Quantitative analysis of desmopressin (DDAVP) response in adult patients with type 1 von Willebrand disease

Achuta Kumar Guddati, Rachel P. Rosovsky, Elizabeth M. Van Cott, David J. Kuter

Research output: Contribution to journalArticle

Abstract

Introduction: Patients with Type 1 von Willebrand disease (VWD) have reduced amounts of von Willebrand factor (VWF) in their blood. Desmopressin (DDAVP) has been used to raise the blood levels of VWF in these patients. However, not all patients with Type 1 VWD are known to respond to DDAVP therapy. We sought to compare the levels of Factor VIII, VWF antigen (VWF:Ag), VWF ristocetin cofactor (VWF:RCo), and fibrinogen at different time points after administration of DDAVP. Methods: A retrospective analysis was conducted at a single institution on 89 patients with Type 1 VWD who received a single intravenous dose of 0.3 μg/kg DDAVP. Levels of Factor VIII, VWF:Ag, VWF:RCo, and fibrinogen were measured before and then 30, 60, 90, and 120 minutes after completion of DDAVP administration. Results: Median levels of Factor VIII, VWF:Ag, and VWF:RCo were significantly elevated (P < 0.001) at 30 (175 IU/dL, 111 IU/dL, 127 IU/dL), 60 (218 IU/dL, 138 IU/dL, 139 IU/dL), and 90 (212 IU/dL, 134 IU/dL, 130 IU/dL) minutes time points when compared to their respective baseline values (71 IU/dL, 54 IU/dL, 54 IU/dL). At 60 minutes, 96% of subjects were responders, but three subjects failed to respond at any time point. Conclusion: Measurement of levels of Factor VIII, VWF:Ag, and VWF:RCo, optimally at 60 minutes after the DDAVP infusion, is sufficient to confirm a patient as a responder in a DDAVP challenge test. Curtailing the number of time points of measurement will result in significant savings in cost and time to patients and their providers.

Original languageEnglish (US)
Pages (from-to)325-330
Number of pages6
JournalInternational Journal of Laboratory Hematology
Volume41
Issue number3
DOIs
StatePublished - Jun 1 2019

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Type 1 von Willebrand Disease
Deamino Arginine Vasopressin
von Willebrand Factor
Chemical analysis
Fibrinogen
Blood
Cost Savings
Antigens

Keywords

  • VWF antigen
  • coagulation
  • desmopressin
  • ristocetin
  • von Willebrand disease

ASJC Scopus subject areas

  • Hematology
  • Clinical Biochemistry
  • Biochemistry, medical

Cite this

Quantitative analysis of desmopressin (DDAVP) response in adult patients with type 1 von Willebrand disease. / Guddati, Achuta Kumar; Rosovsky, Rachel P.; Van Cott, Elizabeth M.; Kuter, David J.

In: International Journal of Laboratory Hematology, Vol. 41, No. 3, 01.06.2019, p. 325-330.

Research output: Contribution to journalArticle

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abstract = "Introduction: Patients with Type 1 von Willebrand disease (VWD) have reduced amounts of von Willebrand factor (VWF) in their blood. Desmopressin (DDAVP) has been used to raise the blood levels of VWF in these patients. However, not all patients with Type 1 VWD are known to respond to DDAVP therapy. We sought to compare the levels of Factor VIII, VWF antigen (VWF:Ag), VWF ristocetin cofactor (VWF:RCo), and fibrinogen at different time points after administration of DDAVP. Methods: A retrospective analysis was conducted at a single institution on 89 patients with Type 1 VWD who received a single intravenous dose of 0.3 μg/kg DDAVP. Levels of Factor VIII, VWF:Ag, VWF:RCo, and fibrinogen were measured before and then 30, 60, 90, and 120 minutes after completion of DDAVP administration. Results: Median levels of Factor VIII, VWF:Ag, and VWF:RCo were significantly elevated (P < 0.001) at 30 (175 IU/dL, 111 IU/dL, 127 IU/dL), 60 (218 IU/dL, 138 IU/dL, 139 IU/dL), and 90 (212 IU/dL, 134 IU/dL, 130 IU/dL) minutes time points when compared to their respective baseline values (71 IU/dL, 54 IU/dL, 54 IU/dL). At 60 minutes, 96{\%} of subjects were responders, but three subjects failed to respond at any time point. Conclusion: Measurement of levels of Factor VIII, VWF:Ag, and VWF:RCo, optimally at 60 minutes after the DDAVP infusion, is sufficient to confirm a patient as a responder in a DDAVP challenge test. Curtailing the number of time points of measurement will result in significant savings in cost and time to patients and their providers.",
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AB - Introduction: Patients with Type 1 von Willebrand disease (VWD) have reduced amounts of von Willebrand factor (VWF) in their blood. Desmopressin (DDAVP) has been used to raise the blood levels of VWF in these patients. However, not all patients with Type 1 VWD are known to respond to DDAVP therapy. We sought to compare the levels of Factor VIII, VWF antigen (VWF:Ag), VWF ristocetin cofactor (VWF:RCo), and fibrinogen at different time points after administration of DDAVP. Methods: A retrospective analysis was conducted at a single institution on 89 patients with Type 1 VWD who received a single intravenous dose of 0.3 μg/kg DDAVP. Levels of Factor VIII, VWF:Ag, VWF:RCo, and fibrinogen were measured before and then 30, 60, 90, and 120 minutes after completion of DDAVP administration. Results: Median levels of Factor VIII, VWF:Ag, and VWF:RCo were significantly elevated (P < 0.001) at 30 (175 IU/dL, 111 IU/dL, 127 IU/dL), 60 (218 IU/dL, 138 IU/dL, 139 IU/dL), and 90 (212 IU/dL, 134 IU/dL, 130 IU/dL) minutes time points when compared to their respective baseline values (71 IU/dL, 54 IU/dL, 54 IU/dL). At 60 minutes, 96% of subjects were responders, but three subjects failed to respond at any time point. Conclusion: Measurement of levels of Factor VIII, VWF:Ag, and VWF:RCo, optimally at 60 minutes after the DDAVP infusion, is sufficient to confirm a patient as a responder in a DDAVP challenge test. Curtailing the number of time points of measurement will result in significant savings in cost and time to patients and their providers.

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