Test Details
Methodology
See individual tests.
Result Turnaround Time
5 - 7 days
Turnaround time is defined as the usual number of days from the date of pickup of a specimen for testing to when the result is released to the ordering provider. In some cases, additional time should be allowed for additional confirmatory or additional reflex tests. Testing schedules may vary.
Test Includes
This test includes factor VIII (x3), von Willebrand factor activity (x3) and von Willebrand factor antigen (x3).
Use
This profile is designed to assess the response of DDAVP treatment in patients presenting with any of a number of different bleeding disorders (see Additional Information) by measuring VWF and factor VIII levels pre-treatment (baseline) and at two time-points (1 hour and 4 hours) post-treatment. Post-treatment VWF levels are evaluated against baseline results to determine whether the response is adequate and sustained. Results should be correlated with clinical symptoms and family history.
Limitations
Circumstances where the use of DDAVP is contraindicated have been reviewed in a recent evidence-based guideline from the American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF) and the World Federation of Hemophilia (WFH).6
Desmopressin has several side effects, including headache, tachycardia, flushing, hypotension, hyponatraemia, and rarer seizures and coronary events.1,40,41 Therefore, the dosing and post-dose status of patients undergoing DDAVP stimulation needs to be carefully monitored by trained clinical personnel.42
Custom Additional Information
Desmopressin (DDAVP) is a synthetic derivative of antidiuretic hormone that can be used to increase functional von Willebrand factor (VWF) and factor VIII (FVIII) levels in a number of bleeding risk conditions including some von Willebrand disease (VWD) subtypes.1-21 A comprehensive review of the clinical and laboratory evidence for the use of DDAVP in von Willebrand disease (VWD), hemophilia A, platelet function disorders, uremia, liver cirrhosis and pregnancy has recently been published.1
The administration of DDAVP intravenously, subcutaneously or intranasally stimulates release of VWF and factor VIII (FVIII) from endogenously stored reserves. This can lead to a doubling (or more) in the level of functional VWF in most cases of type 1 VWD.1,6 DDAVP can be the appropriate treatment choice in some cases of type 2 VWD and or acquired von Willebrand syndrome.1,22 However, its utility is limited in most cases of type 2 VWD because release of “dysfunctional” VWF generally does not restore full hemostatic function. DDAVP use is contraindicated in type 2B VWD because the release of gain-of-function (hyperadhesive) VWF may lead to thrombocytopenia.3,9,12,23,24
In type 2N VWD, the plasma “defect” is a diminished FVIII level. DDAVP can make up for the lack of FVIII, but it only stays active in the system for a short time.25 Similarly, treatment of these patients with replacement FVIII will only increase plasma FVIII levels temporarily without properly functioning VWF to bind to and protect it.3,6 DDAVP is not useful in type 3 VWD because this condition represents a complete absence of VWF, both in plasma and in endothelial cells.
