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Angiodysplasie digestive révélant un syndrome de Willebrand acquis associé à un myélome multiple A propos d’un cas

Digestive angiodysplasia revealing an acquired von Willebrand syndrome associated to a multiple myeloma

  • Cas Cliniques
  • Published:
Acta Endoscopica

Résumé

Les angiodysplasies digestives ont fait l’objet de nombreux travaux et suscitent encore plusieurs controverses sur la pathogénie des lésions, leur histoire naturelle et leur traitement. Leur association au syndrome de Willebrand acquis (AvWS) est rapportée, mais demeure inexpliquée. Le syndrome de Willebrand acquis est un syndrome hémorragique rare. Parmi les affections sous-jacentes à l’origine de ce syndrome, le myélome multiple est le plus fréquent. L’apparition du AvWS est liée soit à la présence d’anticorps dirigés contre le facteur von Willebrand (vWF), soit à une adsorption sur des cellules tumorales.

Le traitement est celui du myélome multiple ou peut-être symptomatique faisant appel à la desmopressine, aux concentrés plasmatiques de vWF, ou à des immunoglobulines en intraveineuse à forte dose.

Nous rapportons le cas d’un patient de 78 ans, ayant présenté des rectorragies massives en rapport avec une angiodysplasie intestinale diffuse et chez qui un syndrome de Willebrand acquis associé un myélome multiple a été diagnostiqué.

Summary

Digestive angiodysplasia was and still is the subject of many works and still causes several controversies on the pathogenesis of the lesions, their natural history and their treatment. Its association with the acquired von Willebrand syndrome (AvWS) is reported, but remains unexplained. The AvWS is a rare hemorrhagic syndrome. Among the underlying affections of this syndrome, the multiple myeloma is the most frequent. The occurrence of the AvWS is related either to the presence of antibodies directed against the von Willebrand factor (vWF), or to an adsorption on tumoral cells. The treatment is that of the myeloma or only symptomatic using desmopressin, vWF concentrates, or high doses of intravenous immunoglobulins.

We report the case of a 78-year old patient with massive intestinal bleeding due to a diffuse digestive angiodysplasia and in whom an AvWS associated to a multiple myeloma has been diagnosed.

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Références

  1. Boley SJ, Sammartano R, Adams A, Dibiase A, Kleinhaus S, Sprayregen S. On the nature and etiology of vascular ectasias of the colon. Degenerative lesions of aging. Gastroenterology 1977;72:650–60.

    PubMed  CAS  Google Scholar 

  2. Foutch P, Rex D. Prevalence of colonic angiodysplasia among healthy, asymptomatic people. Am J Gastroenterol 1992;87:62–6.

    Google Scholar 

  3. Barbier J, Demont H. Angiodysplasies digestives. Encycl Méd Chir, Gastro-entérologie, 9-006-b-10, 2002.

  4. Hochter W, Weingart J. Angiodysplasia in the colon and rectum: endoscopic morphology, localization and frequency. Endoscopy 1985;17:182–5.

    PubMed  CAS  Google Scholar 

  5. Gilmare P. Angiodysplasia of the upper gastrointestinal tract. J Clin Gastroenterol 1988;10:86–94.

    Google Scholar 

  6. Blutani M, Gupha S. A prospective controlled evaluation of endoscopic detection of angiodysplasia and its association with aortic valve disease. Gastrointest Endosc 1995;42:389–402.

    Google Scholar 

  7. Zuckerman G, Cornette G, Upper gastrointestinal bleeding in patients with chronic renal failure. Ann Intern Med 1985;102:588–92.

    PubMed  CAS  Google Scholar 

  8. Fressinaud E, Meyer D. À propos de l’association entre maladie de Willebrand et angiodysplasie. Sang Thrombose Vaisseaux 1993;5:287–9.

    Google Scholar 

  9. Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand’s disease. Blood 1987;69:454–9.

    PubMed  CAS  Google Scholar 

  10. Kumar S, Pruthi RK, Nichols WL. Acquired von Willebrand’s syndrome: a single institution experience. Am J Hematol 2003;72:243–7.

