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Genomes and Genes | H J LachmannSummaryAffiliation: University College London Country: UK Publications
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Publications
AL amyloidosis associated with IgM paraproteinemia: clinical profile and treatment outcomeAshutosh D Wechalekar
National Amyloidosis Centre, Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine Hampstead Campus, Royal Free and University College Medical School, London, United Kingdom
Blood 112:4009-16. 2008....
Amyloidosis and the lungH J Lachmann
National Amyloidosis Centre and Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, Royal Free and University College Medical School, Royal Free Campus, London, UK
Chron Respir Dis 3:203-14. 2006....- AA amyloidosis complicating hyperimmunoglobulinemia D with periodic fever syndrome: a report of two casesHelen J Lachmann
Royal Free and University College Medical School, London, UK
Arthritis Rheum 54:2010-4. 2006..We report 2 further cases of patients with AA amyloidosis in HIDS, both of whom developed dialysis-dependent renal failure, and we describe the outcome of the first renal transplant in this setting... - Systemic amyloidosisHelen J Lachmann
National Amyloidosis Centre, Department of Medicine, Royal Free and University College Medical School, Royal Free Campus, London NW3 2PF, UK
Curr Opin Pharmacol 6:214-20. 2006.... - Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutationsH J Lachmann
Centre for Amyloidosis and Acut Phase Proteins, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
Rheumatology (Oxford) 45:746-50. 2006..To prospectively monitor inflammatory activity over a prolonged period in a cohort of Turkish patients with FMF, their healthy relatives and healthy controls and to relate this to their MEFV genotypes... 
Developments in the scientific and clinical understanding of autoinflammatory disordersHelen J Lachmann
National Amyloidosis Centre and Centre for Acute Phase Proteins, Department of Medicine, University College London Medical School, Hampstead Campus, Rowland Hill Street, London NW32PF, UK
Arthritis Res Ther 11:212. 2009....- Use of canakinumab in the cryopyrin-associated periodic syndromeHelen J Lachmann
University College London Medical School, London, United Kingdom
N Engl J Med 360:2416-25. 2009..The cryopyrin-associated periodic syndrome (CAPS) is a rare inherited inflammatory disease associated with overproduction of interleukin-1. Canakinumab is a human anti-interleukin-1beta monoclonal antibody... - The genetic basis of autosomal dominant familial Mediterranean feverD R Booth
Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, Royal Free and University College Medical School, Royal Free Campus, London, UK
QJM 93:217-21. 2000.... - Hereditary lysozyme amyloidosis -- phenotypic heterogeneity and the role of solid organ transplantationP T Sattianayagam
National Amyloidosis Centre, Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, Royal Free Campus, University College London Medical School, London, UK
J Intern Med 272:36-44. 2012..The aim of this study is to describe the phenotype and outcome of patients with ALys including the role of solid organ transplantation... - Allelic variants in genes associated with hereditary periodic fever syndromes as susceptibility factors for reactive systemic AA amyloidosisE Aganna
Department of Diabetes and Metabolic Medicine, Barts and London, Queen Mary s School of Medicine and Dentistry, Whitechapel, London, UK
Genes Immun 5:289-93. 2004..Although allelic variants in HPFs genes are not major susceptibility factors for AA amyloidosis in chronic inflammatory disease, low-penetrance variants of MEFV and TNFRSF1A may have clinically significant proinflammatory effects... - Renal transplantation in systemic amyloidosis-importance of amyloid fibril type and precursor protein abundanceJ H Pinney
UK National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK UCL Centre for Nephrology, University College London Medical School, Royal Free Campus, London, UK
Am J Transplant 13:433-41. 2013..02). Post-RTx chemotherapy was administered successfully to four AL patients. RTx outcome is influenced by amyloid type. Suppression of the fibril precursor protein is desirable in the amyloidoses that have a rapid natural history... - Solid organ transplantation in AL amyloidosisP T Sattianayagam
Department of Medicine, National Amyloidosis Centre, University College London Medical School, London, UK
Am J Transplant 10:2124-31. 2010..01). Amyloid was widespread in all liver transplant recipients. Solid organ transplantation has rarely been performed in AL amyloidosis, but these findings demonstrate feasibility and support a role in selected patients... - Prevalence and significance of the familial Mediterranean fever gene mutation encoding pyrin Q148D R Booth
Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, Royal Free and University College Medical School, Royal Free Campus, London, UK
QJM 94:527-31. 2001.... - Association of mutations in the NALP3/CIAS1/PYPAF1 gene with a broad phenotype including recurrent fever, cold sensitivity, sensorineural deafness, and AA amyloidosisEbun Aganna
Barts and The London, Queen Mary s School of Medicine and Dentistry, University of London, London, UK
