Von Willebrand disease-associated angiodysplasia: A few answers, still many questions

British Journal of Haematology

Volumn 161, Issue 2, 2013, Pages 177-182

  Franchini, Massimo ^a   Mannucci, Pier Mannuccio ^b  

^a CARLO POMA HOSPITAL   (Italy)

^b FONDAZIONE IRCCS CA GRANDA OSPEDALE MAGGIORE POLICLINICO   (Italy)

Author keywords

Acquired von Willebrand syndrome; Angiodysplasia; Congenital von Willebrand disease; Gastrointestinal bleeding

Indexed keywords

BLOOD CLOTTING FACTOR 8 CONCENTRATE; BLOOD CLOTTING FACTOR 8 PLUS VON WILLEBRAND FACTOR; DESMOPRESSIN; IMMUNOGLOBULIN; OCTREOTIDE; RECOMBINANT BLOOD CLOTTING FACTOR 8; RECOMBINANT VON WILLEBRAND FACTOR; TRANEXAMIC ACID; VON WILLEBRAND FACTOR;

ANGIODYSPLASIA; ANGIOGENESIS; AORTA STENOSIS; AORTA VALVE REPLACEMENT; CHRONIC LYMPHATIC LEUKEMIA; DISEASE ASSOCIATION; DRUG EFFICACY; GASTROINTESTINAL HEMORRHAGE; HUMAN; HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY; MONOCLONAL IMMUNOGLOBULINEMIA; MULTIPLE MYELOMA; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; PROPHYLAXIS; REVIEW; VON WILLEBRAND DISEASE;

ANGIODYSPLASIA; GASTROINTESTINAL HEMORRHAGE; HUMANS; MALE; PARAPROTEINEMIAS; VON WILLEBRAND DISEASES; VON WILLEBRAND FACTOR;

EID: 84875814537     PISSN: 00071048     EISSN: 13652141     Source Type: Journal    
DOI: 10.1111/bjh.12272     Document Type: Review

References (54)

