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Paroxysmal nocturnal hemoglobinuria

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https://www.readbyqxmd.com/read/29779325/-comparison-of-hemolytic-characteristics-among-paroxysmal-nocturnal-hemoglobinuria-autoimmune-hemolytic-anemia-and-hereditary-spherocytosis
#1
W W Li, J Shi, Z D Huang, N Nie, Y Q Shao, X X Li, M L Ge, J Zhang, J B Huang, P Jin, M Wang, Y Z Zheng
Objective: To determine the valuable hemolytic characteristics in differential diagnosis of paroxysmal nocturnal hemoglobinuria (PNH), autoimmune hemolytic anemia (AIHA) and hereditary spherocytosis (HS). Method: The clinical and hemolytic characteristics of 108 PNH patients, 127 AIHA patients and 172 HS patients diagnosed from January 1998 to April 2017 were compared. Results: ①Reticulocyte percentage (Ret%) of PNH patients [6.70% (0.14%-22.82%)] was significantly lower than that of AIHA [14.00%(0.10%-55...
April 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29755372/use-of-laser-assisted-optical-rotational-cell-analyzer-lorrca-maxsis-in-the-diagnosis-of-rbc-membrane-disorders-enzyme-defects-and-congenital-dyserythropoietic-anemias-a-monocentric-study-on-202-patients
#2
Anna Zaninoni, Elisa Fermo, Cristina Vercellati, Dario Consonni, Anna P Marcello, Alberto Zanella, Agostino Cortelezzi, Wilma Barcellini, Paola Bianchi
Chronic hemolytic anemias are a group of heterogeneous diseases mainly due to abnormalities of red cell (RBC) membrane and metabolism. The more common RBC membrane disorders, classified on the basis of blood smear morphology, are hereditary spherocytosis (HS), elliptocytosis, and hereditary stomatocytoses (HSt). Among RBC enzymopathies, the most frequent is pyruvate kinase (PK) deficiency, followed by glucose-6-phosphate isomerase, pyrimidine 5' nucleotidase P5'N, and other rare enzymes defects. Because of the rarity and heterogeneity of these diseases, diagnosis may be often challenging despite the availability of a variety of laboratory tests...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29749402/clonal-piga-mosaicism-and-dynamics-in-paroxysmal-nocturnal-hemoglobinuria
#3
Michael J Clemente, Bartlomiej Przychodzen, Cassandra M Hirsch, Yasunobu Nagata, Taha Bat, Marcin W Wlodarski, Tomas Radivoyevitch, Hideki Makishima, Jaroslaw P Maciejewski
No abstract text is available yet for this article.
April 25, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/29722478/successful-reduced-intensity-conditioning-hematopoietic-stem-cell-transplantation-for-paroxysmal-nocturnal-hemoglobinuria-with-aplastic-anemia-in-two-children
#4
Jeffrey R Andolina, Ariel L Reinish, Razia Akhtar, Suzie Noronha, Jessica C Shand, Angela Girvin, David N Korones, Lauren B Bruckner, Craig A Mullen, Kevin J Curran, Farid Boulad
Paroxysmal nocturnal hemoglobinuria (PNH) is an extremely rare cause of bone marrow failure in children. We report two children who presented with pancytopenia, and were diagnosed with PNH with severe aplastic anemia. Both children underwent upfront, successful hematopoietic stem cell transplantation with reduced-intensity conditioning. One patient had a syngeneic donor, and one patient had a 10/10 matched unrelated donor. Neither patient developed graft versus host disease, infections, or recurrent PNH. Reduced-intensity conditioning hematopoietic stem cell transplantation is a reasonable therapy for PNH with marrow failure in children...