DDAVP is mostly prescribed in patients in which VWF and FVIII are not completely deficient, such as patients with VWD type 1, patients with moderate and mild hemophilia A,26 and patients with platelet function disorders.27 The principal treatment for VWD for cases where DDAVP is not efficacious or is contraindicated is replacement of missing/defective VWF (and in some cases replacement of missing FVIII), using VWF or FVIII concentrates.1,6 Type 2N VWD needs to be distinguished from hemophilia A (both of which have low FVIII levels), so that the appropriate therapy can be prescribed (VWF replacement in 2N VWD; FVIII replacement in hemophilia A).8,10,28
The ability to increase plasma FVIII and VWF levels in response to DDAVP depends largely on the availability of stored FVIII/VWF, the efficacy of FVIII/VWF secretion and rate of clearance,14,29,30 and there is a large inter-individual variation in responsiveness to DDAVP.1,2,6,31,32 A trial of DDAVP can be completed to assess individual responsiveness before employing DDAVP clinically.1,2,28,31 It is recommended that measurement of vWF antigen (VWF:Ag), activity (VWF:Ac) and factor FVIII activity (FVIII) should be performed at baseline (immediately before dosing with DDAVP) (T0), at one hour after dose (T1) and at four hours after dose (T4).12,33,34
The American Society of Hematology, the International Society on Thrombosis and Haemostasis, the National Hemophilia Foundation and the World Federation of Hemophilia 2021 guidelines31 define a complete response is as a VWF or FVIII level increasing at least twofold over baseline and reaching levels >50 U/dL for VWD and hemophilia A, respectively after one hour.2 Partial response can be defined as a VWF:Ag and/or FVIII:C between 30 U/dL and 50 U/dL, and nonresponse is VWF:Ag and/or FVIII levels <30 U/dL.2 These definitions are not applicable to patients with baseline levels >50 U/dL.2 However, the efficacy of DDAVP response can be judged to some extent in these individuals by noting the change in VWF:Ag and/or FVIII:C levels from baseline and the extent to which levels drop between T1 and T4.2
There are several VWF mutations (including R1205H Vicenza) that are associated with rapid VWF clearance.35 Type 1C of VWD is characterized by a reduced VWF half-life and is associated with a lack of sustained efficacy of DDAVP treatment.36-39 A decrease of greater than 30% in VWF and/or FVIII at T4 relative to the peak concentrations (at T1) indicates increased VWF clearance, compatible with type 1C VWD.3
Specimen Requirements
Specimen
Citrated plasma, frozen
Volume
2 mL
Minimum Volume
1.5 mL per timepoint (Note: This volume does not allow for repeat testing.)
Container
Blue-top (sodium citrate) tube
Collection Instructions
This challenge protocol is accomplished as follows:
Citrate whole blood is collected (see Sample Collection). This sample should be labeled (Baseline-T0) with the time of collection.
Immediately after collecting T0 sample, patient is dosed with DDAVP (Desmopressin) and described in drug labeling. This is not to be done by Labcorp staff.
Desmopressin has several side effects, including headache, tachycardia, flushing, hypotension, hyponatraemia, and rarer seizures and coronary events.1,40 ,41 Therefore, the dosing and post-dose status of patients undergoing DDAVP stimulation needs to be carefully monitored by trained clinical personnel.42
One hour after drug dosing, a second citrate whole blood is collected. This sample should be labeled (One Hour-T1) with the time of collection.
Three hours later (four hours after drug dosing), a third citrate whole blood is collected. This sample should be labeled (One Hour-T4) with the time of collection.
Sample Collection
Blood should be collected in a blue-top tube containing 3.2% buffered sodium citrate.43 Evacuated collection tubes must be filled to completion to ensure a proper blood to anticoagulant ratio.44,45 The sample should be mixed immediately by gentle inversion at least six times to ensure adequate mixing of the anticoagulant with the blood. A discard tube is not required prior to collection of coagulation samples.46,47 When noncitrate tubes are collected for other tests, collect sterile and nonadditive (red-top) tubes prior to citrate (blue-top) tubes. Any tube containing an alternate anticoagulant should be collected after the blue-top tube. Gel-barrier tubes and serum tubes with clot initiators should also be collected after the citrate tubes. Centrifuge and carefully remove the plasma using a plastic transfer pipette, being careful not to disturb the cells. Transfer the plasma into a Labcorp PP transpak frozen purple tube with screw cap (Labcorp No. 49482). Freeze immediately and maintain frozen until tested.
To avoid delays in turnaround time when requesting multiple tests on frozen samples, please submit separate frozen specimens for each test requested.
Please print and use the Volume Guide for Coagulation Testing to ensure proper draw volume.
Storage Instructions
Freeze.
Patient Preparation
Do not draw from an arm with a heparin lock or heparinized catheter.