1. Abi-Akar, R., El-Rassi, I., Karam, M., Jassar, Y., Slim, R. & Jebara, V. (2011) Treatment of Heyde's Syndrome by aortic valve replacement. Current Cardiology Reviews, 7, 47-49.
2. Abshire, T. (2009) The role of prophylaxis in the management of von Willebrand disease: today and tomorrow. Thrombosis Research, 124(Suppl. 1), S15-S19.
3. Abshire, T.C., Federici, A.B., Alvárez, M.T., Bowen, J., Carcao, M.D., Cox Gill, J., Key, N.S., Kouides, P.A., Kurnik, K., Lail, A.E., Leebeek, F.W., Makris, M., Mannucci, P.M., Winikoff, R. & Berntorp, E.; VWD PN. (2013) Prophylaxis in severe forms of von Willebrand's disease: results from the von Willebrand Disease Prophylaxis Network (VWD PN). Haemophilia, 19, 76-81.
4. Alikhan, R. & Keeling, D. (2009) Von Willebrand disease, angiodysplasia and atorvastatin. British Journal of Haematology, 149, 159-160.
5. Berntorp, E. (2008) Prophylaxis in von Willebrand disease. Haemophilia, 14(Suppl 5), 47-53.
6. Berntorp, E. & Petrini, P. (2005) Long-term prophylaxis in von Willebrand disease. Blood Coagulation & Fibrinolysis, 16(Suppl. 1), S23-S26.
7. Berntorp, E. & Windyga, J.; European Wilate Study Group. (2009) Treatment and prevention of acute bleedings in von Willebrand disease-efficacy and safety of Wilate, a new generation von Willebrand factor/factor VIII concentrate. Haemophilia, 15, 122-130.
8. Blackshear, J.L., Schaff, H.V., Ommen, S.R., Chen, D. & Nichols, W.L. (2011) Hypertrophic obstructive cardiomyopathy, bleeding history, and acquired von Willebrand syndrome: response to septal myectomy. Mayo Clinic Proceedings, 86, 219-224.
9. Bowers, M., McNulty, O. & Mayne, E. (2000) Octreotide in the treatment of gastrointestinal bleeding caused by angiodysplasia in two patients with von Willebrand's disease. British Journal of Haematology, 108, 524-527.
10. van Breevoort, D., van Agtmaal, E.L., Dragt, B.S., Gebbinck, J.K., Dienava-Verdoold, I., Kragt, A., Bierings, R., Horrevoets, A.J., Valentijn, K.M., Eikenboom, J.C., Fernandez-Borja, M., Meijer, A.B. & Voorberg, J. (2012) Proteomic screen identifies IGFBP7 as a novel component of endothelial cell- specific Weibel-Palade bodies. Journal of Proteome Research, 11, 2925-2936.
11. Cass, A.J., Bliss, B.P., Bolton, R.P. & Cooper, B.T. (1980) Gastrointestinal bleeding, angiodysplasia of the colon and acquired von Willebrand's disease. British Journal of Surgery, 67, 639-641.
12. Castaman, G., Federici, A.B., Tosetto, A., La Marca, S., Stufano, F., Mannucci, P.M. & Rodeghiero, F. (2012) Different bleeding risk in type 2 A and 2 M Von Willebrand disease: a two-year prospective study in 107 patients. Journal of Thrombosis & Haemostasis, 10, 632-638.
13. Chey, W.D., Hasler, W.L. & Bockenstedt, P.L. (1992) Angiodysplasia and von Willebrand's disease type IIB treated with estrogen/progesterone therapy. American Journal of Hematology, 41, 276-279.
14. Coppola, A., Cimino, E., Conca, P., De Simone, C., Tufano, A., Tarantino, G., Cerbone, A.M. & Di Minno, G. (2006) Long-term prophylaxis with intermediate-purity factor VIII concentrate (Haemate P) in a patient with type 3 von Willebrand disease and recurrent gastrointestinal bleeding. Haemophilia, 12, 90-94.
15. Federici, A.B. (2008) Prophylaxis of bleeding episodes in patients with von Willebrand's disease. Blood Transfusion, 6(Suppl. 2), s26-s32.
16. Federici, A.B., Stabile, F., Castaman, G., Canciani, M.T. & Mannucci, P.M. (1998) Tretament of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches. Blood, 92, 2707-2711.
17. Federici, A.B., Rand, J.H., Bucciarelli, P., Budde, U., van Genderen, P.J., Mohri, H., Meyer, D., Rodeghiero, F. & Sadler, J.E.; Subcommittee on von Willebrand Factor. (2000) Acquired von Willebrand syndrome: data from an international registry. Thrombosis & Haemostasis, 84, 345-349.
