Association of acquired von Willebrand syndrome with AL amyloidosis.

• Abstract: Acquired loss of functional von Willebrand factor (VWF) has been termed the acquired von Willebrand syndrome (AVWS). AVWS is a rare adult-onset bleeding diathesis that is clinically similar to congenital von Willebrand disease (VWD), and occurs with a variety of autoimmune, lymphoproliferative, or myeloproliferative disorders. We have identified four patients with AVWS in association with immunoglobulin light chain (AL) amyloidosis. These patients, lacking any pre-existing or family history of abnormal bleeding, developed cutaneous, mucosal, or gastrointestinal bleeding in the course of their disease without deficiency of clotting factor X or other factors; the activated partial thromboplastin time (aPTT) was prolonged in three out of the four cases. Despite normal VWF antigen levels, VWF ristocetin cofactor activity (VWF:RCo) was low. Electrophoresis patterns of high molecular weight (HMW) VWF multimers were abnormal in two of the four cases. Two of the patients were treated with high-dose intravenous melphalan followed by autologous stem cell transplantation (HDM/SCT) and achieved hematologic remission. In these two patients, the bleeding diathesis improved and the coagulation parameters normalized, confirming a causal relationship between the plasma cell dyscrasia and the AVWS. AVWS should be considered in AL amyloidosis patients with hemorrhagic diatheses and normal clotting factor levels.
• Authors: Cynthia A Kos, Jennifer E Ward, Karim Malek, Vaishali Sanchorawala, Daniel G Wright, Carl O'Hara, Lawreen Connors, Martha Skinner, David C Seldin (Affiliation: Department of Medicine, Boston University Medical Center, Boston, Massachusetts, USA.)
• Journal: American journal of hematology (Am J Hematol) Vol. 82 Issue 5 Pg. 363-7 (May 2007) ISSN: 0361-8609 [Print] United States
• PMID: 17205535 (Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
• Copyright: (c) 2007 Wiley-Liss, Inc.

Chemical References

• Antigens
• Immunoglobulin Light Chains
• Von Willebrand antigen
• von Willebrand Factor
• Ristocetin
• Melphalan

Topics

• Adult
• Amyloidosis (blood, complications, drug therapy, surgery)
• Antigens (analysis)
• Blood Protein Electrophoresis
• Electrophoresis, Agar Gel
• Hemorrhage (etiology)
• Humans
• Immunoglobulin Light Chains (analysis)
• Male
• Melphalan (therapeutic use)
• Molecular Weight
• Partial Thromboplastin Time
• Peripheral Blood Stem Cell Transplantation
• Remission Induction
• Ristocetin (pharmacology)
• Transplantation, Autologous
• von Willebrand Diseases (etiology, immunology)
• von Willebrand Factor (immunology)