Causes for Rejection
Gross hemolysis; clotted specimen; frozen specimen thawed in transit; improper labeling; improper collection tube
References
Gringeri A, Mannucci PM, Gringeri M, Castaman G, Peyvandi F. Global estimation of the bleeding episodes treatable with desmopressin in von Willebrand disease and hemophilia A. Haematologica. 2025 Aug 1;110(8):1710-1722. PubMed 40176766
Mannucci PM. Use of desmopressin in the treatment of hemophilia A: towards a golden jubilee. Haematologica. 2018 Mar;103(3):379-381. PubMed 29491128
Mannucci PM, Siboni SM. Factor VIII and von Willebrand factor are wavering proteins: the basis for the therapeutic development of desmopressin. Res Pract Thromb Haemost. 2024 Mar 15;8(2):102369. PubMed 38595335
Footnotes
1. Reardon B, Pasalic L, Favaloro EJ. A Review of Desmopressin Use in Bleeding Disorders: An Unsung Hero? Biomolecules. 2025 Jul 5;15(7):967. PubMed 40723839
2. Laan S, Del Castillo Alferez J, Cannegieter S et al. DDAVP response and its determinants in bleeding disorders: a systematic review and meta-analysis. Blood. 2025 Apr 17;145(16):1814-1825. PubMed 39854691
3. Kaur V, Elghawy O, Deshpande S, Riley D. von Willebrand disease: A guide for the internist. Cleve Clin J Med. 2024 Feb 2;91(2):119-127. PubMed 38307601
4. Fogarty H, Doherty D, O'Donnell JS. New developments in von Willebrand disease. Br J Haematol. 2020 Nov;191(3):329-339. PubMed 32394456
5. Beltran A, Jaramillo AP, Vallejo MP et al. Desmopressin as a Treatment in Patients With Von Willebrand Disease: A Systematic Review. Cureus. 2023 Aug 29;15(8):e44310. PubMed 37649925
6. Connell NT, Flood VH, Brignardello-Petersen R, et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv. 2021 Jan 12;5(1):301-325. PubMed 33570647
7. Favaloro EJ, Pasalic L, Curnow J. Monitoring Therapy during Treatment of von Willebrand Disease. Semin Thromb Hemost. 2017 Apr;43(3):338-354. PubMed 27472426
8. Loomans JI, Kruip MJHA, Carcao M, et al. Desmopressin in moderate hemophilia A patients: a treatment worth considering. Haematologica. 2018 Mar;103(3):550-557. PubMed 29305412
9. Lavin M, O’Donnell JS. New treatment approaches to von Willebrand disease. Hematology Am Soc Hematol Educ Program. 2016 Dec 2;2016(1):683-689. PubMed 27913547
10. Curnow J, Pasalic L, Favaloro EJ. Treatment of von Willebrand Disease. Semin Thromb Hemost. 2016 Mar;42(2):133-146. PubMed 26838696
11. Leebeek FWG, Eikenboom JCJ. Von Willebrand’s disease. N Engl J Med. 2016 Nov 24;375(21):2067-2080. PubMed 27959741
12. Laffan MA, Lester W, O'Donnell JS, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors' Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol. 2014 Nov;167(4):453-465. PubMed 25113304
13. van Galen KPM, Mauser-Bunschoten EP, Leebeek FWG. Hemophilic arthropathy in patients with von Willebrand disease. Blood Rev. 2012 Nov;26(6):261-266. PubMed 23010260
14. Castaman G, Lethagen S, Federici AB, et al. Response to desmopressin is influenced by the genotype and phenotype in type 1 von Willebrand disease (VWD): results from the European study MCMDM-1VWD. Blood. 2008 Apr 1;111(7):3531-3539. PubMed 18230755
15. Ozgönenel B, Rajpurkar M, Lusher JM. How do you treat bleeding disorders with desmopressin? Postgrad Med J. 200 Mar7; 83(977):159-163. PubMed 17344569
