keyword
MENU ▼
Read by QxMD icon Read
search

PNH

keyword
https://www.readbyqxmd.com/read/29395255/lessons-learned-from-bone-marrow-failure-in-systemic-lupus-erythematosus-case-reports-and-review-of-the-literature
#1
Erik Anderson, Bhakti Shah, Anne Davidson, Richard Furie
OBJECTIVE: In the present review, four new cases of bone marrow failure are presented and the potential contribution of systemic lupus erythematosus (SLE) is discussed. Furthermore, a comprehensive literature review of cases of autoimmune myelofibrosis (AIMF), aplastic anemia (AA), and paroxysmal nocturnal hemoglobinuria (PNH) with concurrent SLE aims to allow their direct comparison. Based on a clearer characterization of reported cases and our own experience, diagnostic and therapeutic strategies of these disorders in SLE are proposed based on lessons learned from the present and previous cases...
December 8, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29389084/special-issue-updated-iccs-escca-pnh-guidelines
#2
F Preffer
No abstract text is available yet for this article.
January 2018: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29383624/effects-of-eculizumab-treatment-on-quality-of-life-in-patients-with-paroxysmal-nocturnal-hemoglobinuria-in-japan
#3
Yasutaka Ueda, Naoshi Obara, Yuji Yonemura, Hideyoshi Noji, Masayoshi Masuko, Yoshinobu Seki, Katsuya Wada, Takahisa Matsuda, Hirozumi Akiyama, Takayuki Ikezoe, Shigeru Chiba, Yoshinobu Kanda, Tatsuya Kawaguchi, Tsutomu Shichishima, Hideki Nakakuma, Shinichiro Okamoto, Jun-Ichi Nishimura, Yuzuru Kanakura, Haruhiko Ninomiya
In paroxysmal nocturnal hemoglobinuria (PNH), various symptoms due to intravascular hemolysis exert a negative impact on patients' quality of life (QOL). To determine clinical factors related with improvements in QOL in PNH patients treated, we analyzed changes in QOL scales in PNH patients treated with eculizumab based on data collected from post-marketing surveillance in Japan. Summary statistics were obtained using figures from QOL scoring systems and laboratory values, and evaluated by t test. One-year administration of eculizumab improved the most QOL items in comparison with the baseline...
January 30, 2018: International Journal of Hematology
https://www.readbyqxmd.com/read/29381839/high-sensitivity-5-6-and-7-color-pnh-wbc-assays-for-both-canto-ii-and-navios-platforms
#4
D Robert Sutherland, Fernando Ortiz, Graeme Quest, Andrea Illingworth, Miroslav Benko, Rakesh Nayyar, Iuri Marinov
BACKGROUND: Paroxysmal Nocturnal Hemoglobinuria (PNH) is a rare acquired Hematopoietic Stem Cell disorder characterized by an inability to make glyco-phosphatidyl-inositol (GPI)-linked cell surface structures. Fluorescent proaerolysin (FLAER-Alexa488) is increasingly used to detect GPI-deficient WBCs by flow cytometry. However, FLAER is not available in all countries and is expensive to obtain in others. An earlier study to compare FLAER-based and non-FLAER assays confirmed very good agreement between the two tubes suggesting a cost effective simultaneous evaluation of PNH neutrophils and monocytes is possible without FLAER...
January 30, 2018: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29360268/the-monoclonal-anti-cd157-antibody-clone-sy11b5-used-for-high-sensitivity-detection-of-pnh-clones-on-wbcs-fails-to-detect-a-common-polymorphic-variant-encoded-by-bst-1
#5
Johanna Blaha, Klaus Schwarz, Claudia Fischer, Peter Schauwecker, Britta Höchsmann, Hubert Schrezenmeier, Markus Anliker
BACKGROUND: CD157, encoded by BST-1, has been described as a useful flow cytometric marker for the analysis of paroxysmal nocturnal hemoglobinuria (PNH) as it is a glycosylphosphatidylinositol (GPI)-linked molecule highly expressed on normal monocytes and neutrophils. We and others observed isolated CD157 signal dropouts during intended PNH analysis. We hypothesize that these negative populations occur due to an antibody failure. To investigate the reason for this finding, we compared two different anti-CD157 antibody clones for PNH analysis...
