A B Federici

Summary

Affiliation: University of Milan
Country: Italy

Publications

  1. ncbi request reprint Von Willebrand's disease: clinical management
    A B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Internal Medicine and Medical Specialties, IRCCS Foundation Maggiore Policlinico Hospital, Mangiagalli, Regina Elena and University of Milan, Milan, Italy
    Haemophilia 12:152-8. 2006
  2. doi request reprint Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: a 2013 update
    Augusto B Federici
    Hematology and Transfusion Medicine, L Sacco University Hospital, Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
    Semin Thromb Hemost 39:191-201. 2013
  3. ncbi request reprint Diagnosis and management of von Willebrand disease
    A B Federici
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine, IRCCS Maggiore Hospital, University of Milan, Italy
    Haemophilia 5:28-37. 1999
  4. doi request reprint Current management of patients with severe von Willebrand disease type 3: a 2012 update
    Augusto B Federici
    Hematology and Transfusion Medicine, L SACCO University Hospital and Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
    Acta Haematol 128:88-99. 2012
  5. doi request reprint Transfusion issues in cancer patients
    Augusto B Federici
    Hematology and Transfusion Medicine, L SACCO University Hospital and Department of Internal Medicine, University of Milan, Milan, Italy
    Thromb Res 129:S60-5. 2012
  6. doi request reprint Management of inherited von Willebrand disease in Italy: results from the retrospective study on 1234 patients
    Augusto B Federici
    Division of Hematology and Transfusion Medicine, L SACCO University Hospital and Department of Internal Medicine, University of Milan, Milan, Italy
    Semin Thromb Hemost 37:511-21. 2011
  7. ncbi request reprint The 80th anniversary of von Willebrand's disease: history, management and research
    A B Federici
    Department of Internal Medicine and Medical Specialities, Angelo Bianchi Bonomi Haemophilia Thrombosis Centre, IRCCS Maggiore Policlinico Hospital, Mangiagalli, Regina Elena Foundation and University of Milan, Milan, Italy
    Haemophilia 12:563-72. 2006
  8. ncbi request reprint Management of inherited von Willebrand disease in 2006
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Medicine and Medical Specialties, IRCCS Maggiore Hospital, Mangiagalli, Regina Elena Foundation, University of Milan, Milan, Italy
    Semin Thromb Hemost 32:616-20. 2006
  9. ncbi request reprint Diagnosis of inherited von Willebrand disease: a clinical perspective
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center Department of Medicine and Medical Specialties, IRCCS Maggiore Hospital, Mangiagalli, Regina Elena Foundation, University of Milan, Milan, Italy
    Semin Thromb Hemost 32:555-65. 2006
  10. ncbi request reprint The natural history of hepatitis C virus infection in Italian patients with von Willebrand's disease: a cohort study
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Medical Specialties, IRCCS Maggiore Hospital, Mangiagalli, Regina Elena Foundation, University of Milan, Italy
    Haematologica 91:503-8. 2006

Collaborators

Detail Information

Publications76

  1. ncbi request reprint Von Willebrand's disease: clinical management
    A B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Internal Medicine and Medical Specialties, IRCCS Foundation Maggiore Policlinico Hospital, Mangiagalli, Regina Elena and University of Milan, Milan, Italy
    Haemophilia 12:152-8. 2006
    ..Appropriate dosage and timing in repeated infusions are also very important in patients exposed to secondary long term prophylaxis for recurrent bleedings...
  2. doi request reprint Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: a 2013 update
    Augusto B Federici
    Hematology and Transfusion Medicine, L Sacco University Hospital, Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
    Semin Thromb Hemost 39:191-201. 2013
    ..Because of the heterogeneous mechanisms of AVWS, more than one therapeutic approach is often required to prevent or treat acute bleedings. Remission from some forms of AVWS can be obtained when the underlying disorders are treated...
  3. ncbi request reprint Diagnosis and management of von Willebrand disease
    A B Federici
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine, IRCCS Maggiore Hospital, University of Milan, Italy
    Haemophilia 5:28-37. 1999
    ..Platelet concentrates or desmopressin can be used as adjunctive treatments when poor correction of the BT after concentrates is associated with continued bleeding...
  4. doi request reprint Current management of patients with severe von Willebrand disease type 3: a 2012 update
    Augusto B Federici
    Hematology and Transfusion Medicine, L SACCO University Hospital and Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
    Acta Haematol 128:88-99. 2012
    ..This review, based on clinical experience, provides an update on the clinical, laboratory and molecular markers of VWD3 that can be useful for determining the optimal therapeutic approach in these patients...