    Article  PubMed  Google Scholar 

  11. Federici A.B, Rand JH. Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost 2000;84:345–9.

    PubMed  CAS  Google Scholar 

  12. Simone JV, Cornet JA. Acquired von Willebrand’s syndrome in systemic lupus erythematous. Blood 1968;31:806–12.

    PubMed  CAS  Google Scholar 

  13. Mohri H. Acquired von Willebrand Syndrome: its pathophysiology, laboratory features and management. J Thromb Thrombolysis 2003;15:141–9.

    Article  PubMed  CAS  Google Scholar 

  14. Lamboley V, Zabraniecki L, Sie P, Pourrat J, Fournié B. Myélome et gammapathie monoclonale de signification indéterminée associés à un syndrome de Willebrand acquis. Sept nouvelles observations et revue de la littérature. Rev Rhum, 2002(69): p. 69–75

  15. Mohri, H, Motomura S. Clinical significance of inhibitors in acquired von Willebrand syndrome. Blood 1998;10:3623–9.

    Google Scholar 

  16. Mannucci PM, Lombardi R, Bader R, Horellou MH, Einazzi G, Cesana C, Conrad J, Samama M. Studies of the pathophysiology of acquired von Willebrand’s disease in seven patients with lymphoproliferative disorders or benign monoclonal gammopathies. Blood 1984;64:614–21.

    PubMed  CAS  Google Scholar 

  17. Veyradier A, Jenkins C. Acquired von Willebrand syndrome: from pathophysiology to management. Thromb Haemost 2000;84:175–82.

    PubMed  CAS  Google Scholar 

  18. Borill EG, Erschler WB, Golden GA, Tindle BH, Edson JR. A human myeloma produced monoclonal protein directed against the active subpopulation of von Willebrand factor. Am J Pathol 1986;85:115–23.

    Google Scholar 

  19. Mohri H, Tanabe J, Ohtsuka M, Yoshida M, Motomura S, Nishida S, Fujimura Y, Okudo T. Acquired von Willebrand disease associated with a multiple myeloma; characterization of an inhibitor to von Willebrand factor. Blood Coag Fibrin 1995; 6:561–6.

    Article  CAS  Google Scholar 

  20. Mohri H, Hisanaga S, Mishima A, Fujimoto S, Uezono S, Okudo T. Autoantibody inhibits binding of von Willebrand factor to glycoprotein Ib and collagen in multiple myeloma: recognition sites present on the A1 loop and A3 domains of von Willebrand factor. Blood Coag Fibrin 1998; 9: 91–7.

    Article  CAS  Google Scholar 

  21. Richard C, Cuadrado MA, Prieto M et al. Acquired von Willebrand disease in multiple myeloma secondary to absorption of von Willebrand factor by plasma cells. Am J Hematol 1990;35:114–7.

    Article  PubMed  CAS  Google Scholar 

  22. Tefferi A, Hanson CA, Kurtin PJ, Katzmann JA, Dalton RJ, Nichols WL. Acquired von Willebrand’s disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells. Br J Haematol 1997; 96:850–3.

    Article  PubMed  CAS  Google Scholar 

  23. Le François C, Dubus L. Hémorragie digestive révélatrice d’une angiodysplasie et d’une maladie de von Willebrand de type 2. Ann Fr Anesth Réanim 1997;16:978–81.

    Google Scholar 

  24. Tefferi A, Nichols WL. Acquired von Willebrand disease: concise review of occurrence, diagnosis, pathogenesis, and treatment. Am J Med 1997;103:536–40.

    Article  PubMed  CAS  Google Scholar 

  25. Michiels JJ, Budde U, van der Planken M, van Vliet H, Schroyens W, Berneman Z, Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. Best Pract Res Clin Haematol 2001;14:401–36.

    Article  PubMed  CAS  Google Scholar 

  26. McGrath, KM, Johnson CA, Stuart JJ. Acquired von Willebrand disease associated with an inhibitor to factor VIII antigen and gastrointestinal telangectasia. Am J Med 1997;67:693.

    Article  Google Scholar 

  27. Siaka C, Rugeri L, Caron C, Goudemand J. A new ELISA assay for diagnosis of acquired von Willebrand syndrome. Haemophilia 2003;9:303–8.

    Article  PubMed  CAS  Google Scholar 

  28. Mc Grawth KM, Johnson CA, Stuart JJ. Acquired von Willebrand disease associated with an inhibitor to factor VIII antigen and gastrointestinal telangiectasia. Am J Med 1979;67:693–6.