January 23, 2018: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29355143/value-of-cd16-cd66b-cd45-in-comparison-to-cd55-cd59-cd45-in-diagnosis-of-paroxysmal-nocturnal-haemoglobinuria-an-indian-experience
#6
Mrinalini Kotru, Rahul Sharma, Suman Kumar Pramanik, Abhishek Purohit, Gurmeet Singh, Avinash Kumar Singh, Deepti Muterja, Pravas Mishra, Tulika Seth, Seema Tyagi, Manoranjan Mahapatra, Hara Prasad Pati, Renu Saxena
BACKGROUND & OBJECTIVES: Diagnosis of paroxysmal nocturnal haemoglobinuria (PNH), a rare haematopoietic stem cell disorder, is challenging in patients with bone marrow failure (BMF) syndrome like aplastic anaemia (AA). This study was conducted with the aim to test the efficacy of the newly recommended markers viz. anti-CD16 and CD66b antibody over the existing anti-CD55 and CD59 antibody for PNH diagnosis in India. METHODS: This study was conducted on 193 suspected cases of PNH by flow cytometry using lyse wash technique to stain the granulocytes with CD16/CD66b and CD55/CD59...
September 2017: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/29327071/diseases-of-complement-dysregulation-an-overview
#7
REVIEW
Edwin K S Wong, David Kavanagh
Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation. Although complement overactivation is common to all, cell surface alternative pathway dysregulation (aHUS), fluid phase alternative pathway dysregulation (C3G), or terminal pathway dysregulation (PNH) predominates resulting in the very different phenotypes seen in these diseases. The mechanism underlying the dysregulation also varies with predominant acquired autoimmune (C3G), somatic mutations (PNH), or inherited germline mutations (aHUS) predisposing to disease...
January 11, 2018: Seminars in Immunopathology
https://www.readbyqxmd.com/read/29323198/paroxysmal-nocturnal-hemoglobinuria-pnh-brain-mri-ischemic-lesions-in-neurologically-asymtomatic-patients
#8
Wilma Barcellini, Elisa Scola, Silvia Lanfranconi, Marika Grottaroli, Francesca Binda, Bruno Fattizzo, Anna Zaninoni, Gloria Valcamonica, Claudia Maria Cinnante, Carla Boschetti, Massimiliano Buoli, Carlo Alfredo Altamura, Nereo Bresolin, Fabio Triulzi, Alberto Zanella, Agostino Cortelezzi
This study investigated for the first time brain ischemic involvement in 19 consecutive neurologically asymptomatic PNH patients by non-enhanced cerebral MRI, and by intracranial arterial and venous angio-MRI. Eleven cases (58%, 7 aged <65) showed pathological findings: 9 white matter (WM) abnormalities related to chronic ischemic small vessel disease, 2 a focal abnormality >5 mm, and 5 cases a score >4 by the age-related white matter changes (ARWMC) scale. Compared with age and sex-matched controls (1:2 ratio), patients showed an increased frequency of periventricular WM vascular degeneration (32% versus 5...
January 11, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29314145/toward-complement-inhibition-2-0-next-generation-anticomplement-agents-for-paroxysmal-nocturnal-hemoglobinuria
#9
Antonio M Risitano, Serena Marotta
Therapeutic complement inhibition by eculizumab has revolutionized the treatment of paroxysmal nocturnal hemoglobinuria (PNH) with a major impact on its natural history. Nevertheless, emerging unmet clinical needs may benefit from the development of novel complement inhibitors. Novel strategies of complement inhibition exploit different agents targeting C5, as well as compound intercepting the complement cascade at the level of its key component C3, or even upstream at the level of components involved in complement alternative pathway initiation...
January 4, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29306655/comparative-study-of-porcine-anti-human-lymphocyte-immunoglobulin-and-rabbit-anti-human-thymocyte-immunoglobulin-as-a-first-line-treatment-of-acquired-severe-aplastic-anemia
#10
Miao Chen, Chao Liu, Xinhui Qiao, Daobin Zhou, Junling Zhuang, Bing Han
Porcine anti-human lymphocyte immunoglobulin (pALG) and rabbit anti-human thymocyte immunoglobulin (rATG) are the only two ATGs for severe aplastic anemia (SAA) treatment in China. 148 treatment-naïve SAA patients who received ATG combined with cyclosporine A (CsA) therapy were analysed retrospectively. The patients were divided into a pALG group (n = 114) and a rATG group (n = 34). After three months, the pALG and rATG groups had an overall response (OR) of 65.8% and 44.1%, respectively (P = 0...