  5. doi request reprint Transfusion issues in cancer patients
    Augusto B Federici
    Hematology and Transfusion Medicine, L SACCO University Hospital and Department of Internal Medicine, University of Milan, Milan, Italy
    Thromb Res 129:S60-5. 2012
    ..In all areas of transfusion therapy, new advances such as pathogen inactivation and synthetic alternatives to blood components should help to increase the safety and tolerance of transfusion in cancer patients...
  6. doi request reprint Management of inherited von Willebrand disease in Italy: results from the retrospective study on 1234 patients
    Augusto B Federici
    Division of Hematology and Transfusion Medicine, L SACCO University Hospital and Department of Internal Medicine, University of Milan, Milan, Italy
    Semin Thromb Hemost 37:511-21. 2011
    ..Therefore, evidence-based studies should be organized only in well-characterized patients tested by laboratories that are expert in the clinical, laboratory, and molecular markers of VWD...
  7. ncbi request reprint The 80th anniversary of von Willebrand's disease: history, management and research
    A B Federici
    Department of Internal Medicine and Medical Specialities, Angelo Bianchi Bonomi Haemophilia Thrombosis Centre, IRCCS Maggiore Policlinico Hospital, Mangiagalli, Regina Elena Foundation and University of Milan, Milan, Italy
    Haemophilia 12:563-72. 2006
    ..The large number of publications quoting VWD and VWF emphasizes the important role of VWF in medicine...
  8. ncbi request reprint Management of inherited von Willebrand disease in 2006
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Medicine and Medical Specialties, IRCCS Maggiore Hospital, Mangiagalli, Regina Elena Foundation, University of Milan, Milan, Italy
    Semin Thromb Hemost 32:616-20. 2006
    ..Appropriate dosage and timing in repeated infusions are also very important in patients exposed to secondary long-term prophylaxis for recurrent bleedings...
  9. ncbi request reprint Diagnosis of inherited von Willebrand disease: a clinical perspective
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center Department of Medicine and Medical Specialties, IRCCS Maggiore Hospital, Mangiagalli, Regina Elena Foundation, University of Milan, Milan, Italy
    Semin Thromb Hemost 32:555-65. 2006
    ....
  10. ncbi request reprint The natural history of hepatitis C virus infection in Italian patients with von Willebrand's disease: a cohort study
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Medical Specialties, IRCCS Maggiore Hospital, Mangiagalli, Regina Elena Foundation, University of Milan, Italy
    Haematologica 91:503-8. 2006
    ..The main objective of this study was to compare the prevalence and outcome of hepatitis C virus (HCV) infection in multi-transfused patients with VWD and in those with hemophilia A or B...
  11. ncbi request reprint Acquired von Willebrand syndrome: an underdiagnosed and misdiagnosed bleeding complication in patients with lymphoproliferative and myeloproliferative disorders
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Dermatology, IRCCS Foundation Maggiore Policlinico Hospital, Mangiagalli, Italy
    Semin Hematol 43:S48-58. 2006
    ..An updated version of the International Registry on AVWS, recently available online, will provide more information on this rare, but underdiagnosed and misdiagnosed, disorder...
  12. ncbi request reprint Von Willebrand factor in high-purity factor VIII complex concentrates: chaperone protein or key to therapies? A meeting report
    A B Federici
    Department of Internal Medicine and Medical Specialties, Angelo Bianchi Bonomi Hemophilia Thrombosis Center, IRCCS Foundation Maggiore Policlinico Hospital, Mangiagalli, Regina Elena and University of Milan, Milan, Italy
    Haemophilia 14:133-9. 2008
  13. ncbi request reprint Diagnosis and management of acquired von Willebrand syndrome
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, University of Milan, Milano, Italy
    Clin Adv Hematol Oncol 1:169-75. 2003
    ..Acquired von Willebrand syndrome, although rare, warrants further understanding for clinical practice...
  14. ncbi request reprint Clinical use of Haemate P in inherited von Willebrand's disease: a cohort study on 100 Italian patients
    Augusto B Federici
    Centro Emofilia e Trombosi Angelo Bianchi Bonomi, Fondazione IRCCS Ospedale Maggiore, Mangiagalli e Regina Elena, Milano
    Haematologica 92:944-51. 2007
    ....