18. Federici, A.B., Gianniello, F. & Mannucci, P.M. (2005) Secondary long-term prophylaxis in severe patients with von Willebrand's disease: an Italian cohort study. Haematologica Reports, 1, 15-20.
19. Federici, A.B., Castaman, G., Franchini, M., Morfini, M., Zanon, E., Coppola, A., Tagliaferri, A., Boeri, E., Mazzucconi, M.G., Rossetti, G. & Mannucci, P.M. (2007) Clinical use of Haemate P in inherited von Willebrand disease: a cohort study on 100 Italian patients. Haematologica, 92, 944-951.
20. Franchini, M. & Lippi, G. (2007a) The role of von Willebrand factor in hemorrhagic and thrombotic disorders. Critical Reviews in Clinical Laboratory Sciences, 44, 115-149.
21. Franchini, M. & Lippi, G. (2007b) Acquired von Willebrand syndrome: an update. American Journal of Hematology, 82, 368-375.
22. Franchini, M., Targher, G. & Lippi, G. (2007) Prophylaxis in von Willebrand disease. Annals of Hematology, 86, 699-704.
23. Fressinaud, E. & Meyer, D. (1993) International survey of patients with von Willebrand disease and angiodysplasia. Thrombosis & Haemostasis, 70, 546.
24. Gritti, G., Cortelezzi, A., Bucciarelli, P., Rezzonico, F., Lonati, S., La Marca, S., Silvestris, I. & Federici, A.B. (2011) Circulating and progenitor endothelial cells are abnormal in patients with different types of von Willebrand disease and correlate with markers of angiogenesis. American Journal of Hematology, 86, 650-656.
25. Heyde, E.C. (1958) Gastrointestinal bleeding in aortic stenosis. New England Journal of Medicine, 259, 196.
26. Hirri, H.M., Green, P.J. & Lindsay, J. (2006) Von Willebrand's disease and angiodysplasia treated with thalidomide. Haemophilia, 12, 285-286.
27. Iannuzzi, M.C., Hidaka, N., Boehnke, M., Bruck, M.E., Hanna, W.T., Collins, F.S. & Ginsburg, D. (1991) Analysis of the relationship of von Willebrand disease (vWD) and hereditary hemorrhagic telangiectasia and identification of a potential type IIA vWD mutation (IIe865 to Thr). American Journal of Human Genetics, 48, 757-763.
28. Lamboley, V., Zabraniecki, L., Sie, P., Pourrat, J. & Fournié, B. (2002) Myeloma and monoclonal gammopathy of uncertain significance associated with acquired von Willebrand's syndrome. Seven new cases with a literature review. Joint Bone Spine, 69, 62-67.
29. Lenting, P., Casari, C., Christophe, O.D. & Denis, C.V. (2012) Von Willebrand factor: the old, the new and the unknown. Journal of Thrombosis & Haemostasis, 10, 2428-2437.
30. Li, M. & Losardo, D.W. (2007) Statins and the endothelium. Vascular Pharmacology, 46, 1-9.
31. Makris, M. (2006) Gastrointestinal bleeding in von Willebrand disease. Thrombosis Research, 118(Suppl 1), S13-S17.
32. Mannucci, P.M., Pareti, F.I., Holmberg, L., Nilsson, I.M. & Ruggeri, Z.M. (1976) Studies on the prolonged bleeding time in von Willebrand's disease. Journal of Laboratory & Clinical Medicine, 88, 662-671.
33. Mannucci, P.M., Franchini, M., Castaman, G. & Federici, A.B.;Italian Association of Hemophilia Centers. (2009) Evidence-based recommendations on the treatment of von Willebrand disease in Italy. Blood Transfusion, 7, 117-126.
34. Morris, E.S., Hampton, K.K., Nesbitt, I.M., Preston, F.E., Thomas, W.E.G. & Makris, M. (2001) The management of von Willebrand's disease associated gastrointestinal dysplasia. Blood Coagulation & Fibrinolysis, 12, 143-148.
35. Nomikou, E., Tsevrenis, V., Gafou, A., Bellia, M. & Theodossiades, G. (2009) Type IIb von Willebrand disease with angiodysplasias and refractory gastrointestinal bleeding successfully treated with thalidomide. Haemophilia, 15, 1340-1342.
36. Odouard, S., Christophe, O.D., de Groot, P.G., Denis, C.V. & Lenting, P.J. (2012) Identification of galectin-1 and galectin-3 as novel partner for von Willebrand factor. Arteriosclerosis Thrombosis & Vascular Biology, 32, 894-901.
