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https://www.readbyqxmd.com/read/29677388/engulfment-of-hb-activated-platelets-differentiates-monocytes-into-pro-inflammatory-macrophages-in-pnh-patients
#1
Rashi Singhal, Sheetal Chawla, Harish Batra, Sameer Gupta, Amrita Ojha, Deepak K Rathore, Tulika Seth, Prasenjit Guchhait
The distinct response shown by different phenotypes of macrophages and monocytes under various clinical conditions have put the heterogeneity of these cells into focus of investigation for several diseases. Recently we have described that after engulfing Hb-activated platelets, classical monocytes differentiated into pro-inflammatory phenotypes, which were abundant in the circulation of PNH and SCD patients. Our current study shows that upon engulfment of Hb-activated platelets, monocytes differentiate into M1-macrophages under M1-polarization stimulus (GM-CSF, IFN-γ+LPS)...
April 20, 2018: European Journal of Immunology
https://www.readbyqxmd.com/read/29649283/design-and-preclinical-characterization-of-alxn1210-a-novel-anti-c5-antibody-with-extended-duration-of-action
#2
Douglas Sheridan, Zhao-Xue Yu, Yuchun Zhang, Rekha Patel, Fang Sun, Melissa A Lasaro, Keith Bouchard, Bruce Andrien, Andre Marozsan, Yi Wang, Paul Tamburini
Eculizumab, a monoclonal antibody (mAb) directed against complement protein C5, is considered to be the current standard of care for patients with paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome. This study describes the generation and preclinical attributes of ALXN1210, a new long-acting anti-C5 mAb, obtained through select modifications to eculizumab to both largely abolish target-mediated drug disposition (TMDD) and increase recycling efficiency via the neonatal Fc receptor (FcRn)...
2018: PloS One
https://www.readbyqxmd.com/read/29622874/utility-of-cd157-as-a-common-leukocytes-marker-for-paroxysmal-nocturnal-hemoglobinuria-screening-in-a-single-tube-five-color-combination
#3
Khaliqur Rahman, Ruchi Gupta, Shivangi Harankhedkar, Tanvi Gupta, Manoj K Sarkar, Soniya Nityanand
To study the utility and advantage of CD157 in the paroxysmal nocturnal hemoglobinuria (PNH) screening along with its ability to replace CD24 and CD14. This was a confirmatory study to analyse the role and advantage of CD157 in a single tube five color combination to identify the PNH clones. A serial tenfold dilution experiments was carried out for sensitivity assessment. Reproducibility was checked in the intra-assay and inter-assay experiments. The results obtained with CD157 based assay were compared with the routinely used single tube six color CD24/CD14 based assay...
April 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29599976/high-sensitivity-8-color-flow-cytometry-assay-for-paroxysmal-nocturnal-hemoglobinuria-granulocyte-and-monocyte-detections
#4
Ray Chun-Fai Chan, Richard H Leung, Albert Posadas, Thomas S Lorey, Allison J Shaw
Flow cytometry is the gold standard in diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) by detecting the absence of glycol-phosphatidyl inositol (GPI)-linked protein expression on granulocyte and monocyte surfaces. However, the current assays are not optimized and require improvement, particularly in reducing background fluorescence and optimizing sensitivity and specificity. With more fluorochromes available and with advances in instrument engineering, rare populations may be identified with high sensitivity...
March 2018: Biomedical Reports
https://www.readbyqxmd.com/read/29551496/cr1-gene-polymorphisms-in-chinese-patients-with-paroxysmal-nocturnal-hemoglobinuria
#5
Zhangbiao Long, Yali Du, Hongmin Li, Bing Han
Patients with paroxysmal nocturnal hemoglobinuria (PNH) who have minor allele of the complement receptor 1 (CR1) gene, displayed more sub-optimal responder to eculizumab compared with major allele. To investigate polymorphism of the CR1 gene in Chinese patients with PNH and its correlation with clinical features and the potential impact on eculizumab efficiency, we genotyped CR1 rs2274567, rs3811381 and the intron 27 Hind III restriction fragment length polymorphism in 95patients with PNH and 96 controls. The results indicated that the genotypes of CR1 rs2274567, rs3811381 and the intron 27 Hind III in PNH patients and controls both consist with Hardy-Weinberg equilibrium...
March 15, 2018: Gene
https://www.readbyqxmd.com/read/29551047/-allogeneic-peripheral-blood-stem-cell-transplantation-for-treatment-of-aa-pnh-syndrome-a-case-report
#6
H Xue, Y C Hu, F Gao, X Y Li, S Q Feng
No abstract text is available yet for this article.
January 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29532535/screening-and-diagnostic-clinical-algorithm-for-paroxysmal-nocturnal-hemoglobinuria-expert-consensus
#7
Alexander Röth, Jaroslaw Maciejewski, Jun-Ichi Nishimura, Deepak Jain, Jeffrey I Weitz
OBJECTIVE: Paroxysmal nocturnal hemoglobinuria (PNH) is a severe, life-threatening disorder for which early diagnosis is essential. However, given the rarity of the disease and non-specificity of symptoms, correct diagnosis may be delayed or missed. While various hematologic guidelines note common signs and symptoms associated with PNH, international expert consensus based on real-world clinical experience and an actionable algorithm for non-specialists to facilitate screening and diagnosis are lacking...