  15. ncbi request reprint Management of inherited von Willebrand disease in 2007
    Augusto B Federici
    Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Department of Medicine and Medical Specialities, IRCCS Maggiore Policlinico Hospital, Mangiagalli and Regina Elena Foundation and University of Milan, Milan, Italy
    Ann Med 39:346-58. 2007
    ..In type 3 and in severe forms of types 1 and 2 VWD, DDAVP is not effective and plasma virally inactivated VWF concentrates should be used in bleedings, surgery, and secondary long-term prophylaxis...
  16. doi request reprint Highly purified VWF/FVIII concentrates in the treatment and prophylaxis of von Willebrand disease: the PRO. WILL Study
    A B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Internal Medicine and Medical Specialties, Foundation IRCCS Maggiore Policlinico Hospital, Mangiagalli, Regina Elena, Milan, Italy
    Haemophilia 13:15-24. 2007
    ..The cost-effectiveness of these prophylaxis regimens versus on demand therapy will be now investigated in one large prospective study (PRO.WILL) organized in Italy...
  17. ncbi request reprint The safety of plasma-derived von Willebrand/factor VIII concentrates in the management of inherited von Willebrand disease
    Augusto B Federici
    Department of Medicine and Medical Specialties, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Foundation Maggiore Policlinico Hospital, Mangiagalli, Regina Elena and University of Milan, Milano, Italy
    Expert Opin Drug Saf 8:203-10. 2009
    ..In repeated infusions during surgery, the dosage and timing of administration should be planned to keep FVIII below 150 - 200 U/dl to avoid any possible risk of thrombosis...
  18. ncbi request reprint Acquired von Willebrand syndrome associated with hypothyroidism: a mild bleeding disorder to be further investigated
    Augusto B Federici
    Division of Hematology and Transfusion Medicine, L Sacco University Hospital, Department of Internal Medicine, University of Milan, Milan, Italy
    Semin Thromb Hemost 37:35-40. 2011
    ..VWF/FVIII concentrates have been used in only a few patients. The use of thyroid hormones can reverse this abnormality...
  19. doi request reprint Classification of inherited von Willebrand disease and implications in clinical practice
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Medical Specialties, IRCCS Maggiore Hospital, and University of Milan, Milan, Italy
    Thromb Res 124:S2-6. 2009
    ..Correct classification is essential to the management of patients with VWD because therapeutic decisions often depend on the specific type of disease...
  20. doi request reprint Efficacy and safety of highly purified, doubly virus-inactivated VWF/FVIII concentrates in inherited von Willebrand's disease: results of an Italian cohort study on 120 patients characterized by bleeding severity score
    A B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Medicine and Medical Specialties, IRCCS Foundation Ospedale Maggiore, Mangiagalli, Regina Elena and Universita degli Studi di Milano, Via Pace 9, Milan, Italy
    Haemophilia 16:101-10. 2010
    ..These results confirm the efficacy and safety of the study concentrates, not only in the management of bleeding and surgery but also in secondary prophylaxis of severe VWD...
  21. pmc Prophylaxis of bleeding episodes in patients with von Willebrand's disease
    Augusto B Federici
    Centro Emofilia e Trombosi Angelo Bianchi Bonomi, Fondazione IRCCS Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Universita degli Studi di Milano, Milano, Italia
    Blood Transfus 6:s26-32. 2008
    ..The cost-effectiveness of these prophylaxis regimens versus on-demand therapy will now be investigated in one large international study..
  22. doi request reprint Clinical and molecular predictors of thrombocytopenia and risk of bleeding in patients with von Willebrand disease type 2B: a cohort study of 67 patients
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Medicine and Medical Specialties, Istituti di Ricovero e Cura a Carattere Scientifico Maggiore Hospital, University of Milan, Italy
    Blood 113:526-34. 2009
    ..66; 95% confidence interval, 1.03-31.07). Prediction of possible thrombocytopenia in VWD2B by measuring VWF-GPIb-alpha/BC is important because a low platelet count is an independent risk factor for bleeding...
  23. ncbi request reprint Acquired von Willebrand syndrome: is it an extremely rare disorder or do we see only the tip of the iceberg?
    A B Federici
    Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Department of Internal Medicine and Medical Specialties, IRCCS Foundation Maggiore Policlinico Hospital, Mangiagalli, Regina Elena and University of Milan, Milan, Italy
    J Thromb Haemost 6:565-8. 2008
  24. doi request reprint The use of desmopressin in von Willebrand disease: the experience of the first 30 years (1977-2007)
    A B Federici
    Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Department of Medicine and Medical Specialities, IRCCS Maggiore Policlinico Hospital, University of Milan, Milan, Italy
    Haemophilia 14:5-14. 2008
    ....