    Article  Google Scholar 

  29. Ramsay DH, Buist TA. Persistent gastrointestinal bleeding toe to angiodysplasia of the gut in Von Willebrand disease. Lancet 1976;2:275–78.

    Article  PubMed  CAS  Google Scholar 

  30. Wautier JL, Caen JP, Rymer R. Angiodysplasia in acquired von Willebrand disease. Lancet 1976;2:973.

    Article  PubMed  CAS  Google Scholar 

  31. Rosborough TK, Swaim WR. Acquired von Willebrand’s disease, platelet-release defect and angiodysplasia. Am J Med 1978;65:96–100.

    Article  PubMed  CAS  Google Scholar 

  32. Cass AJ, Bliss BP, Bolton RP, Cooper BT. Gastrointestinal bleeding, angiodysplasia of the colon and acquired von Willebrand’s disease. Br J Surg 1980; 67:639–41.

    Article  PubMed  CAS  Google Scholar 

  33. Fressinaud E, Meyer D. International survey of patients with von Willebrand disease and angiodysplasia [letter]. Thromb Haemost 1994;71:527.

    Google Scholar 

  34. Salah A, Alhumood D, Devine V, Lawson LW. Idiopathic Immune-Mediated Acquired von Willebrand’s Disease in a Patient With Angiodysplasia: Demonstration of an Unusual Inhibitor Causing a Functional Defect and Rapid Clearance of von Willebrand Factor. American Journal of Hematology 1999;60:151–7.

    Article  Google Scholar 

  35. Mahapatra M, Mishra P, Makharia G, Kumar R, Saxena R Acquired von Willebrand’s disease associated with gastrointestinal angiodysplasia and monoclonal gammopathy. J Assoc Physicians India 2006;54:963.

    Google Scholar 

  36. Gupta PK, Kannan M, Chatterjee T, et al. Acquired von Willebrand’s disease associated with gastrointestinal angiodysplasia: a case report. Haemophilia 2006;12:452–5.

    Article  PubMed  CAS  Google Scholar 

  37. Duray P, Marcal JM. Gastrointestinal angiodysplasia: a possible component of von Willebrand’s disease. Hum Pathol 1984;15:539–44.

    Article  PubMed  CAS  Google Scholar 

  38. Moake JL, Turner NA, Stathopoulos NA, Nolasco LH, Hellums JD. Involvement of large plasma von Willebrand factor (vWF) multimers and unusually large vWF form derived from endothelial cells in shear stress-induced platelet aggregation. J Clin Invest 1986;78: 1456–61.

    Article  PubMed  CAS  Google Scholar 

  39. Inbal A, Bank I, Zivelin A, Varon D, Dardik R, Shapiro R. Acquired von Willebrand disease in a patient with angiodysplasia resulting from immune-mediated clearance of von Willebrand factor. British Journal of Haematology 1997;96:179–82.

    Article  PubMed  CAS  Google Scholar 

  40. Coleman LS. A capillary hemostasis mechanism regulated by sympathetic tone and activity via factor VIII or von Willebrand’s factor may function as a “capillary gate” and may explain angiodysplasia, angioneurotic edema, and variations in systemic vascular resistance. Med Hypotheses 2006;66:773–5.

    Article  PubMed  CAS  Google Scholar 

  41. Lecleirea S, Di Fiorea F, Ben Soussana E, Borgb JY, Antoniettia J, Lereboursa E. The use of wireless capsule endoscopy to detect small bowel angiodysplasia in a patient with obscure gastrointestinal bleeding and von Willebrand disease. European Journal of Gastroenterology & Hepatology 2004;16:115–16.

    Article  Google Scholar 

  42. Ell C, Remke S, May A, Helou L, Henrich R, Mayer G. The first prospective controlled trial comparing wireless capsule endoscopy with push enteroscopy in chronic gastrointestinal bleeding. Endoscopy 2002;34:685–9.

    Article  PubMed  CAS  Google Scholar 

  43. Makris M. Gastrointestinal bleeding in von Willebrand disease. Thromb Res 2006; 118 [Suppl 1]:S13–7.

    Article  PubMed  CAS  Google Scholar 

  44. Takahashi H, Nagayama R, Tanabe Y. et al. DDAVP in acquired von Willebrand syndrome associated with multiple myeloma. Am J Hematol, 1986(22): p. 421–9.

