Stephen J Richards
- The role of flow cytometry in the diagnosis of paroxysmal nocturnal hemoglobinuria in the clinical laboratoryStephen J Richards
Department of Haematology, Haematological Malignancy Diagnostic Service, The Algernon Firth Building, Leeds General Infirmary, Leeds LS1 3EX, United Kingdom
Clin Lab Med 27:577-90, vii. 2007..As with all rare diseases, internal and external quality assurance is essential for good laboratory practice and to fulfill the requirements of national laboratory accreditation schemes...
- Recent advances in the diagnosis, monitoring, and management of patients with paroxysmal nocturnal hemoglobinuriaStephen J Richards
Haematological Malignancy Diagnostic Service, Department of Haematology, Leeds Teaching Hospitals NHS Trust, The Algernon Firth Building, Leeds General Infirmary, Leeds, LS1 3EX, United Kingdom
Cytometry B Clin Cytom 72:291-8. 2007..In both these developments, flow cytometry played a critical role. In the first instance, in monitoring direct response to a new therapeutic agent; second, in demonstrating the phenotypic/genotypic link in a new form of GPI deficiency...
- Rapid diagnosis of acute promyelocytic leukemia (PML): applicability of flow cytometry and PML protein immunofluorescenceMatthew J Cullen
Cancer Genet Cytogenet 148:176-7. 2004
- Immunophenotypic analysis of PNH cellsStephen J Richards
Haematological Malignancy Diagnostic Service, Leeds, United Kingdom
Curr Protoc Cytom . 2002....
- Development and evaluation of a stabilized whole-blood preparation as a process control material for screening of paroxysmal nocturnal hemoglobinuria by flow cytometryStephen J Richards
Haematological Malignancy Diagnostic Service, St James s University Hospital, Leeds LS9 7TF, United Kingdom
Cytometry B Clin Cytom 76:47-55. 2009..Currently, no external quality assessment (EQA) programme or reference material is available for whole-blood PNH testing (red cells and leucocytes) by flow cytometry...
- Sustained response and long-term safety of eculizumab in paroxysmal nocturnal hemoglobinuriaAnita Hill
Department of Haematology, Leeds Teaching Hospitals NHS Trust, Leeds, LS1 3EX, United Kingdom
Blood 106:2559-65. 2005..Eculizumab continued to be safe and well tolerated, and all patients completed the study. The close relationship between sustained terminal complement inhibition, hemolysis, and symptoms was demonstrated...
- Early prediction of outcome and response to alemtuzumab therapy in chronic lymphocytic leukemiaAndy C Rawstron
Academic Unit of Haematology and Oncology, Haematological Malignancy Diagnostic Service, Leeds General Infirmary, United Kingdom
Blood 103:2027-31. 2004..If validated in prospective studies, blood monitoring at 2 and 4 weeks may be used to optimize therapy...
- Monoclonal B-cell lymphocytosis and chronic lymphocytic leukemiaAndy C Rawstron
Haematological Malignancy Diagnostic Service, Leeds Teaching Hospitals, Leeds, United Kingdom
N Engl J Med 359:575-83. 2008..Asymptomatic persons with fewer CLL-phenotype cells have monoclonal B-cell lymphocytosis (MBL). The goal of this study was to investigate the relation between MBL and CLL...
- Effect of eculizumab on haemolysis-associated nitric oxide depletion, dyspnoea, and measures of pulmonary hypertension in patients with paroxysmal nocturnal haemoglobinuriaAnita Hill
Department of Haematology, St James s Institute of Oncology, Leeds
Br J Haematol 149:414-25. 2010..These data demonstrated that intravascular haemolysis in PNH produces a state of NO catabolism leading to signs of PH, including elevated NT pro-BNP and dyspnoea that are significantly improved by treatment with eculizumab...
- Recent developments in the understanding and management of paroxysmal nocturnal haemoglobinuriaAnita Hill
Department of Haematology, Leeds Teaching Hospitals NHS Trust, Great George Street, Leeds, UK
Br J Haematol 137:181-92. 2007....