  25. ncbi request reprint Use of intravenous immunoglobulin in patients with acquired von Willebrand syndrome
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Internal Medicine and Dermatology, IRCCS Maggiore Hospital and University of Milan, Milan, Italy
    Hum Immunol 66:422-30. 2005
    ..Prospective studies are required to evaluate the efficacy and safety of IVIg in AVWS...
  26. ncbi request reprint Management of von Willebrand disease with factor VIII/von Willebrand factor concentrates: results from current studies and surveys
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Dermatology, Fondazione Ospedale Policlinico, Mangiagalli e Regina Elena, and University of Milan, Italy
    Blood Coagul Fibrinolysis 16:S17-21. 2005
    ..On the basis of these observations, the dosage and timing of FVIII/VWF administration should be planned to keep FVIII:C concentrations between 50 U/dl and 150 U/dl in the postoperative period...
  27. ncbi request reprint Therapeutic approaches to acquired von Willebrand syndrome
    A B Federici
    Haemophilia and Thrombosis Centre, Via Pace 9, 20122 Milano, Italy
    Expert Opin Investig Drugs 9:347-54. 2000
    ..Therefore, treatment must be customized for each patient according to the underlying disorder, as well as to the type and the severity of bleeding episode and must be targeted to each specific case...
  28. ncbi request reprint Acquired von Willebrand syndrome: data from an international registry
    A B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy
    Thromb Haemost 84:345-9. 2000
    ..On the basis of the information provided by this registry guidelines for diagnosis and management of the AvWS are given...
  29. ncbi request reprint Optimising local therapy during oral surgery in patients with von Willebrand disease: effective results from a retrospective analysis of 63 cases
    A B Federici
    Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre and Department of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Milan, Italy
    Haemophilia 6:71-7. 2000
    ....
  30. ncbi request reprint Acquired von Willebrand syndrome 2004: International Registry--diagnosis and management from online to bedside
    A B Federici
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Dermatology, IRCCS Maggiore Hospital and University of Milan, Via Pace 9, 20122 Milano, Italy
    Hamostaseologie 24:50-5. 2004
    ..The authors are co-ordinating an updated version of the International Registry on AVWS that will allow data to be entered directly online...
  31. ncbi request reprint Optimizing therapy with factor VIII/von Willebrand factor concentrates in von Willebrand disease
    A B Federici
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, University of Milan, Italy
    Haemophilia 4:7-10. 1998
    ....
  32. ncbi request reprint Mild forms of von Willebrand disease: diagnosis and management
    Augusto B Federici
    Department of Internal Medicine, Angelo Bianchi Bonomi Hemophilia Thrombosis Center, IRCCS Maggiore Hospital and University of Milan, Via Pace 9, 20122 Milano, Italy
    Curr Hematol Rep 2:373-80. 2003
    ..In type 1 and type 2 VWD unresponsive to DDAVP, plasma virally inactivated concentrates containing VWF and factor VIII are the mainstay of treatment...
  33. ncbi request reprint Clinical efficacy of highly purified, doubly virus-inactivated factor VIII/von Willebrand factor concentrate (Fanhdi) in the treatment of von Willebrand disease: a retrospective clinical study
    A B Federici
    Department of Internal Medicine, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Ospedale Maggiore, University of Milan, Italy
    Haemophilia 8:761-7. 2002
    ..These results confirm the efficacy and safety of this concentrate in the management of bleeding episodes and in the prevention of excessive bleeding during major and minor surgery...
  34. ncbi request reprint Diagnosis of von Willebrand disease
    A B Federici
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, University of Milan, Italy
    Haemophilia 4:654-60. 1998
    ..Due to the heterogeneity of vWF defects, a correct diagnosis of types and subtypes may be sometimes difficult but is very important for an appropriate treatment of patients with vWD...
  35. ncbi request reprint Clinical diagnosis of von Willebrand disease
    A B Federici
    Angelo Bianchi Bonomi Haemophilia Thrombosis Center, Department of Internal Medicine and Dermatology, IRCCS Maggiore Hospital and University of Milan, 20122 Milan, Italy
    Haemophilia 10:169-76. 2004
    ..In a more recent Italian prospective study (815/1234 cases observed for 1 year in 6/16 Italian centres), only 147 (18%) VWD patients showed bleeding episodes (n = 318) and minor or major surgeries (n = 87)...