May 3, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29721255/paroxysmal-nocturnal-hemoglobinuria-when-delay-in-diagnosis-and-long-therapy-occurs
#5
Salvatrice Mancuso, Giuseppe Sucato, Melania Carlisi, Marco Santoro, Giuseppe Tarantino, Emilio Iannitto, Mariasanta Napolitano, Sergio Siragusa
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disorder characterized by hemolytic anemia, bone marrow failure and thrombosis, caused by a somatic mutation in PIG-A gene that results in the absence of CD55 and CD59, two important complement regulatory proteins. In this paper, a case of PNH is retrospectively examined looking for clinical and laboratory features, and the entire course of the disease from the onset of the symptoms is described, together with an adequate follow- up over a 7-years treatment period...
March 2, 2018: Hematology Reports
https://www.readbyqxmd.com/read/29649283/design-and-preclinical-characterization-of-alxn1210-a-novel-anti-c5-antibody-with-extended-duration-of-action
#6
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/29629284/paroxysmal-nocturnal-hemoglobinuria-kidney-biopsy-and-magnetic-resonance-imaging
#7
Changhyun Park, So Mi Kim, Doh Hyung Kim, Eun Kyoung Lee
No abstract text is available yet for this article.
March 2018: Kidney Research and Clinical Practice
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
#8
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
#9
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
#10
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...
June 15, 2018: Gene
https://www.readbyqxmd.com/read/29545341/concurrent-treatment-of-aplastic-anemia-paroxysmal-nocturnal-hemoglobinuria-syndrome-with-immunosuppressive-therapy-and-eculizumab-a-uk-experience
#11
Morag Griffin, Austin Kulasekararaj, Shreyans Gandhi, Talha Munir, Stephen Richards, Louise Arnold, Nana Benson-Quarm, Nicola Copeland, Isabel Duggins, Kathryn Riley, Peter Hillmen, Judith Marsh, Anita Hill
No abstract text is available yet for this article.
March 15, 2018: Haematologica
https://www.readbyqxmd.com/read/29532535/screening-and-diagnostic-clinical-algorithm-for-paroxysmal-nocturnal-hemoglobinuria-expert-consensus
#12
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
#13
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/29503741/beyond-the-role-of-cd55-as-a-complement-component
#14
REVIEW
So Hee Dho, Jae Cheong Lim, Lark Kyun Kim
The complement is a part of the immune system that plays several roles in removing pathogens. Despite the importance of the complement system, the exact role of each component has been overlooked because the complement system was thought to be a nonspecific humoral immune mechanism that worked against pathogens. Decay-accelerating factor (DAF or CD55) is a known inhibitor of the complement system and has recently attracted substantial attention due to its role in various diseases, such as cancer, protein-losing enteropathy, and malaria...
February 2018: Immune Network
https://www.readbyqxmd.com/read/29486674/renal-involvement-in-paroxysmal-nocturnal-hemoglobinuria-an-update-on-clinical-features-pathophysiology-and-treatment
#15
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/29467851/proteinase-3-expression-on-the-neutrophils-of-patients-with-paroxysmal-nocturnal-hemoglobinuria
#16
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/29447841/crispr-cas9-generated-human-cd46-cd55-and-cd59-knockout-cell-lines-as-a-tool-for-complement-research
#17
Astrid J F Thielen, Iris M van Baarsen, Marlieke L Jongsma, Sacha Zeerleder, Robbert M Spaapen, Diana Wouters
BACKGROUND: To prevent unwanted complement activation and subsequent damage, complement activation must be tightly regulated on healthy host cells. Dysregulation of the complement system contributes to the pathology of diseases like Paroxysmal Nocturnal Hemoglobinuria and atypical Hemolytic Uremic Syndrome. To investigate complement regulator deficiencies, primary patient cells may be used, but access to patient cells may be limited and cells are heterogeneous between different patients...
May 2018: Journal of Immunological Methods
https://www.readbyqxmd.com/read/29395255/lessons-learned-from-bone-marrow-failure-in-systemic-lupus-erythematosus-case-reports-and-review-of-the-literature
#18
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/29389086/introduction-to-iccs-escca-consensus-guidelines-to-detect-gpi-deficient-cells-in-paroxysmal-nocturnal-hemoglobinuria-and-related-disorders
#19
Stephen J Richards
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
#20
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
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