January 2, 2018: Leukemia Research
https://www.readbyqxmd.com/read/29296844/potential-impact-of-complement-regulator-deficiencies-on-hemolytic-reactions-due-to-minor-abo-mismatched-transfusions
#11
Priyanka Pandey, Waseem Q Anani, Jerome L Gottschall, Gregory A Denomme
Minor ABO-mismatched transfusions are a common occurrence, although infrequent transfusion reactions occur. We sought to investigate the regulation of complement C3 activation induced by anti-A. In vitro complement C3 activation was observed with 10 of 30 group O samples and correlated with immunoglobulin M (IgM) anti-A titers. We developed an in vitro paroxysmal nocturnal hemoglobinuria (PNH) model of hemolysis in which group A1 red blood cells (RBCs) were chemically treated with 2-aminoethylisothiouronium (AET) to alter regulators of complement C3 activation...
October 24, 2017: Blood Advances
https://www.readbyqxmd.com/read/29251828/iccs-escca-consensus-guidelines-for-the-flow-cytometric-testing-for-patients-with-suspected-paroxysmal-nocturnal-hemoglobinuria-pnh-validation-and-quality-assurance-part-4
#12
Oldaker Teri, Liam Whitby, Maryam Saber, Jeannine Holden, Wallace Paul K, Virginia Litwin
Over the past six years, a diverse group of stakeholders have put forth recommendations regarding the analytical validation of flow cytometric methods and described in detail the differences between cell-based and traditional soluble analyte assay validations. This manuscript is based on these general recommendations as well as the published experience of experts in the area of PNH testing. The goal is to provide practical assay-specific guidelines for the validation of high-sensitivity flow cytometric PNH assays...
December 18, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29249231/hemoglobinuric-acute-kidney-injury-in-a-patient-with-a-kinked-prosthetic-aortic-graft%C3%A2
#13
Hema V Sheelvanth, Khaleel A Sayeed, William A Whittier
Acute kidney injury (AKI) due to intravascular hemolysis has been well described in paroxysmal nocturnal hemoglobinuria (PNH) and in cardiac valvular dysfunction. However, reported cases of hemoglobinuric AKI from prosthetic aortic grafts are sparse. We present a case of microangiopathic hemolytic anemia and hemoglobinuric AKI presenting 9 days after repair of an ascending aortic dissection with a prosthetic graft. After an unremarkable recovery following the initial procedure, the patient presented on postoperative day 9 with dyspnea, fatigue, and cola-colored urine...
December 18, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29236353/iccs-escca-consensus-guidelines-for-the-high-sensitivity-flow-cytometric-detection-of-paroxysmal-nocturnal-hemoglobinuria-pnh-part-2-assay-optimization-and-reagent-selection
#14
EDITORIAL
D Robert Sutherland, Andrea Illingworth, Iuri Marinov, Fernando Ortiz, John Andreasen, Dan Payne, Paul K Wallace, Michael Keeney
Since publication in 2010 of the International Clinical Cytometry Society (ICCS) Consensus Guidelines for detection of Paroxysmal nocturnal hemoglobinuria (PNH) by flow cytometery, a great deal of work has been performed to develop, optimize and validate a number of high-sensitivity assays to detect PNH phenotypes in both Red Blood Cells (RBCs) and White Blood Cells (WBCs, neutrophils and monocytes). This section (Part 2) of the updated ICCS PNH Consensus Guidelines will focus on specific instrument setup for these PNH assays, the identification and proper testing of appropriate antibody conjugates and combinations therof, and basic assay design...
December 13, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29236352/iccs-escca-consensus-guidelines-for-the-clinical-utility-of-testing-for-gpi-anchor-deficient-clones-in-paroxysmal-nocturnal-hemoglobinuria-pnh-and-other-bone-marrow-disorders-part-1
#15
Amy E Dezern, Michael J Borowitz
Paroxysmal nocturnal hemoglobinuria (PNH) arises as a consequence of the non-malignant clonal expansion of one or more hematopoietic stem cells with an acquired somatic mutation of the PIGA gene.(1) Progeny of affected stem cells are deficient in glycosyl phosphatidylinositol-anchored proteins (GPI-APs). This deficiency is readily detected by flow cytometry. Though this seems straightforward, the clinical utility of this testing requires that the ordering clinician understand not only the characteristics of the test, but also the biology of the underlying disease, and the clinical and laboratory manifestations in the individual patient...