37. Pareti, F.I., Lattuada, A., Bressi, C., Zanobini, M., Sala, A., Steffan, A. & Ruggeri, Z.M. (2000) Proteolysis of von Willebrand factor and shear stress-induced platelet aggregation in patients with aortic valve stenosis. Circulation, 102, 1290-1295.
38. Pate, G.E. & Mulligan, A. (2004) An epidemiological study of Heyde's syndrome: an association between aortic stenosis and gastrointestinal bleeding. Journal of Heart Valve Disease, 13, 713-716.
39. Pi, L., Shenoy, A.K., Liu, J., Kim, S., Nelson, N., Xia, H., Hauswirth, W.W., Petersen, B.E., Schultz, G.S. & Scott, E.W. (2012) CCN2/CTGF regulates neovessel formation via targeting structurally conserved cystine knot motifs in multiple angiogenic regulators. FASEB Journal, 26, 3365-3379.
40. Quick, A.J. (1967) Telangiectasia: its relationship to the Minot-von Willebrand syndrome. American Journal of Medical Science, 254, 585.
41. Rahmani, R., Rozen, P., Papo, J., Iellin, A. & Seligsohn, U. (1990) Association of von Willebrand's disease with plasma cell dyscrasia and gastrointestinal angiodysplasia. Israeli Journal of Medical Science, 26, 504-509.
42. Rosborough, T.K. & Swaim, W.R. (1978) Acquired von Willebrand's disease, platelet-release defect and angiodysplasia. American Journal of Medicine, 65, 96-100.
43. Ruggeri, Z.M. (2001) Structure of von Willebrand factor and its function in platelet adhesion and thrombus formation. Best Practice & Research Clinical Haematology, 14, 257-279.
44. Sadler, J.E. (2003) Aortic stenosis, von Willebrand factor and bleeding. New England Journal of Medicine, 349, 323-325.
45. Satoh, Y., Kita, H., Kihira, K., Mutoh, H., Osawa, H., Satoh, K., Ido, K., Sakata, Y. & Sugano, K. (2004) Gastrointestinal angiodysplasia in a patient with type 2 von Willebrand's disease and analysis of exon 28 of the von Willebrand factor gene. American Journal of Gastroenterology, 99, 2495-2498.
46. Siragusa, S., Malato, A., Lo Coco, L., Cigna, V., Saccullo, G., Abbene, I., Anastasio, R., Caramazza, D., Patti, R., Arcara, M. & Di Vita, G. (2008) Gastrointestinal bleeding due to angiodysplasia in patients with type 1 von Willebrand disease: report on association and management. Haemophilia, 14, 150-152.
47. Slaughter, M.S. (2010) Hematologic effects of continuous flow left ventricular assist devices. Journal of Cardiovascular Translational Research, 3, 618-624.
48. Sohal, M. & Laffan, M. (2008) Von Willebrand disease and angiodysplasia responding to atorvastatin. British Journal of Haematology, 142, 309.
49. Starke, R.D., Ferraro, F., Paschalaki, K.E., Dryden, N.H., McKinnon, T.A., Sutton, R.E., Payne, E.M., Haskard, D.O., Hughes, A.D., Cutler, D.F., Laffan, M.A. & Randi, A.M. (2011) Endothelial von Willebrand factor regulates angiogenesis. Blood, 117, 1071-1080.
50. Veyradier, A., Balian, A., Wolf, M., Giraud, V., Montembault, S., Obert, B., Dagher, I., Chaput, J.C., Meyer, D. & Naveau, S. (2001) Abnormal von Willebrand factor in bleeding angiodysplasias of the digestive tract. Gastroenterology, 129, 346-353.
51. Vincentelli, A., Susen, S., Le Tourneau, T., Six, I., Fabre, O., Juthier, F., Bauters, A., Decoene, C., Goudemand, J., Prat, A. & Jude, B. (2003) Acquired von Willebrand syndrome in aortic stenosis. New England Journal of Medicine, 349, 343-349.
52. Warkentin, T.E., Moore, J.C. & Morgan, D.G. (1992) Aortic stenosis and bleeding gastrointestinal angiodysplasia: is acquired von Willebrand's disease the link? Lancet, 340, 35-37.
53. Warkentin, T.E., Moore, J.C., Anand, S.S., Lonn, E.M. & Morgan, D.G. (2003) Gastrointestinal bleeding, angiodysplasia, cardiovascular disease, and acquired von Willebrand syndrome. Transfusion Medicine Reviews, 17, 272-286.
54. Wautier, J.L., Caen, J.P. & Rymer, R. (1976) Angiodysplasia in acquired von Willebrand disease. Lancet, 2, 973.