-leukemic disorders, characterized by ineffective hematopoiesis and the abnormal blood cell development of one or more lineages. Oxidative stress, as an important factor in the carcinogenesis of onco-hematological diseases, is also one of the known factors involved in the pathogenesis of MDS. An increase of reactive oxygen species (ROS) may lead to the oxidation of DNA, lipids, and proteins, thereby causing cell damage. Protein carbonylation caused by ROS is defined as an irreversible post-translational oxidative modification of amino acid side chains, and could play an important role in signaling processes...
March 12, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28300447/sweet-syndrome
#13
Jose Ricardo Casarin Costa, Anangelica Rodrigues Virgens, Luisa de Oliveira Mestre, Natasha Favoretto Dias, Luciana Paula Samorano, Neusa Yuriko Sakai Valente, Cyro Festa Neto
BACKGROUND: Sweet syndrome (SS) is an infrequent skin disease characterised by sudden onset of fever, leukocytosis, neutrophilia, and tender erythematous plaques infiltrated by neutrophils. Multiple conditions have been associated with this syndrome. OBJECTIVES: The aim of this study was to evaluate the clinical, epidemiological, laboratory, and histopathological findings and associations of patients with SS. METHODS: We conducted a retrospective study of 83 patients with SS followed between January 1, 2006, and January 31, 2015...
February 1, 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28299663/runx1-and-cbf%C3%AE-mutations-and-activities-of-their-wild-type-alleles-in-aml
#14
R Katherine Hyde, Paul Liu, Alan D Friedman
Mutations in RUNX1 and CBFB have long been recognized as important in hematological malignancies. Point mutations and deletions of RUNX1 are frequently found in myelodysplastic syndrome, myeloproliferative disease, and acute myeloid leukemia. Germline mutations in RUNX1 are associated with familial platelet disorder with predisposition to AML. In addition, as will be discussed in other chapters, both RUNX1 and CBFB are involved in recurrent chromosomal rearrangements in leukemia. More recently, roles for the non-mutated RUNX1 and CBFB genes have been identified in multiple leukemia subtypes...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28299658/clinical-relevance-of-runx1-and-cbfb-alterations-in-acute-myeloid-leukemia-and-other-hematological-disorders
#15
Klaus H Metzeler, Clara D Bloomfield
The translocation t(8;21), leading to a fusion between the RUNX1 gene and the RUNX1T1 locus, was the first chromosomal translocation identified in cancer. Since the first description of this balanced rearrangement in a patient with acute myeloid leukemia (AML) in 1973, RUNX1 translocations and point mutations have been found in various myeloid and lymphoid neoplasms. In this chapter, we summarize the currently available data on the clinical relevance of core binding factor gene alterations in hematological disorders...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28299211/epistaxis-as-a-common-presenting-symptom-of-glanzmann-s-thrombasthenia-a-rare-qualitative-platelet-disorder-illustrative-case-examples
#16
Michael Recht, Meera Chitlur, Derek Lam, Syana Sarnaik, Madhvi Rajpurkar, David L Cooper, Sriya Gunawardena
Children often present to emergency departments (EDs) with uncontrollable nose bleeding. Although usually due to benign etiologies, epistaxis may be the presenting symptom of an inherited bleeding disorder. Whereas most bleeding disorders are detected through standard hematologic assessments, diagnosing rare platelet function disorders may be challenging. Here we present two case reports and review diagnostic and management challenges of platelet function disorders with a focus on Glanzmann's thrombasthenia (GT)...
2017: Case Reports in Emergency Medicine
https://www.readbyqxmd.com/read/28299210/radiographic-enlargement-of-mandibular-canal-as-an-extranodal-primary-non-hodgkin-s-lymphoma-early-sign-in-an-asymptomatic-patient
#17
Luciana Munhoz, Felipe Pereira Marcos Marsan, Emiko Saito Arita
Non-Hodgkin's lymphoma (NHL) is a lymphoproliferative disorder, from a subgroup of heterogeneous hematologic malignancies; the term "extranodal" refers to malignant involvement of tissues other than lymph nodes, tonsils, spleen, pharyngeal lymphatic ring, or thymus. Only 0.6% of all NHL are at mandible alone, and it may involve the inferior alveolar canal. We describe a case of bilateral enlargement of the mandibular canal without symptomatology, which was shown in a panoramic radiograph and cone beam computed tomography in a rehabilitation routine exam, as an early sign of primary extranodal NHL...
2017: Case Reports in Dentistry
https://www.readbyqxmd.com/read/28297629/genomics-of-myeloproliferative-neoplasms
#18
Katerina Zoi, Nicholas C P Cross
Myeloproliferative neoplasms (MPNs) are a group of related clonal hematologic disorders characterized by excess accumulation of one or more myeloid cell lineages and a tendency to transform to acute myeloid leukemia. Deregulated JAK2 signaling has emerged as the central phenotypic driver of BCR -ABL1-negative MPNs and a unifying therapeutic target. In addition, MPNs show unexpected layers of genetic complexity, with multiple abnormalities associated with disease progression, interactions between inherited factors and phenotype driver mutations, and effects related to the order in which mutations are acquired...
March 20, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28295968/nosocomial-bk-polyomavirus-infection-causing-hemorrhagic-cystitis-among-patients-with-hematological-malignancies-after-hematopoietic-stem-cell-transplantation
#19
Jun Kato, Takehiko Mori, Tetsuro Suzuki, Masahiko Ito, Tian-Cheng Li, Masatoshi Sakurai, Yusuke Yamane, Rie Yamazaki, Yuya Koda, Takaaki Toyama, Naoki Hasegwa, Shinichiro Okamoto
BK polyomavirus (BKPyV) is recognized as a pathogen that causes diseases such as hemorrhagic cystitis and nephritis after allogeneic hematopoietic stem cell transplantation (HSCT) or renal transplantation. BKPYV-associated disease is thought to occur through reactivation under immunosuppression. However, the possibility of its nosocomial transmission and the clinical significance of such transmission have not been elucidated. During a 6-month period, 9 adult patients (median age: 47 years) with hematological disorders who were treated with HSCT (N=7) or chemotherapy (N=2) in a single hematology department developed hemorrhagic cystitis due to BKPYV infection...
March 14, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28293461/a-rare-cause-of-cytopenia-in-a-patient-with-systemic-lupus-erythematosus-autoimmune-myelofibrosis
#20
Döndü Üsküdar Cansu, Hava Üsküdar Teke, Cengiz Korkmaz
Hematological abnormalities are very common in the course of systemic lupus erythematosus (SLE). Myelofibrosis is a bone marrow disorder in which there is excessive fibrous tissue formation in the bone marrow. Various benign and malignant disorders can cause or be associated with a diffuse increase in the bone marrow reticular tissue. Some diseases such as infections, neoplasms, and autoimmune diseases may also induce bone marrow fibrosis (secondary myelofibrosis). Cytopenia from autoimmune myelofibrosis (AIMF) in SLE is a rare condition...
March 2017: European Journal of Rheumatology
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