16. Pasi KJ, Collins PW, Keeling DM et al. Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization. Haemophilia. 2004 May;10(3):218-231. PubMed 15086319
17. Mannucci PM. Desmopressin (DDAVP) in the treatment of bleeding disorders: the first twenty years. Haemophilia. 2000 Jul;6 Suppl 1:60-67. PubMed 10982270
18. Mannucci PM. Desmopressin (DDAVP) in the treatment of bleeding disorders: the first 20 years. Blood. 1997 Oct 1;90(7):2515-2521. PubMed 9326215
19. Nance D, Fletcher SN, Bolgiano DC, Thompson AR, Josephson NC, Konkle BA. Factor VIII mutation and desmopressin responsiveness in 62 patients with mild haemophilia A. Haemophilia. 2013 Sep;19(5):720-726. 23711294
20. Revel-Vilk S, Blanchette VS, Sparling C, Stain AM, Carcao MD. DDAVP challenge tests in boys with mild/moderate haemophilia A. Br J Haematol. 2002 Jun;117(4):947-951. PubMed 12060135
21. Revel-Vilk S, Schmugge M, Carcao MD, Blanchette P, Rand ML, Blanchette VS. Desmopressin (DDAVP) responsiveness in children with von Willebrand disease. J Pediatr Hematol Oncol. 2003 Nov;25(11):874-879. PubMed 14608197
22. Biguzzi E, Siboni SM, Peyvandi F. Acquired Von Willebrand syndrome and response to desmopressin. Haemophilia. 2018 Jan;24(1):e25-e28. PubMed 29239101
23. Federici AB. The use of desmopressin in von Willebrand disease: the experience of the first 30 years (1977-2007). Haemophilia. 2008 Jan;14 Suppl 1:5-14. PubMed 18173689
24. Casonato A, Steffan A, Pontara E, et al. Post-DDAVP Thrombocytopenia in Type 2B von Willebrand Disease Is not Associated with Platelet Consumption: Failure to Demonstrate Glycocalicin Increase or Platelet Activation. Thromb Haemost. 1999 Feb;81:224-228. PubMed 10063996
25. Franchini M, Seidizadeh O, Mannucci PM. Prophylactic management of patients with von Willebrand disease. Ther Adv Hematol. 2021 Dec 22;12:20406207211064064. PubMed 34987743
26. Borel-Derlon A, Federici AB, Roussel-Robert V, et al. Treatment of severe von Willebrand disease with a high-purity von Willebrand factor concentrate (Wilfactin®): a prospective study of 50 patients. J Thromb Haemost. 2007 Jun;5(6):1115-1124. PubMed 17403090
27. Leissinger C, Carcao M, Gill JC, Journeycake J, Singleton T, Valentino L. Desmopressin (DDAVP) in the management of patients with congenital bleeding disorders. Haemophilia. 2014 Mar;20(2):158-167. PubMed 23937614
28. Colonne CK, Reardon B, Curnow J, Favaloro EJ. Why is Misdiagnosis of von Willebrand Disease Still Prevalent and How Can We Overcome It? A Focus on Clinical Considerations and Recommendations. J Blood Med. 2021 Aug 17;12:755-768. PubMed 34429677
29. Di Perna C, Riccardi F, Franchini M, Rivolta GF, Pattacini C, Tagliaferri A. Clinical efficacy and determinants of response to treatment with desmopressin in mild hemophilia A. Semin Thromb Hemost. 2013 Oct;39(7):732-739. PubMed 24030345
30. Seary ME, Feldman D, Carcao MD. DDAVP responsiveness in children with mild or moderate haemophilia A correlates with age, endogenous FVIII:C level and with haemophilic genotype. Haemophilia. 2012 Jan;18(1):50-55. PubMed 21592259
31. James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv. 2021 Jan 12;5(1):280-300. PubMed 33570651
32. Castaman G, Tosetto A, Eikenboom JC, Rodeghiero F. Blood group significantly influences von Willebrand factor increase and half-life after desmopressin in von Willebrand disease Vicenza. J Thromb Haemost. 2010 Sep;8(9):2078-2080. PubMed 20561182
