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Acquired von Willebrand factor abnormalities in myeloproliferative disorders and other hematologic diseases: a retrospective analysis by a single institution.
Haematologica 2002; 87(3):264-70H

Abstract

BACKGROUND AND OBJECTIVES

Acquired von Willebrand syndrome (AVWS) is a rare acquired disorder. In most cases it is associated with lymphoproliferative disorders and monoclonal gammopathies, while less frequently myeloproliferative disorders (MPD) are involved. Although bleeding is the most important symptom, thrombotic complications have also been observed in cases associated with MPD. Our aim was to review the clinical and laboratory findings in AVWS patients from a single institution.

DESIGN AND METHODS

The records of 99 patients with AVWS were reviewed to identify the underlying diseases, the symptoms and the laboratory parameters.

RESULTS

In 75% of cases the AVWS was associated with MPD. The most frequent pattern was type 2 (67.7%). Abnormalities of bleeding time, factor VIII levels or platelet retention to glass beads were observed in 83.8% of cases. Bleeding was present in 38.4% of patients, more frequently in the not-MPD-associated (58.3%) vs. MPD-associated cases (32%) (p=0.022), with a significant predominance in females, irrespective of the underlying disease (p=0.0007). In 32% of patients with MPD, thrombotic manifestations, mostly microvascular and arterial episodes, were observed.

INTERPRETATION AND CONCLUSIONS

AVWS in MPD seems to be mainly a laboratory diagnosis, without clinical symptoms in most cases, although bleeding as well as ischemic events can be present. In contrast, AVWS in not-MPD-associated cases is most frequently associated with severe bleeding symptoms. Performing appropriate laboratory tests may be useful for screening for AVWS.

Authors+Show Affiliations

Hemostasis and Thrombosis Department, Hematologic Research Institute Mariano R. Castex, National Academy of Medicine, Pacheco de Melo 3081 (C1425AUM) Buenos Aires, Argentina. sanchezluceros@hematologia.anm.edu.arNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

11869938

Citation

Sánchez-Luceros, Analía, et al. "Acquired Von Willebrand Factor Abnormalities in Myeloproliferative Disorders and Other Hematologic Diseases: a Retrospective Analysis By a Single Institution." Haematologica, vol. 87, no. 3, 2002, pp. 264-70.
Sánchez-Luceros A, Meschengieser SS, Woods AI, et al. Acquired von Willebrand factor abnormalities in myeloproliferative disorders and other hematologic diseases: a retrospective analysis by a single institution. Haematologica. 2002;87(3):264-70.
Sánchez-Luceros, A., Meschengieser, S. S., Woods, A. I., Blanco, A. N., Kempfer, A. C., Casais, P., ... Lazzari, M. A. (2002). Acquired von Willebrand factor abnormalities in myeloproliferative disorders and other hematologic diseases: a retrospective analysis by a single institution. Haematologica, 87(3), pp. 264-70.
Sánchez-Luceros A, et al. Acquired Von Willebrand Factor Abnormalities in Myeloproliferative Disorders and Other Hematologic Diseases: a Retrospective Analysis By a Single Institution. Haematologica. 2002;87(3):264-70. PubMed PMID: 11869938.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Acquired von Willebrand factor abnormalities in myeloproliferative disorders and other hematologic diseases: a retrospective analysis by a single institution. AU - Sánchez-Luceros,Analía, AU - Meschengieser,Susana S, AU - Woods,Adriana I, AU - Blanco,Alicia N, AU - Kempfer,Ana C, AU - Casais,Patricia, AU - Salviú,María J, AU - Lazzari,María A, PY - 2002/3/1/pubmed PY - 2002/5/25/medline PY - 2002/3/1/entrez SP - 264 EP - 70 JF - Haematologica JO - Haematologica VL - 87 IS - 3 N2 - BACKGROUND AND OBJECTIVES: Acquired von Willebrand syndrome (AVWS) is a rare acquired disorder. In most cases it is associated with lymphoproliferative disorders and monoclonal gammopathies, while less frequently myeloproliferative disorders (MPD) are involved. Although bleeding is the most important symptom, thrombotic complications have also been observed in cases associated with MPD. Our aim was to review the clinical and laboratory findings in AVWS patients from a single institution. DESIGN AND METHODS: The records of 99 patients with AVWS were reviewed to identify the underlying diseases, the symptoms and the laboratory parameters. RESULTS: In 75% of cases the AVWS was associated with MPD. The most frequent pattern was type 2 (67.7%). Abnormalities of bleeding time, factor VIII levels or platelet retention to glass beads were observed in 83.8% of cases. Bleeding was present in 38.4% of patients, more frequently in the not-MPD-associated (58.3%) vs. MPD-associated cases (32%) (p=0.022), with a significant predominance in females, irrespective of the underlying disease (p=0.0007). In 32% of patients with MPD, thrombotic manifestations, mostly microvascular and arterial episodes, were observed. INTERPRETATION AND CONCLUSIONS: AVWS in MPD seems to be mainly a laboratory diagnosis, without clinical symptoms in most cases, although bleeding as well as ischemic events can be present. In contrast, AVWS in not-MPD-associated cases is most frequently associated with severe bleeding symptoms. Performing appropriate laboratory tests may be useful for screening for AVWS. SN - 0390-6078 UR - https://www.unboundmedicine.com/medline/citation/11869938/Acquired_von_Willebrand_factor_abnormalities_in_myeloproliferative_disorders_and_other_hematologic_diseases:_a_retrospective_analysis_by_a_single_institution_ L2 - http://www.haematologica.org/cgi/pmidlookup?view=long&pmid=11869938 DB - PRIME DP - Unbound Medicine ER -