Arthritis Rheum 46:2445-52. 2002..This study was undertaken to characterize the genetic basis of FCU, MWS, and an overlapping disorder in French Canadian, British, and Indian families, respectively... - Unicentric Castleman's disease complicated by systemic AA amyloidosis: a curable diseaseH J Lachmann
NHS National Amyloidosis Centre and the Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, Royal Free and University College Medical School, Royal Free Campus, London, UK
QJM 95:211-8. 2002..All types may be complicated by acquired systemic amyloidosis, usually of AA type, but occasionally of AL type associated with monoclonal gammopathy... - In vivo regulation of interleukin 1beta in patients with cryopyrin-associated periodic syndromesHelen J Lachmann
National Amyloidosis Centre, Royal Free and University College Medical School, London NW3 2PF, England, UK
J Exp Med 206:1029-36. 2009..Together, the study allowed new insights into the production and regulation of IL-1beta in man. It also indicated that CAPS is entirely mediated by IL-1beta and that canakinumab treatment restores physiological IL-1beta production... - Organ transplantation in hereditary apolipoprotein AI amyloidosisJ D Gillmore
National Amyloidosis Centre, Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, Hampstead Campus, Royal Free and University College Medical School, London, UK
Am J Transplant 6:2342-7. 2006..Failing organs in hereditary apoAI amyloidosis should be replaced since graft survival is excellent and confers substantial survival benefit... - Targeted pharmacological depletion of serum amyloid P component for treatment of human amyloidosisM B Pepys
Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, Royal Free and University College Medical School, London NW3 2PF, UK
Nature 417:254-9. 2002.... - Renal amyloidosis in intravenous drug usersJ O Connolly
Centre for Nephrology, Royal Free Hospital, Pond Street, London, UK
QJM 99:737-42. 2006..We recently observed an increase in the number of cases of renal amyloidosis among intravenous drug users in central London... - Clinical immunology review series: An approach to the patient with a periodic fever syndromeH J Lachmann
National Amyloidosis Centre and Centre for Amyloidosis and Acute Phase Proteins, Division of Medicine, University College London Medical School, Royal Free Campus, London, UK
Clin Exp Immunol 165:301-9. 2011..Over the past decade-and-a-half there has been significant progress in their understanding and treatment... - Cardiac amyloidosis, a monoclonal gammopathy and a potentially misleading mutationAshutosh D Wechalekar
National Amyloidosis Centre, Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine Hampstead Campus, Royal Free and University College Medical School, London, UK
Nat Clin Pract Cardiovasc Med 6:128-33. 2009..A 46-year-old Afro-Caribbean man presented with progressive dyspnea and recurrent syncope. Clinical examination revealed evidence of biventricular failure... - Efficacy of bortezomib in systemic AL amyloidosis with relapsed/refractory clonal diseaseAshutosh D Wechalekar
National Amyloidosis Centre, Department of Medicine, Royal Free and University College Medical School, Rowland Hill St, London NW3 2PF, United Kingdom
Haematologica 93:295-8. 2008..Fifteen (75%) patients experienced some degree of toxicity, which in 8 (40%) cases resulted in discontinuation of bortezomib. Bortezomib shows promise in the treatment of systemic AL amyloidosis... - Natural history and outcome in systemic AA amyloidosisHelen J Lachmann
National Amyloidosis Centre and Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, Royal Free and University College Medical School, London
N Engl J Med 356:2361-71. 2007..Little is known about the natural history of AA amyloidosis or its response to treatment... - Diagnosis, pathogenesis, treatment, and prognosis of hereditary fibrinogen A alpha-chain amyloidosisJulian D Gillmore
National Amyloidosis Centre, CAAPP, Department of Medicine, Royal Free Campus, University College London, Rowland Hill Street, London NW3 2PF, United Kingdom
J Am Soc Nephrol 20:444-51. 2009..Survival is markedly better than observed with systemic AL amyloidosis, and outcomes with renal replacement therapy are comparable to those for age-matched individuals with nondiabetic renal disease... - Spectrum of clinical features in Muckle-Wells syndrome and response to anakinraPhilip N Hawkins
Royal Free and University College Medical School, Royal Free Hospital, London, UK
Arthritis Rheum 50:607-12. 2004..Molecular studies suggest that NALP3 is involved in the processing of interleukin-1beta (IL-1beta), prompting us to investigate whether IL-1 blockade may be therapeutic in patients with MWS... - Safety and efficacy of risk-adapted cyclophosphamide, thalidomide, and dexamethasone in systemic AL amyloidosisAshutosh D Wechalekar
National Amyloidosis Centre, Department of Medicine, Royal Free and University College Medical School, Rowland Hill St, London NW3 2PF, UK
Blood 109:457-64. 2007..The clonal response rates to CTD reported here are higher than any previously reported nontransplantation regimen in AL amyloidosis, and risk adaptation allows its use in poorer risk patients. CTD merits prospective randomized study... - Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosisHelen J Lachmann