33. Platton S, Baker P, Bowyer A et al. Guideline for laboratory diagnosis and monitoring of von Willebrand disease: A joint guideline from the United Kingdom Haemophilia Centre Doctors' Organisation and the British Society for Haematology. Br J Haematol. 2024 May;204(5):1714-1731. PubMed 38532595
34. Kalot MA, Husainat N, Abughanimeh O, et al. Laboratory assays of VWF activity and use of desmopressin trials in the diagnosis of VWD: a systematic review and meta-analysis. Blood Adv. 2022 Jun 28;6(12):3735-3745. PubMed 35192687
35. Casari C, Lenting PJ, Wohner N, Christophe OD, Denis CV. Clearance of von Willebrand factor. J Thromb Haemost. 2013 Jun;11 Suppl 1:202-211. PubMed 23809124
36. Sharma R, Haberichter SL. New advances in the diagnosis of von Willebrand disease. Hematology Am Soc Hematol Educ Program. 2019 Dec 6;2019(1):596-600. PubMed 31808831
37. Ng C, Motto DG, Di Paola J. Diagnostic approach to von Willebrand disease. Blood. 2015 Mar 26;125(13):2029-2037. PubMed 25712990
38. Castaman G, Goodeve A, Eikenboom J, European Group on von Willebrand Disease. Principles of care for the diagnosis and treatment of von Willebrand disease. Haematologica. 2013 May; 98:667-674. PubMed 23633542
39. Millar CM, Riddell AF, Brown SA, et al. Survival of von Willebrand factor released following DDAVP in a type 1 von Willebrand disease cohort: influence of glycosylation, proteolysis and gene mutations. Thromb Haemost. 2008 May;99(5):916-924. PubMed 18449422
40. Desborough MJ, Oakland KA, Landoni G, et al. Desmopressin for treatment of platelet dysfunction and reversal of antiplatelet agents: a systematic review and meta-analysis of randomized controlled trials. J Thromb Haemost. 2017 Feb;15(2):263-272. PubMed 27893176
41. Mannucci PM, Cattaneo M. Desmopressin: a nontransfusional treatment of hemophilia and von Willebrand disease. Haemostasis. 1992;22(5):276-280. PubMed 1478539
42. Romano LGR, Schütte LM, van Hest RM, et al. Tachyphylaxis and reproducibility of desmopressin response in perioperative persons with nonsevere hemophilia A: implications for clinical practice. Res Pract Thromb Haemost. 2024 Mar 5;8(3):102367. PubMed 38660455
43. Adcock DM, Kressin DC, Marlar RA. Effect of 3.2% vs 3.8% sodium citrate concentration on routine coagulation testing. Am J Clin Pathol. 1997 Jan;107(1):105-110. PubMed 8980376
44. Reneke J, Etzell J, Leslie S, Ng VL, Gottfried EL. Prolonged prothrombin time and activated partial thromboplastin time due to underfilled specimen tubes with 109 mmol/L (3.2%) citrate anticoagulant. Am J Clin Pathol. 1998 Jun;109(6):754-757. PubMed 9620035
45. National Committee for Clinical Laboratory Standardization. Collection, Transport, and Processing of Blood Specimens for Coagulation Testing and General Performance of Coagulation Assays; Approved Guideline. 5th ed. Villanova, Pa: NCCLS; 2008. Document H21-A5:28(5).
46. Gottfried EL, Adachi MM. Prothrombin time and activated partial thromboplastin time can be performed on the first tube. Am J Clin Pathol. 1997 Jun;107(6):681-683. PubMed 9169665
47. McGlasson DL, More L, Best HA, Norris WL, Doe RH, Ray H. Drawing specimens for coagulation testing: Is a second tube necessary? Clin Lab Sci. 1999 May-Jun;12(3):137-139. PubMed 10539100