  36. ncbi request reprint The factor VIII/von Willebrand factor complex: basic and clinical issues
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Italy
    Haematologica 88:EREP02. 2003
    ..The correct use of FVIII/VWF concentrates in VWD and HA have been reported in several national and international guidelines...
  37. ncbi request reprint Guidelines for the diagnosis and management of von Willebrand disease in Italy
    A B Federici
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Department of Internal Medicine, IRCCS Maggiore Hospital, University of Milan, Italy
    Haemophilia 8:607-21. 2002
    ..Platelet concentrates or desmopressin can be used as adjunctive treatments when poor correction of BT after plasma concentrate treatment is associated with continued bleeding...
  38. ncbi request reprint Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches
    A B Federici
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, University of Milano and Department of Hematology, S Bortolo Hospital, Vicenza, Italy
    Blood 92:2707-11. 1998
    ....
  39. ncbi request reprint A sensitive ristocetin co-factor activity assay with recombinant glycoprotein Ibalpha for the diagnosis of patients with low von Willebrand factor levels
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, Department of Internal Medicine and Dermatology, IRCCS Maggiore Hospital and University of Milan, Italy
    Haematologica 89:77-85. 2004
    ..A correct diagnosis of VWD relies on platelet agglutination tests that have a low accuracy within and between assays. A more accurate VWF:RCo assay would improve VWD diagnosis and classification...
  40. doi request reprint Expression studies of missense mutations p.D141Y, p.C275S located in the propeptide of von Willebrand factor in patients with type 3 von Willebrand disease
    L Baronciani
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Medical Specialties, Foundation IRCCS Maggiore Policlinico Hospital, Mangiagalli, Regina Elena and University of Milan, Milan, Italy
    Haemophilia 14:549-55. 2008
    ..Co-expression of hybrid rVWFs confirmed the recessive inheritance pattern of these missense mutations...
  41. doi request reprint Validation of a rapid test (VWF-LIA) for the quantitative determination of von Willebrand factor antigen in type 1 von Willebrand disease diagnosis within the European multicenter study MCMDM-1VWD
    G Castaman
    Department of Hematology, San Bortolo Hospital, Vicenza, Italy
    Thromb Res 126:227-31. 2010
    ..Accurate measurement of von Willebrand factor (VWF) is a critical requirement for the diagnosis of von Willebrand disease (VWD)...
  42. ncbi request reprint Impact of plasma von Willebrand factor levels in the diagnosis of type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1VWD)
    A Tosetto
    San Bortolo Hospital, Vicenza, Italy
    J Thromb Haemost 5:715-21. 2007
    ..However, quantitative analysis of the importance of VWF antigen (VWF:Ag) and ristocetin cofactor activity (VWF:RCo) levels in the diagnosis is lacking...
  43. ncbi request reprint Hemorrhagic symptoms and bleeding risk in obligatory carriers of type 3 von Willebrand disease: an international, multicenter study
    G Castaman
    Department of Hematology, S Bortolo Hospital, Vicenza, Italy
    J Thromb Haemost 4:2164-9. 2006
    ..We undertook an international, multicenter study to describe the clinical picture and to estimate the bleeding risk in a group of obligatory carriers of type 3 von Willebrand disease (VWD)...
  44. ncbi request reprint Biochemical characterization of a recombinant von Willebrand factor (VWF) with combined type 2B and type 1 defects in the VWF gene in two patients with a type 2A phenotype of von Willebrand disease
    L Baronciani
    Department of Medicine and Medical Specialties, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation and University of Milan, Milan, Italy
    J Thromb Haemost 5:282-8. 2007
    ..The two brothers were compound heterozygotes (C275R/P1337L), whereas the others members of the family were heterozygous for C275R (a novel mutation in the D1 domain) or P1337L (a type 2B mutation in the A1 domain)...
  45. ncbi request reprint A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD)
    A Tosetto
    San Bortolo Hospital, Vicenza, Italy
    J Thromb Haemost 4:766-73. 2006
    ..A quantitative description of bleeding symptoms in type 1 von Willebrand disease (VWD) has never been reported...
  46. doi request reprint Different bleeding risk in type 2A and 2M von Willebrand disease: a 2-year prospective study in 107 patients
    G Castaman
    Department of Hematology and Hemophilia and Thrombosis Center, San Bortolo Hospital, Vicenza, Italy
    J Thromb Haemost 10:632-8. 2012
    ..VWF gene mutations were characterized in all of them...