March 12, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29527312/maintenance-of-renal-function-in-a-patient-with-a-history-of-acute-paroxysmal-nocturnal-hemoglobinuria-associated-kidney-injury
#8
Francesca Re, Ilenia Manfra, Filomena Russo, Caterina Plenteda, Angelica Spolzino, Elena Follini, Maria Gullo, Claudia Romano, Maria Cristina Baroni, Franco Aversa
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, life-threatening blood disorder characterized by intravascular hemolysis, thrombosis and bone marrow failure. Acute kidney injury, including acute renal failure, have been reported in patients with PNH. We report the case of a 36-year-old male patient with PNH who developed acute kidney injury following an infection of undetermined diagnosis. Although hemolysis was initially controlled and renal function stabilized following packed red blood cell transfusion and empirical levofloxacin and prednisone, he later experienced recurrent episodes of hemolysis and hematuria requiring monthly red blood cell support...
March 2018: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/29504353/a-case-of-thrombosis-due-to-paroxysmal-nocturnal-haemoglobinuria-presenting-at-an-early-age
#9
Qurrat Ul Ain, Huzaifa Saleem, Sarwat Iqbal, Rabia Ghayas
Paroxysmal Nocturnal Haemoglobinuria (PNH) is an acquired, rare life-threatening disorder characterised by compliment mediated hemolytic anemia, thrombosis and impaired bone marrow function. It occasionally presents in childhood or adolescence. This is a case of a 14-year old female presented with complaints of shortness of breath, palpitation and abdominal pain whose laboratory test results were consistent with Coomb's test negative haemolytic anaemia. Contrast enhanced Computed Tomography Scan (CT scan) of abdomen revealed splanchnic circulation thrombosis as well as partially occluding thrombus in the inferior vena cava...
January 2018: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/29486674/renal-involvement-in-paroxysmal-nocturnal-hemoglobinuria-an-update-on-clinical-features-pathophysiology-and-treatment
#10
Styliani I Kokoris, Eleni Gavriilaki, Aggeliki Miari, Αnthi Travlou, Elias Kyriakou, Achilles Anagnostopoulos, Elissavet Grouzi
OBJECTIVES: The present review summarizes the available knowledge regarding acute and chronic kidney dysfunction in patients with paroxysmal nocturnal hemoglobinuria (PNH) focusing on its clinical features, pathophysiology and treatment. METHODS: A thorough PubMed search was performed using as main keywords: 'paroxysmal nocturnal hemoglobinuria', 'acute kidney injury', 'chronic kidney disease' and 'eculizumab'. RESULTS: PNH's etiopathogenesis is based on acquired mutations that lead to the reduction or absence of CD55 and CD59 complement regulators, which are responsible for some of the disease's major clinical features, like intravascular hemolysis, cytopenias and thrombosis...
February 28, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29484054/paroxysmal-nocturnal-haemoglobinuria-pnh-manifesting-on-ct-as-a-pathologic-segment-of-small-bowel
#11
David Zekria, Peter Boavida
Paroxysmal nocturnal haemoglobinuria, a rare, acquired, life-threatening disease of the blood, is characterised by a triad of haemolysis previously believed to occur mainly at night, bone marrow dysfunction, and thrombophilia. Paroxysmal nocturnal haemoglobinuria is customarily regarded to manifest clinically as haemolytic anaemia and haemoglobinuria experienced as reddened urine in the morning, pancytopenia, and thrombosis. We describe a case in which an abnormal segment of small bowel as visualised on computed tomography was the principal sign of the disease process on presentation...
December 2017: Radiology Case Reports
https://www.readbyqxmd.com/read/29467851/proteinase-3-expression-on-the-neutrophils-of-patients-with-paroxysmal-nocturnal-hemoglobinuria
#12
Hui Liu, Yi Liu, Yi Li, Zhaoyun Liu, Liyan Li, Shaoxue Ding, Yihao Wang, Tian Zhang, Lijuan Li, Zonghong Shao, Rong Fu
Proteinase 3 (PR3) is released from neutrophils and regulates platelet activity, which is associated with cluster of differentiation (CD)177 antigen (NB1), a glycosylphosphatidylinositol-linked protein. In the present study, the effect of PR3 on thrombosis in paroxysmal nocturnal hemoglobinuria (PNH) and PNH-aplastic anemia (AA) syndrome was explored. The expression of PR3 and NB1 on CD59- neutrophils was detected by flow cytometry, immunofluorescence (IF), reverse transcription-quantitative polymerase chain reaction analysis and western blotting...
March 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29395255/lessons-learned-from-bone-marrow-failure-in-systemic-lupus-erythematosus-case-reports-and-review-of-the-literature
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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 2018: Seminars in Immunopathology
https://www.readbyqxmd.com/read/29323198/paroxysmal-nocturnal-hemoglobinuria-pnh-brain-mri-ischemic-lesions-in-neurologically-asymtomatic-patients
#20
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
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