December 13, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29236350/iccs-escca-consensus-guidelines-to-detect-gpi-deficient-cells-in-paroxysmal-nocturnal-hemoglobinuria-pnh-and-related-disorders-part-3-data-analysis-reporting-and-case-studies
#16
Andrea Illingworth, Iuri Marinov, D Robert Sutherland, Orianne Wagner Ballon, Luigi DelVecchio
Over the past several years, a diverse group of physicians and other laboratory scientists have developed various recommendations and guidelines regarding best practices for PNH testing. This manuscript is based on these previous recommendations as well as various other relevant publications of experts in the area of PNH testing. The goal is to provide flow cytometry laboratories with an updated consensus approach to analysis and reporting of PNH results and to address the most common analytical challenges for accurate reporting of this rare disease...
December 13, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29214126/pharmacologic-complement-inhibition-in-clinical-transplantation
#17
REVIEW
Vasishta S Tatapudi, Robert A Montgomery
Purpose of Review: Over the past two decades, significant strides made in our understanding of the etiology of antibody-mediated rejection (AMR) in transplantation have put the complement system in the spotlight. Here, we review recent progress made in the field of pharmacologic complement inhibition in clinical transplantation and aim to understand the impact of this therapeutic approach on outcomes in transplant recipients. Recent Findings: Encouraged by the success of agents targeting the complement cascade in disorders of unrestrained complement activation like paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), investigators are testing the safety and efficacy of pharmacologic complement blockade in mitigating allograft injury in conditions ranging from AMR to recurrent post-transplant aHUS, C3 glomerulopathies and antiphospholipid anti-body syndrome (APS)...
2017: Current Transplantation Reports
https://www.readbyqxmd.com/read/29212979/-clinical-features-and-quality-of-life-assessment-in-japanese-pnh-patients-enrolled-in-the-international-pnh-registry
#18
Masatoshi Sakurai, Shinichiro Okamoto
The aim of the present study was to clarify the clinical characteristics and quality of life (QOL) of Japanese patients (N=116) enrolled in the International PNH Registry, compared to the whole registry cohort (N=3,457), censored as of March 2015. The proportion of patients treated with eculizumab was comparable between the two cohorts; the Japanese cohort showed higher levels of lactate dehydrogenase and lower hemoglobin values at baseline. Compared to the whole cohort, the Japanese cohort had a greater incidence of bone marrow failure (67...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29190926/polymorphism-of-the-abo-gene-associate-with-thrombosis-risk-in-patients-with-paroxysmal-nocturnal-hemoglobinuria
#19
Zhangbiao Long, Yali Du, Hongmin Li, Bing Han
Thrombosis is one of the most common causes of mortality in Paroxysmal nocturnal hemoglobinuria (PNH), but the predisposing factors for thrombosis are yet to be defined. In this study, we outline the clinical characters and the susceptible genes which lead to thrombotic formation in 104 patients with PNH. The results displayed that the genotypes with minor alleles of rs495828 or rs2519093 in the ABO gene were associated with high risk to thrombus formation (OR 5.95, 95% CI 1.90-18.65 and OR 6.3, 95% CI 2.01-19...
November 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29187158/associations-of-intimate-partner-violence-with-postnatal-health-practices-in-bihar-india
#20
Sabrina C Boyce, Lotus McDougal, Jay G Silverman, Yamini Atmavilas, Diva Dhar, Katherine Hay, Anita Raj
BACKGROUND: Reducing neonatal mortality is a global priority, and improvements in postnatal health (PNH) practices in India are needed to do so. Intimate partner violence (IPV) may be associated with PNH practices, but little research has assessed this relationship. METHODS: A cross-sectional analysis of data from a representative household sample of mothers of neonates 0-11 months old in Bihar, India was conducted. The relationship between lifetime IPV experience (physical violence only, sexual violence only, or both physical and sexual violence) and PNH practices [clean cord care, kangaroo mother care, early initiation of breastfeeding (EIBF), delayed bathing, receipt of a postnatal care visit, exclusive breastfeeding, and current post-partum contraceptive use] was assessed using multivariate logistic regression...
November 29, 2017: BMC Pregnancy and Childbirth
keyword
keyword
1152
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"