CASE REPORT
Acquired von Willebrand syndrome associated with plasma cell myeloma – a rare histopathological bone marrow image
 
More details
Hide details
1
Department of Diagnostic Hematology, Institute of Hematology and Transfusion Medicine, Warsaw, Poland
 
 
Submission date: 2018-10-17
 
 
Final revision date: 2019-05-03
 
 
Acceptance date: 2019-06-14
 
 
Publication date: 2020-03-06
 
 
Pol J Pathol 2019;70(4):311-316
 
KEYWORDS
TOPICS
ABSTRACT
Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that is associated with a variety of underlying diseases. We report a case of AVWS associated with plasma cell myeloma. The patient was a 57-year-old male with recurrent bleeding symptoms for a few months. Physical examination was normal. Laboratory studies revealed isolated prolongation of the activated partial thromboplastin time. His factor VIII activity, von Willebrand factor (VWF) Ag, and VWF activity were low. Bone marrow aspirate showed diffuse infiltration of atypical plasma cells and erythroid line hyperplasia.
REFERENCES (34)
1.
Kumar S, Pruthi RK, Nichols WL. Acquired von Willebrand disease. Mayo Clin Proc 2002; 77: 181-187.
 
2.
Collins P, Budde U, Rand JH, et al. Epidemiology and general guidelines of the management of acquired haemophilia and von Willebrand syndrome. Hemophilia 2008; 14 Suppl 3: 49-55.
 
3.
Tefferi A, Nichols WL. Acquired von Willebrand disease: concise review of occurrence, diagnosis, pathogenesis, and treatment. Am J Med 1997; 103: 536-540.
 
4.
Rajkumar SV, Dimopoulos MA, Palumbo A, et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol 2014; 15: e538-48.
 
5.
Hennessy BJ, White B, Byrne M, Smith OP. Acquired von Willebrand’s disease. Ir J Med Sci 1998; 167: 81-85.
 
6.
Lamboley V, Zabraniecki L, Sie P, et al. Myeloma and monoclonal gammopathy of uncertain significance associated with acquired von Willebrand’s syndrome. Seven new cases with a literature review. Joint Bone Spine 2002; 69: 62-67.
 
7.
Voisin S, Hamidou M, Lefrancois A, et al. Acquired von Willebrand syndrome associated with monoclonal gammopathy: a single-center study of 36 patients. Medicine 2011; 90: 404-411.
 
8.
Dicke C, Schneppenheim S, Holstein K, et al. Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias. Ann Hematol 2016; 95: 945-957.
 
9.
Djunic I, Elezovic I, Ilic V, et al. Acquired von Willebrand syndrome in multiple myeloma Pathogenesis and management of bleeding and thrombosis in plasma cell dyscrasias Immune-mediated etiology of acquired von Willebrand syndrome in systemic lupus erythematosus and in benign monoclonal gammopathy: therapeutic implications. Hematology 2011; 16: 209-212.
 
10.
Federici AB. Acquired von Willebrand syndrome: an underdiagnosed and misdiagnosed bleeding complication in patients with lymphoproliferative and myeloproliferative disorders. Semin Hematol 2006; 43 (1 Suppl 1): S48-S58.
 
11.
Jennes E, Guggenberger D, Zotz R, et al. Perioperative intravenous immunoglobulin treatment in a patient with severe acquired von Willebrand syndrome: case report and review of the literature. Clin Case Rep 2017; 5: 664-670.
 
12.
Jin N, Salahuddin FF, Nesbitt JA. Acquired von Willebrand disease and multiple myeloma: a case report of a breast cancer survivor. Blood Coagul Fibrinolysis 2014; 25: 890-893.
 
13.
RicoRios N, Bowles L, Ayling RM. Multiple myeloma and acquired von Willebrand disease: a combined cause of preanalytical interference causing gel formation? Ann Clin Biochem 2018; 55: 181-184.
 
