H J Lachmann

Summary

Affiliation: University College London
Country: UK

Publications

  1. doi request reprint AL amyloidosis associated with IgM paraproteinemia: clinical profile and treatment outcome
    Ashutosh 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
  2. ncbi request reprint Amyloidosis and the lung
    H 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
  3. ncbi request reprint AA amyloidosis complicating hyperimmunoglobulinemia D with periodic fever syndrome: a report of two cases
    Helen J Lachmann
    Royal Free and University College Medical School, London, UK
    Arthritis Rheum 54:2010-4. 2006
  4. ncbi request reprint Systemic amyloidosis
    Helen 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
  5. ncbi request reprint Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations
    H 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
  6. pmc Developments in the scientific and clinical understanding of autoinflammatory disorders
    Helen 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
  7. doi request reprint Use of canakinumab in the cryopyrin-associated periodic syndrome
    Helen J Lachmann
    University College London Medical School, London, United Kingdom
    N Engl J Med 360:2416-25. 2009
  8. doi request reprint Renal transplantation in systemic amyloidosis-importance of amyloid fibril type and precursor protein abundance
    J H Pinney
    UK National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK
    Am J Transplant 13:433-41. 2013
  9. ncbi request reprint The genetic basis of autosomal dominant familial Mediterranean fever
    D 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
  10. doi request reprint Hereditary lysozyme amyloidosis -- phenotypic heterogeneity and the role of solid organ transplantation
    P 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

Detail Information

Publications42

  1. doi request reprint AL amyloidosis associated with IgM paraproteinemia: clinical profile and treatment outcome
    Ashutosh 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
    ....
  2. ncbi request reprint Amyloidosis and the lung
    H 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
    ....
  3. ncbi request reprint AA amyloidosis complicating hyperimmunoglobulinemia D with periodic fever syndrome: a report of two cases
    Helen 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...
  4. ncbi request reprint Systemic amyloidosis
    Helen 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
    ....
  5. ncbi request reprint Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations
    H 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...
  6. pmc Developments in the scientific and clinical understanding of autoinflammatory disorders
    Helen 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
    ....
  7. doi request reprint Use of canakinumab in the cryopyrin-associated periodic syndrome
    Helen 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...
  8. doi request reprint Renal transplantation in systemic amyloidosis-importance of amyloid fibril type and precursor protein abundance
    J H Pinney
    UK National Amyloidosis Centre, 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...
  9. ncbi request reprint The genetic basis of autosomal dominant familial Mediterranean fever
    D 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
    ....
  10. doi request reprint Hereditary lysozyme amyloidosis -- phenotypic heterogeneity and the role of solid organ transplantation
    P 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...
  11. ncbi request reprint Allelic variants in genes associated with hereditary periodic fever syndromes as susceptibility factors for reactive systemic AA amyloidosis
    E 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...
  12. doi request reprint Solid organ transplantation in AL amyloidosis
    P 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...
  13. ncbi request reprint Prevalence and significance of the familial Mediterranean fever gene mutation encoding pyrin Q148
    D 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
    ....
  14. ncbi request reprint Association of mutations in the NALP3/CIAS1/PYPAF1 gene with a broad phenotype including recurrent fever, cold sensitivity, sensorineural deafness, and AA amyloidosis
    Ebun 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...
  15. ncbi request reprint Unicentric Castleman's disease complicated by systemic AA amyloidosis: a curable disease
    H 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...
  16. pmc In vivo regulation of interleukin 1beta in patients with cryopyrin-associated periodic syndromes
    Helen 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...
  17. ncbi request reprint Organ transplantation in hereditary apolipoprotein AI amyloidosis
    J 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...
  18. ncbi request reprint Targeted pharmacological depletion of serum amyloid P component for treatment of human amyloidosis
    M 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
    ....
  19. ncbi request reprint Renal amyloidosis in intravenous drug users
    J 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...
  20. pmc Clinical immunology review series: An approach to the patient with a periodic fever syndrome
    H 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...
  21. doi request reprint Cardiac amyloidosis, a monoclonal gammopathy and a potentially misleading mutation
    Ashutosh 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...
  22. pmc Diagnosis, pathogenesis, treatment, and prognosis of hereditary fibrinogen A alpha-chain amyloidosis
    Julian 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...
  23. doi request reprint Efficacy of bortezomib in systemic AL amyloidosis with relapsed/refractory clonal disease
    Ashutosh 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...
  24. ncbi request reprint Natural history and outcome in systemic AA amyloidosis
    Helen 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...
  25. ncbi request reprint Spectrum of clinical features in Muckle-Wells syndrome and response to anakinra
    Philip 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...
  26. ncbi request reprint Safety and efficacy of risk-adapted cyclophosphamide, thalidomide, and dexamethasone in systemic AL amyloidosis
    Ashutosh 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...
  27. ncbi request reprint Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis
    Helen 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
    ....
  28. ncbi request reprint Sequential heart and autologous stem cell transplantation for systemic AL amyloidosis
    Julian 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...
  29. doi request reprint Outcome in renal Al amyloidosis after chemotherapy
    Jennifer 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...
  30. ncbi request reprint Outcome in systemic AL amyloidosis in relation to changes in concentration of circulating free immunoglobulin light chains following chemotherapy
    Helen 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...
  31. ncbi request reprint Outcome of autologous stem cell transplantation for AL amyloidosis in the UK
    Hugh 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
    ....
  32. ncbi request reprint Heterogeneity among patients with tumor necrosis factor receptor-associated periodic syndrome phenotypes
    Ebun 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...
  33. ncbi request reprint Renal amyloidosis
    Helen 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...
  34. ncbi request reprint Hypercalcemia in a patient with common variable immunodeficiency and renal granulomas
    Alex 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...
  35. doi request reprint Sustained pharmacological depletion of serum amyloid P component in patients with systemic amyloidosis
    Julian 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...
  36. ncbi request reprint Novel pharmacological strategies in amyloidosis
    Helen 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
    ....
  37. ncbi request reprint Translocations of 14q32 and deletions of 13q14 are common chromosomal abnormalities in systemic amyloidosis
    Christine 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...
  38. ncbi request reprint High-dose melphalan versus melphalan plus dexamethasone for AL amyloidosis
    Helen J Lachmann
    N Engl J Med 358:91-2; author reply 92-3. 2008
  39. ncbi request reprint Eprodisate for the treatment of renal disease in AA amyloidosis
    Laura M Dember
    Boston University School of Medicine, Boston, MA 02118, USA
    N Engl J Med 356:2349-60. 2007
    ....
  40. ncbi request reprint Interleukin-1-receptor antagonist in the Muckle-Wells syndrome
    Philip N Hawkins
    N Engl J Med 348:2583-4. 2003
  41. ncbi request reprint Phenotype, genotype, and sustained response to anakinra in 22 patients with autoinflammatory disease associated with CIAS-1/NALP3 mutations
    Kieron 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...
  42. ncbi request reprint Familial amyloidotic polyneuropathy with severe renal involvement in association with transthyretin Gly47Glu in Dutch, British and American-Finnish families
    Elizabeth 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...