- Protection of erythrocytes from human complement-mediated lysis by membrane-targeted recombinant soluble CD59: a new approach to PNH therapyAnita Hill
Haematological Malignancy Diagnostic Service, Leeds Teaching Hospitals National Health Service Trust, Algernon Firth Building, Great George Street, Leeds, United Kingdom, LS1 3EX
Blood 107:2131-7. 2006..Thus, rhCD59-P corrects the CD59 deficiency in vitro and can bind to erythrocytes in an in vivo murine model, protecting the cells from the activity of human complement, and represents a potential therapeutic strategy in PNH...
- Effect of eculizumab on hemolysis and transfusion requirements in patients with paroxysmal nocturnal hemoglobinuriaPeter Hillmen
Department of Haematology, Leeds Teaching Hospitals National Health Science Trust, Leeds, United Kingdom
N Engl J Med 350:552-9. 2004..We tested the clinical efficacy of eculizumab, a humanized antibody that inhibits the activation of terminal complement components, in patients with PNH...
- Under-recognized complications in patients with paroxysmal nocturnal haemoglobinuria: raised pulmonary pressure and reduced right ventricular functionAnita Hill
Department of Haematology, St James s Institute of Oncology, Leeds, UK
Br J Haematol 158:409-14. 2012..Together, these data suggest a high prevalence of haemolysis-associated NO scavenging, Doppler-estimated systolic pulmonary hypertension, and depressed right ventricular function in patients with PNH...
- Eculizumab prevents intravascular hemolysis in patients with paroxysmal nocturnal hemoglobinuria and unmasks low-level extravascular hemolysis occurring through C3 opsonizationAnita Hill
1Department of Haematology, St James University Hospital, Leeds, UK
Haematologica 95:567-73. 2010..This may be due to C3-mediated clearance of paroxysmal nocturnal hemoglobinuria red blood cells through the reticuloendothelial system...
- Long-term treatment with eculizumab in paroxysmal nocturnal hemoglobinuria: sustained efficacy and improved survivalRichard J Kelly
Department of Haematology, Leeds Teaching Hospitals, Leeds, UK
Blood 117:6786-92. 2011..001). Eculizumab dramatically alters the natural course of PNH, reducing symptoms and disease complications as well as improving survival to a similar level to that of the general population...
- The glycosylphosphatidylinositol anchor and paroxysmal nocturnal haemoglobinuria/aplasia modelClaire Hall
Haematological Malignancy Diagnostic Service, Leeds General Infirmary, Leeds, UK
Acta Haematol 108:219-30. 2002..Gene therapy remains a tantalising possibility, although a greater understanding of the pathophysiology of PNH is required, as well as advances in gene therapy techniques, before such an approach can be seriously considered...
- Standardizing leucocyte PNH clone detection: an international studyMatthew Fletcher
UK NEQAS for Leucocyte Immunophenotyping UK NEQAS LI, Department of Haematology, Royal Hallamshire Hospital, Sheffield, United Kingdom
Cytometry B Clin Cytom 86:311-8. 2014..Consensus and Practical Guidelines for robust high-sensitivity detection of glycophosphatidylinostitol-deficient structures on red blood cells and white blood cells in paroxysmal nocturnal hemoglobinuria (PNH) were recently published...
- Monoclonal B lymphocytes with the characteristics of "indolent" chronic lymphocytic leukemia are present in 3.5% of adults with normal blood countsAndy C Rawstron
Haematological Malignancy Diagnostic Service, Academic Unit of Haematology and Oncology, Algernon Firth Building, University of Leeds, United Kingdom
Blood 100:635-9. 2002..8%-6.6%) of IgH somatic hypermutation. The monoclonal CLL phenotype cells present in otherwise healthy individuals may represent a very early stage of indolent CLL and should be useful in elucidating the mechanisms of leukemogenesis...
- Inherited predisposition to CLL is detectable as subclinical monoclonal B-lymphocyte expansionAndy C Rawstron
Academic Unit of Haematology and Oncology, University of Leeds, HMDS, West Yorkshire, Surrey, United Kingdom
Blood 100:2289-90. 2002..The detection of CLL-phenotype cells provides a surrogate marker of carrier status, potentially facilitating gene identification through mapping in families and direct analysis of isolated CLL-phenotype cells...