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Acquired von Willebrand's syndrome: a single institution experience.
Am J Hematol. 2003 Apr; 72(4):243-7.AJ

Abstract

Acquired von Willebrand's disease or syndrome (AVWS) is a rare bleeding disorder distinguished from congenital von Willebrand's disease by age at presentation and absence of personal and family history of bleeding disorders. We report on 22 patients with AVWS seen over 25 years. Mean age at diagnosis was 61.3 years (range 38-86 years); most patients had a spontaneous or a post-operative hemorrhage at presentation. Gastrointestinal bleeding and epistaxis were the most common spontaneous symptoms. Bleeding time was prolonged in most patients, associated with marked reductions in plasma von Willebrand factor antigen and ristocetin cofactor activity. Plasma VWF multimer distribution was normal (type 1 pattern) in 5 patients, indeterminate (no multimers detectable) in 6 patients (type 3 pattern), and abnormal (decreased higher-molecular-weight multimers, type 2 pattern) in 11 patients. None of 17 patients tested had an inhibitor of ristocetin cofactor activity. An underlying malignant or benign hematologic disease was found in 18 patients, and 1 patient had Crohn's disease. Desmopressin was effective in only half the patients so treated, but all patients responded to treatment with VWF-containing concentrates. Resolution of AVWS occurred with therapy of lymphoma (1 patient) and chronic lymphocytic leukemia (1 patient). Sixteen patients were alive at last follow-up; no deaths were related to bleeding. AVWS may be more prevalent than has been appreciated; we estimate up to 0.04%. Awareness of the existence of AVWS is essential for diagnosis and appropriate management. Therapy of associated diseases may improve the bleeding disorder.

Authors+Show Affiliations

Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

12666134

Citation

Kumar, Shaji, et al. "Acquired Von Willebrand's Syndrome: a Single Institution Experience." American Journal of Hematology, vol. 72, no. 4, 2003, pp. 243-7.
Kumar S, Pruthi RK, Nichols WL. Acquired von Willebrand's syndrome: a single institution experience. Am J Hematol. 2003;72(4):243-7.
Kumar, S., Pruthi, R. K., & Nichols, W. L. (2003). Acquired von Willebrand's syndrome: a single institution experience. American Journal of Hematology, 72(4), 243-7.
Kumar S, Pruthi RK, Nichols WL. Acquired Von Willebrand's Syndrome: a Single Institution Experience. Am J Hematol. 2003;72(4):243-7. PubMed PMID: 12666134.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Acquired von Willebrand's syndrome: a single institution experience. AU - Kumar,Shaji, AU - Pruthi,Rajiv K, AU - Nichols,William L, PY - 2003/4/1/pubmed PY - 2003/4/22/medline PY - 2003/4/1/entrez SP - 243 EP - 7 JF - American journal of hematology JO - Am. J. Hematol. VL - 72 IS - 4 N2 - Acquired von Willebrand's disease or syndrome (AVWS) is a rare bleeding disorder distinguished from congenital von Willebrand's disease by age at presentation and absence of personal and family history of bleeding disorders. We report on 22 patients with AVWS seen over 25 years. Mean age at diagnosis was 61.3 years (range 38-86 years); most patients had a spontaneous or a post-operative hemorrhage at presentation. Gastrointestinal bleeding and epistaxis were the most common spontaneous symptoms. Bleeding time was prolonged in most patients, associated with marked reductions in plasma von Willebrand factor antigen and ristocetin cofactor activity. Plasma VWF multimer distribution was normal (type 1 pattern) in 5 patients, indeterminate (no multimers detectable) in 6 patients (type 3 pattern), and abnormal (decreased higher-molecular-weight multimers, type 2 pattern) in 11 patients. None of 17 patients tested had an inhibitor of ristocetin cofactor activity. An underlying malignant or benign hematologic disease was found in 18 patients, and 1 patient had Crohn's disease. Desmopressin was effective in only half the patients so treated, but all patients responded to treatment with VWF-containing concentrates. Resolution of AVWS occurred with therapy of lymphoma (1 patient) and chronic lymphocytic leukemia (1 patient). Sixteen patients were alive at last follow-up; no deaths were related to bleeding. AVWS may be more prevalent than has been appreciated; we estimate up to 0.04%. Awareness of the existence of AVWS is essential for diagnosis and appropriate management. Therapy of associated diseases may improve the bleeding disorder. SN - 0361-8609 UR - https://www.unboundmedicine.com/medline/citation/12666134/Acquired_von_Willebrand's_syndrome:_a_single_institution_experience_ L2 - https://doi.org/10.1002/ajh.10298 DB - PRIME DP - Unbound Medicine ER -