  47. doi request reprint Type 2A (IIH) von Willebrand disease is due to mutations that affect von Willebrand factor multimerization
    L Baronciani
    Department of Medicine and Medical Specialties, Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, IRCCS Maggiore Policlinico Hospital, Mangiagalli, Regina Elena Foundation and University of Milan, Milan, Italy
    J Thromb Haemost 7:1114-22. 2009
    ..Type IIH von Willebrand disease was reported 20 years ago as a novel variant characterized by the loss of the largest multimers in plasma and platelets and absence of the typical triplet structure...
  48. ncbi request reprint Factor VIII and von Willebrand factor changes after desmopressin and during pregnancy in type 2M von Willebrand disease Vicenza: a prospective study comparing patients with single (R1205H) and double (R1205H-M740I) defect
    G Castaman
    Department of Hematology and Hemophilia and Thrombosis Center, San Bortolo Hospital, Vicenza, Italy
    J Thromb Haemost 4:357-60. 2006
    ..No data on FVIII/VWF changes after desmopressin and during pregnancy in patients with phenotypic VWD Vicenza has been reported...
  49. doi request reprint Efficacy and safety during formulation switch of a pasteurized VWF/FVIII concentrate: results from an Italian prospective observational study in patients with von Willebrand disease
    G Castaman
    San Bortolo Hospital, Vicenza, Italy
    Haemophilia 19:82-8. 2013
    ..Volume-reduced Haemate(®) P was at least as effective and well-tolerated as the previous formulation...
  50. ncbi request reprint The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study
    F Rodeghiero
    Hematology Department, S Bortolo Hospital, Vicenza, Italy
    J Thromb Haemost 3:2619-26. 2005
    ..The aim of this study was the validation of the criteria defining a significant mucocutaneous-bleeding history in type 1 von Willebrand disease (VWD)...
  51. doi request reprint Management of bleeding disorders in adults
    F Peyvandi
    U O S Dipartimentale per la Diagnosi e la Terapia delle Coagulopatie, A Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione I R C C S Ca Granda Ospedale Maggiore Policlinico, Università degli Studi di Milano and Luigi Villa Foundation, Milan, Italy
    Haemophilia 18:24-36. 2012
    ..Will study, current evidence relating to economic aspects of the treatment of haemophilic patients with inhibitors (based on the PROFIT study), and an overview of musculoskeletal complications in adults with severe bleeding disorders...
  52. doi request reprint Understanding FVIII/VWF complex--report from a symposium of XXIX WFH meeting 2010
    A Gringeri
    Department of Internal Medicine, Università degli Studi di Milano and Fondazione Ca Granda IRCCS Ospedale Maggiore Policlinico, Milan, Italy
    Haemophilia 18:469-75. 2012
    ..The ongoing PRO.Will study is a prospective, multicenter trial aimed at assessing the efficacy, safety and pharmacoeconomics of secondary long-term prophylaxis in patients with severe inherited VWD...
  53. ncbi request reprint Evaluation of the PFA-100 system in the diagnosis and therapeutic monitoring of patients with von Willebrand disease
    M Cattaneo
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine, University of Milano, Italy
    Thromb Haemost 82:35-9. 1999
    ..The PFA-100 test reflects vWF-dependent platelet function under high shear stress and could be useful in the diagnosis and therapeutic monitoring of patients with vWD...
  54. doi request reprint Response to desmopressin is influenced by the genotype and phenotype in type 1 von Willebrand disease (VWD): results from the European Study MCMDM-1VWD
    Giancarlo Castaman
    Department of Hematology, San Bortolo Hospital, Vicenza, Italy
    Blood 111:3531-9. 2008
    ..The presence of subtle multimeric abnormalities did not hamper potential clinically useful responses, as in typical type 1 VWD...
  55. ncbi request reprint Molecular characterization of a multiethnic group of 21 patients with type 3 von Willebrand disease
    L Baronciani
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, IRCCS Maggiore Hospital and University of Milan, Italy
    Thromb Haemost 84:536-40. 2000
    ..Our results indicate that molecular defects responsible for type 3 VWD are scattered throughout the entire VWF gene (from exon 3 to 52), and that there is no prevalent and common gene defect in the three populations studied by us...
