Diagnosis and treatment of von Willebrand's disease

Johnson, Robert; Heldt, Lee; Keaton, Wilfrid M.

doi:10.1016/0278-2391(87)90271-0

Cited by 4 publications

(2 citation statements)

References 11 publications

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“…vWD is often classified into four basic types based on the separation of vWF multimers or subunits of varying molecular weights by electrophoresis. 55 Type I accounts for approximately 85% of occurrences, with all multimeric forms present in reduced concentrations. Type II is characterized by an absence of high-molecular-weight multimers and occurs in 10 to 15% of vWD patients.…”

Section: Factors XIII and I Deficienciesmentioning

confidence: 99%

“…Intermediate-purity F VIII concentrates, FFP, and cryoprecipitate are held in reserve for DDAVP nonresponders. 55 Types II and III require intermediate-purity F VIII concentrates, such as Humate-P or Koate-HS, or, rarely, cryoprecipitate or FFP. Bleeding episodes in patients with platelettype vWD are usually controlled with platelet concentrate infusions.…”

Section: Von Willebrand's Diseasementioning

confidence: 99%

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Bleeding and Clotting Disorders

Napeñas¹,

Patton²

2021

Burket's Oral Medicine

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Dental health care workers are increasingly called upon to provide quality dental care to individuals whose bleeding and clotting mechanisms have been altered by inherited or acquired diseases. This provides an opportunity for the dentist who is trained in the recognition of oral and systemic signs of altered hemostasis to assist in the diagnosis of the underlying condition. A number of dental procedures result in the risk of bleeding that can have serious consequences, such as severe hemorrhage or possibly death, for the patient with a bleeding disorder. Safe dental care may require consultation with the patient's physician, systemic management, and dental treatment modifications.Of the inherited coagulopathies, von Willebrand's disease (vWD) is the most common. It results from deficiency of von Willebrand's factor (vWF) and affects about 0.8 to 1% of the population. 1 Hemophilia A, caused by coagulation factor (F) VIII deficiency, is the next most common, followed by hemophilia B, a F IX deficiency. The age-adjusted prevalence of hemophilia in six surveillance states in 1994 was 13.4 cases in 100,000 males (10.5 for hemophilia A and 2.9 for hemophilia B). 2 Application to the US population resulted in an estimated national prevalence of 13,320 cases of hemophilia A and 3,640 cases of hemophilia B, with an incidence rate of 1 per 5,032 live male births. Hemophilia A was predominant, accounting for 79% of all hemophiliacs, and prevalence of disease severity was 43% severe (< 1% F VIII), 26% moderate (1-5% F VIII), and 31% with mild (6-30% F VIII) disease. 2 Acquired coagulation disorders can result from drug actions or side effects, or underlying systemic disease. A stratified household sample of 4,163 community residents aged 65 years or older living in a five-county area of North Carolina revealed 51.7% to be taking one or more medications (aspirin, warfarin, dipyridamole, nonsteroidal anti-inflammatory drugs

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Section: Factors XIII and I Deficienciesmentioning

confidence: 99%

Section: Von Willebrand's Diseasementioning

confidence: 99%

Bleeding and Clotting Disorders

Napeñas¹,

Patton²

2021

Burket's Oral Medicine

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Treatment of Patients With Bleeding Disorders

Patton

Ship

1994

Dental Clinics of North America

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Evaluation of the PFA-100® System in the Diagnosis and Therapeutic Monitoring of Patients with von Willebrand Disease

Cattaneo¹,

Federici²,

Lecchi³

et al. 1999

Thromb Haemost

112

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SummaryWe have evaluated platelet function at high shear with the PFA-100® system in different subtypes of von Willebrand disease (vWD), before and after the intravenous infusions of desmopressin or a factor-VIII/von Willebrand factor (vWF) concentrate. Closure times with the PFA-100® system were determined for both the collagen/ADP and the collagen/epinephrine cartridges in 52 patients with vWD (9 type 1 “platelet normal”, 5 type 1 “platelet-discordant”, 8 type 1 “platelet-low”, 6 type 2A, 9 type 2B, 6 type 2M Vicenza, 6 type 3 and 3 acquired vWD) and 40 controls. Measurements were repeated 1 and 4 h after the i.v. infusion of desmopressin (0.3 μg/Kg) in 26 patients with types 1, type 2M Vicenza or type 2A vWD, or of a factorVIII/vWF concentrate (Alphanate HT, 60 U/Kg) in 4 patients with type 3 vWD. At all time points, vWF plasma levels and the bleeding time (Symplate II) were also determined. Baseline closure times were longer in vWD patients than in controls with both the collagen/ADP and the collagen/ epinephrine cartridges. The sensitivity of the PFA-100® system (88% and 87% with the two cartridges) was higher than that of the bleeding time (65%). Treatment with desmopressin normalized the closure times in patients with type 1 “platelet-normal” or type 2M Vicenza vWD, had no significant effects in patients with type 1 “platelet-low”, type 1 “platelet-discordant” or type 2A vWD. Infusion of a factorVIII/vWF concentrate in patients with type 3 vWD slightly shortened their prolonged closure times. In general, changes in PFA-100® were paralleled by shortenings of the bleeding times and increases in plasma vWF levels. The PFA-100® test reflects vWF-dependent platelet function under high shear stress and could be useful in the diagnosis and therapeutic monitoring of patients with vWD.

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Diagnosis and treatment of von Willebrand's disease

Cited by 4 publications

References 11 publications

Bleeding and Clotting Disorders

Bleeding and Clotting Disorders

Treatment of Patients With Bleeding Disorders

Evaluation of the PFA-100® System in the Diagnosis and Therapeutic Monitoring of Patients with von Willebrand Disease

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