National Amyloidosis Centre, Department of Medicine, Royal Free and University College Medical School, Royal Free Campus, London, United Kingdom
N Engl J Med 346:1786-91. 2002.... - Sequential heart and autologous stem cell transplantation for systemic AL amyloidosisJulian D Gillmore
National Amyloidosis Centre, Department of Medicine, Royal Free and University College Medical School, London, United Kingdom
Blood 107:1227-9. 2006..Heart transplantation followed by SCT is feasible in selected patients with cardiac AL amyloidosis and may confer substantial survival benefit... - Outcome in renal Al amyloidosis after chemotherapyJennifer H Pinney
National Amyloidosis Centre, Division of Medicine, University College London Medical School, Rowland Hill St, London NW3 2PF, United Kingdom
J Clin Oncol 29:674-81. 2011..The purpose of this study was to evaluate the effect of chemotherapy on clinical outcome among patients with renal AL amyloidosis... - Outcome in systemic AL amyloidosis in relation to changes in concentration of circulating free immunoglobulin light chains following chemotherapyHelen J Lachmann
The National Amyloidosis Centre and Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, Royal Free and University College Medical School, London, UK
Br J Haematol 122:78-84. 2003..Clinical improvement following chemotherapy in AL amyloidosis is delayed, but treatment strategies can be guided by their early effect on serum FLC concentration... - Outcome of autologous stem cell transplantation for AL amyloidosis in the UKHugh J B Goodman
National Amyloidosis Centre, Centre for Amyloidosis and Acute Phase Proteins, Royal Free and University College Medical Schools, Hampstead Campus, Royal Free Hospital, London, UK
Br J Haematol 134:417-25. 2006.... - Heterogeneity among patients with tumor necrosis factor receptor-associated periodic syndrome phenotypesEbun Aganna
Barts and London, Queen Mary s School of Medicine and Dentistry, London, UK
Arthritis Rheum 48:2632-44. 2003..To investigate the prevalence of tumor necrosis factor receptor-associated periodic syndrome (TRAPS) among outpatients presenting with recurrent fevers and clinical features consistent with TRAPS... - Renal amyloidosisHelen J Lachmann
National Amyloidosis Centre, UCL Medical School, Royal Free Hospital Campus, London
Br J Hosp Med (Lond) 71:83-6. 2010..With effective treatment of the underlying amyloidotic condition and good supportive care renal function can stabilize or improve but many patients still progress to end-stage renal failure... - Hypercalcemia in a patient with common variable immunodeficiency and renal granulomasAlex Meyer
Department of Nephrology and Immunology, and Histopathology, Royal Free Hospital, London, United Kingdom
Am J Kidney Dis 45:e90-3. 2005..The mechanism of hypercalcemia in granulomatous disorders, and the difficulty of differentiating granulomatous CVID from sarcoidosis, are discussed... - Sustained pharmacological depletion of serum amyloid P component in patients with systemic amyloidosisJulian D Gillmore
Centre for Amyloidosis and Acute Phase Proteins and the National Amyloidosis Centre, University College London Medical School, London, UK
Br J Haematol 148:760-7. 2010..These promising clinical observations merit further study... - Novel pharmacological strategies in amyloidosisHelen J Lachmann
National Amyloidosis Centre, Department of Medicine, Royal Free and University College Medical School, Royal Free Hospital, London, UK
Nephron Clin Pract 94:c85-8. 2003.... - Translocations of 14q32 and deletions of 13q14 are common chromosomal abnormalities in systemic amyloidosisChristine J Harrison
Department of Haematology, Royal Free and University College Medical School, London, UK
Br J Haematol 117:427-35. 2002..Ten patients (27% overall and 33% of those with systemic disease) showed del(13q). The gain or loss of IGH and CCND1 signals provided evidence of numerical chromosomal changes in three patients... - High-dose melphalan versus melphalan plus dexamethasone for AL amyloidosisHelen J Lachmann
N Engl J Med 358:91-2; author reply 92-3. 2008 - Eprodisate for the treatment of renal disease in AA amyloidosisLaura M Dember
Boston University School of Medicine, Boston, MA 02118, USA
N Engl J Med 356:2349-60. 2007.... - Interleukin-1-receptor antagonist in the Muckle-Wells syndromePhilip N Hawkins
N Engl J Med 348:2583-4. 2003 - Phenotype, genotype, and sustained response to anakinra in 22 patients with autoinflammatory disease associated with CIAS-1/NALP3 mutationsKieron S Leslie
Department of Dermatology, University of California, San Francisco, USA
Arch Dermatol 142:1591-7. 2006..To characterize the multisystem chronic inflammatory phenotype, dermatopathologic features, and response to therapy with interleukin 1 receptor antagonist (anakinra) in patients with mutations in the CIAS-1/NALP3 gene... - Familial amyloidotic polyneuropathy with severe renal involvement in association with transthyretin Gly47Glu in Dutch, British and American-Finnish familiesElizabeth B Haagsma
Department of Gastroenterology and Hepatology, University Hospital Groningen, Hanzeplein 1, P.O. Box 30.001, 9700 RB Groningen, The Netherlands
Amyloid 11:44-9. 2004..These observations support early intervention with orthotopic liver transplantation in patients with FAP associated with TTR Gly47Glu...