  56. ncbi request reprint Expression studies on a novel type 2B variant of the von Willebrand factor gene (R1308L) characterized by defective collagen binding
    L Baronciani
    Angelo Bianchi Bonomi Haemophilia and Thrombosis Center, Department of Internal Medicine and Dermatology IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation and University of Milan, Milan, Italy
    J Thromb Haemost 3:2689-94. 2005
    ..The latter finding suggests a more important role than recognized so far for the VWF A1 domain in VWF binding to collagen, which may contribute to the in vivo hemostatic defect associated with type 2B VWD...
  57. ncbi request reprint Management of inherited von Willebrand disease
    P M Mannucci
    Angelo Bianchi Bonomi Haemophilia and Thrombosis Center, Department of Internal Medicine, IRCCS Maggiore Hospital, University of Milan, Italy
    Best Pract Res Clin Haematol 14:455-62. 2001
    ....
  58. ncbi request reprint Advances in the genetics and treatment of von Willebrand disease
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Department of Internal Medicine, IRCCS Maggiore Hospital, University of Milan, Italy
    Curr Opin Pediatr 14:23-33. 2002
    ..Platelet concentrates or desmopressin can be used as adjunctive treatments when poor correction of the BT after concentrates is associated with continued bleeding...
  59. pmc Evidence-based recommendations on the treatment of von Willebrand disease in Italy
    Pier Mannuccio Mannucci
    Centro Emofilia e Trombosi Angelo Bianchi Bonomi, Dipartimento di Medicina e Specialità Mediche, Università di Milano e IRCCS Fondazione Ospedale Maggiore, Italy
    Blood Transfus 7:117-26. 2009
    ..The aim of treatment is to correct the dual defect of haemostasis caused by the abnormal/reduced VWF and the concomitant deficiency of factor VIII (FVIII)...
  60. ncbi request reprint Update on the management of von Willebrand disease
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia Thrombosis Center, IRCCS Foundation Maggiore Policlinico Hospital, University of Milan, Milan, Italy
    Clin Adv Hematol Oncol 6:29-30. 2008
  61. ncbi request reprint Different bleeding risk in type 2A and 2M von Willebrand disease: a 2-year prospective study in 107 patients: a reply to a rebuttal
    G Castaman
    Department of Cell Therapy and Hematology, Hemophilia and Thrombosis Center, San Bortolo Hospital, Vicenza Department of Medicine and Medical Specialties, A Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Cà Granda Foundation Maggiore Policlinico Hospital, Milan Division of Hematology and Transfusion Medicine, Department of Clinical and Community Sciences, L Sacco University Hospital, University of Milan, Milan, Italy
    J Thromb Haemost 10:1458-60. 2012
    ..J Thromb Haemost 2012; 10: 1458-60.See also Favaloro EJ, Bonar R, Marsden K. Different bleeding risk in type 2A and 2M von Willebrand disease: a 2-year prospective study in 107 patients: a rebuttal. This issue, pp 1455-8...
  62. doi request reprint Gynaecological and obstetrical problems in women with different bleeding disorders
    S M Siboni
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, University of Milan and Department of Medicine and Medical Specialties, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, Lugi Villa Foundation, Milan, Italy
    Haemophilia 15:1291-9. 2009
    ..These instruments may help to identify those women for whom a therapeutic intervention is warranted...
  63. ncbi request reprint Biologic response to desmopressin in patients with severe type 1 and type 2 von Willebrand disease: results of a multicenter European study
    Augusto B Federici
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Dermatology, IRCCS Maggio e Hospital and University of Milan, Italy
    Blood 103:2032-8. 2004
    ..This prospective study showed that the rate of biologic response to DDAVP is relatively low not only in type 2 but also in type 1 VWD when uniform and stringent criteria for patient selection and responsiveness are applied...
  64. ncbi request reprint Molecular defects in type 3 von Willebrand disease: updated results from 40 multiethnic patients
    Luciano Baronciani
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, IRCCS Maggiore Hospital and University of Milan, Italy
    Blood Cells Mol Dis 30:264-70. 2003
    ..This study extend our previous finding that mutations responsible for type 3 VWD are scattered throughout the entire VWF gene and that there is no founder effect in these three populations studied...
  65. ncbi request reprint Von Willebrand's disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment
    Giancarlo Castaman
    Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital and University of Milan, Italy
    Haematologica 88:94-108. 2003
    ..Bleeding manifestations are heterogeneous: mucosal bleeding is typical of all VWD cases but hemarthrosis and hematomas may also be present when FVIII levels are low...