14.
Shinagawa A, Kojima H, Berndt MC, et al. Characterization of a myeloma patient with a life-threatening hemorrhagic diathesis: presence of a lambda dimer protein inhibiting shear-induced platelet aggregation by binding to the A1 domain of von Willebrand factor. Thromb Haemost 2005; 93: 889-896.
 
15.
Jakway JL. Acquired von Willebrand’s disease. Hematol Oncol Clin North Am 1992; 6: 1409-1419.
 
16.
Jakway JL. Acquired von Willebrand’s disease in malignancy. Semin Thromb Hemost 1992; 18: 434-439.
 
17.
Nitu-Whalley IC, Lee CA. Acquired von Willebrand syndrome – report of 10 cases and review of the literature. Haemophilia 1999; 5: 318-326.
 
18.
Shetty S, Kasatkar P, Ghosh K. Pathophysiology of acquired von Willebrand disease: a concise review. Eur J Haematol 2011; 87: 99-106.
 
19.
Basnet S, Lin C, Dhital R, et al. Acquired von Willebrand Disease Associated with Monoclonal Gammopathy of Unknown Significance. Case Rep Oncol Med 2017; 2017: 9295780.
 
20.
Bovill EG, Ershler WB, Golden EA, et al. A human myeloma-produced monoclonal protein directed against the active subpopulation of von Willebrand factor. Am J Clin Pathol 1986; 85: 115-123.
 
21.
Mohri H, Tanabe J, Ohtsuka M, et al. Acquired von Willebrand disease associated with multiple myeloma; characterization of an inhibitor to von Willebrand factor. Blood Coagul Fibrinolysis 1995; 6: 561-566.
 
22.
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: 114-117.
 
23.
Eby CS. Bleeding and thrombosis risks in plasma cell dyscrasias. Hematology Am Soc Hematol Educ Program 2007; 2007: 158-164.
 
24.
Mohri H, Motomura S, Kanamori H, et al. Clinical significance of inhibitors in acquired von Willebrand syndrome. Blood 1998; 91: 3623-3629.
 
25.
Branchford BR, Di Paola J. Making a diagnosis of VWD. Hematology Am Soc Hematol Educ Program 2012; 2012: 161-167.
 
26.
Ng C, Motto DG, Di Paola J. Diagnostic approach to von Willebrand disease. Blood 2015; 125: 2029-2037.
 
27.
Bain BJ, Clark DM, Wilkins BS. Bone Marrow Pathology. John Wiley & Sons 2019.
 
28.
Auwerda JJ, Sonneveld P, Leebeek FW. Temporary relief of symptomatic Von Willebrand disease by multiple myeloma. J Thromb Haemost 2005; 3: 1088-1089.
 
29.
Lavin M, Brophy TM, Rawley O, et al. Lenalidomide as a novel treatment for refractory acquired von Willebrand syndrome associated with monoclonal gammopathy. J Thromb Haemost 2016; 14: 1200-1205.
 
30.
Ojeda-Uribe M, Caron C, Itzhar-Baikian N, et al. Bortezomib effectiveness in one patient with acquired von Willebrand syndrome associated to monoclonal gammopathy of undetermined significance. Am J Hematol 2010; 85: 396.
 
31.
Hayashi T, Yagi H, Suzuki H, et al. Low-dosage intravenous immunoglobulin in the management of a patient with acquired von Willebrand syndrome associated with monoclonal gammopathy of undetermined significance. Pathophysiol Haemost Thromb 2002; 32: 33-39.
 
32.
Katagiri S, Akahane D, Amano K, Ohyashiki K. Long-term remission of acquired von Willebrand syndrome associated with multiple myeloma using bortezomib and dexamethasone therapy. Haemophilia 2016; 22: e557-e559.
 
33.
Takahashi H, Nagayama R, Tanabe Y, et al. DDAVP in acquired von Willebrand syndrome associated with multiple myeloma. Am J Hematol 1986; 22: 421-429.
 
34.
Tiede A, Rand JH, Budde U, et al. How I treat the acquired von Willebrand syndrome. Blood 2011; 117: 6777-6785.
 
eISSN:2084-9869
ISSN:1233-9687
Journals System - logo
Scroll to top