  45. Federici AB., Stabile F, Castaman G, Canciani MT, Mannucci PM. Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches. Blood 1998;92:2707–11.

    PubMed  CAS  Google Scholar 

  46. Kumar S, Pruthi RK, Nichols WL. Acquired von Willebrand disease. Mayo Clin Proc 2002;77:181–7.

    PubMed  Google Scholar 

  47. Stewart AK, Glynn MF. Acquired von Willebrand disease associated with free lambda light monoclonal gammopathy, normal bleeding time and response to prednisone. Postgrad Med J 1990;66:560.

    PubMed  CAS  Google Scholar 

  48. Jehlinger J, Rose E, Aledort LM. Successful treatment of an acquired von Willebrand’s factor antibody by experimental immunoadsorption. N Engl J Med 1989;320: 254.

    Google Scholar 

  49. Friederich PW, Wever PC, Briet E, Doorenbos CJ, Levi M. Successful treatment with recombinant factor VIIa of therapy resistant severe bleeding in a patient with acquired von Willebrand disease. Am J Hematol 2001;66:292–4.

    Article  PubMed  CAS  Google Scholar 

  50. Heidt J, Langers A, van der Meer F, Brouwer R. Thalidomide as treatment for digestive tract angiodysplasias. Neth J Med 2006;64:425–8.

    PubMed  CAS  Google Scholar 

  51. Gross S, Traulle C, Capiod JC et al. Efficacy of high-dose intravenous gammaglobulin in the management of acquired von Willebrand’s disease during orthopaedic surgery. Br J Haematol 1992;8:170–1.

    Article  Google Scholar 

  52. Arkel YS, Lynch J, Kamiyama M. Treatment of acquired von Willebrand syndrome with intravenous immunoglobulin [letter]. Thromb Haemost 1994;72:643.

    PubMed  CAS  Google Scholar 

  53. Castaman G, Tosetto A, Rodeghiero F. Effectiveness of high-dose intravenous immunoglobulin in a case of acquired von Willebrand syndrome with chronic melena not responsive to desmopressin and factor VIII concentrate. Am J Hematol 1992;41:132.

    Article  PubMed  CAS  Google Scholar 

  54. Van Genderen PJ, Papatsonis DN, Michiels JJ, Wielenga JJ, Stibbe J, Huikeshoven FJ. High-dose intravenous gammaglobulin therapy for acquired von Willebrand disease. Postgrad Med J 1994;70:916.

    Article  PubMed  Google Scholar 

  55. Whit LA, Chisholm M. Gastrointestinal bleeding in acquired von Willebrand’s disease: efficacy of high-dose immunoglobulin where substitution treatment failed. Br J Haematol 1993;84:332.

    Article  Google Scholar 

  56. Delmer A, Horellou M, Brechot J, Prudent J, Potevin F, Lecrubier C, Girard-Longhinin C, Samama M, Zittoun R. Acquired von Willebrand disease: correction of hemostatic defect by high-dose intravenous immunoglobulins. Am J Hematol 1992;40:151.

    Article  PubMed  CAS  Google Scholar 

  57. Briat A, Tapon-Bretaudière J. Le syndrome de Willebrand acquis. Hématologie 2005; 11:111–6.

    Google Scholar 

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Author notes

  1. Sanae Reggoug

    Present address: Clinique médicale B, Hépato-Gastroentérologie et Proctologie, CHU Ibn-Sina, Rabat, Maroc

Authors and Affiliations

  1. Clinique médicale B, Hépato-Gastroentérologie et Proctologie, CHU Ibn-Sina, Rabat, Maroc

    Ikrame Errabih, Nadia Benzzoubeir, Hayat Krami, Laziza Ouazzani & Houria Ouazzani

  2. Service des Explorations Fonctionnelles Digestives, CHU Ibn-Sina, Rabat, Maroc

    Naima Amrani

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Reggoug, S., Errabih, I., Benzzoubeir, N. et al. Angiodysplasie digestive révélant un syndrome de Willebrand acquis associé à un myélome multiple A propos d’un cas. Acta Endosc 37, 547–557 (2007). https://doi.org/10.1007/BF02961801

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