  66. doi request reprint The impact of bleeding history, von Willebrand factor and PFA-100(®) on the diagnosis of type 1 von Willebrand disease: results from the European study MCMDM-1VWD
    Giancarlo Castaman
    Department of Haematology, San Bortolo Hospital, Vicenza, Italy
    Br J Haematol 151:245-51. 2010
    ....
  67. pmc Activation-independent platelet adhesion and aggregation under elevated shear stress
    Zaverio M Ruggeri
    The Scripps Research Institute MEM 175, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA
    Blood 108:1903-10. 2006
    ..Platelet and VWF modulation by hydrodynamic force is a mechanism for activation-independent aggregation that may support thrombotic arterial occlusion...
  68. ncbi request reprint von Willebrand disease type 2B must be always considered in the differential diagnosis of genetic thrombocytopenias with giant platelets
    Giuseppe Loffredo
    Department of Oncology, Azienda Santobono Pausilipon, Pausilipon Hospital, Naples, Italy
    Platelets 17:149-52. 2006
    ..Based on this observation and literature review, we suggest that the diagnosis of VWD 2B should be always considered in patients with chronic thrombocytopenia and giant platelets...
  69. ncbi request reprint Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD)
    Anne Goodeve
    The Academic Unit of Haematology, University of Sheffield, United Kingdom
    Blood 109:112-21. 2007
    ..About one third of the type 1 VWD cases recruited could be reconsidered as type 2. The remaining group could be considered "true" type 1 VWD, although mutations were found in only 55%...
  70. ncbi request reprint Evaluation of a new turbidimetric assay for von Willebrand factor activity useful in the general screening of von Willebrand disease
    Montse Piñol
    Haematologica 92:712-3. 2007
    ..Samples from healthy donors and previously diagnosed VWD patients were blindly analyzed by this new activity assay and standard VWF:RCo. Results agreed and both assays showed a similar sensitivity for the screening of VWD...
  71. ncbi request reprint An association of candidate gene haplotypes and bleeding severity in von Willebrand disease (VWD) type 1 pedigrees
    Thomas J Kunicki
    Department of Molecular and Experimental Medicine, The Scripps Research Institute, 10550 N Torrey Pines Rd, MEM 150, La Jolla, CA 92037, USA
    Blood 104:2359-67. 2004
    ..02). These results establish that genetic differences in the adhesion receptor subunits alpha(2), alpha(IIb,) and GPVI can influence the phenotype of VWD type 1...
  72. ncbi request reprint A new candidate mutation, G1629R, in a patient with type 2A von Willebrand's disease: basic mechanisms and clinical implications
    Lysiane Hilbert
    Preclinical Department, LFB, Lille, France
    Haematologica 89:1128-33. 2004
    ..The candidate G1629R mutation, identified in an Italian patient with type 2A VWD, was expressed to confirm the relationship between phenotype and genotype...
  73. ncbi request reprint A rapid assay for ristocetin cofactor activity using an automated coagulometer (ACL 9000)
    Antonella Lattuada
    Immunohematology and Blood Transfusion Service, L Sacco Hospital, Milan
    Blood Coagul Fibrinolysis 15:505-11. 2004
    ..We can conclude that this automatic ACL-VWF : RCo gives a reliable and useful measure of VWF activity and can be proposed as a screening test for rapid diagnosis of VWD even in patients with low VWF levels in plasma...
  74. ncbi request reprint Duodenal and gastric Dieulafoy's lesions in a patient with type 2A von Willebrand's disease
    Michele Marchese
    Gastrointest Endosc 61:322-5. 2005
  75. ncbi request reprint Molecular mapping of the chloride-binding site in von Willebrand factor (VWF): energetics and conformational effects on the VWF/ADAMTS-13 interaction
    Raimondo De Cristofaro
    Hemostasis Research Centre, Institute of Internal Medicine and Geriatrics, Catholic University School of Medicine, 00168 Rome, Italy
    J Biol Chem 281:30400-11. 2006
    ....
  76. pmc Identification of type 1 von Willebrand disease patients with reduced von Willebrand factor survival by assay of the VWF propeptide in the European study: molecular and clinical markers for the diagnosis and management of type 1 VWD (MCMDM-1VWD)
    Sandra L Haberichter
    Department of Pediatrics, Medical College of Wisconsin, Milwaukee, USA
    Blood 111:4979-85. 2008
    ..The systematic assay of both plasma VWF and the VWF propeptide in moderately severe type 1 VWD patients may identify patients with a reduced VWF survival phenotype...