Subscribe to RSS
DOI: 10.1055/s-0038-1642758
Patients With Prolonged Bleeding Time of Undefined Etiology, and Their Response to Desmopressin
Publication History
Received 01 July 1987
Accepted after revision 27 November 1987
Publication Date:
31 May 2018 (online)
Summary
Between 1981 and 1986, we evaluated 59 patients who presented with isolated prolongation of bleeding time with normal platelet counts, platelet aggregation and coagulation parameters (including von Willebrand’s factor), and without evidence of liver or kidney disease, or exposure to anti-platelet agents. These patients, termed as vascular fragility syndrome (VFS), were analyzed to further characterize their bleeding patterns. The pattern of bleeding manifestations was similar to that of patients with platelet dysfunction, such as mucocutaneous bleeding or excessive post-operative bleeding. In 16 patients, desmopressin (l-desamino-8-d-arginine vasopressin, DDAVP) was infused to control active bleeding or as a part of pre-surgical evaluation. Bleeding time improved (pre-DDAVP bleeding time 15.3 ± 3.4 min, mean±S.D.; post-DDAVP bleeding time 10.7 ± 3.9 min; p<0.01) within 30 minutes following the DDAVP infusion with either satisfactory arrest of acute bleeding or good control of subsequent hemostasis with surgery. Side effects with DDAVP were transient and minor, i.e. facial flushing, or conjunctival erythema. These findings indicate that VFS with isolated prolongation of bleeding time is a frequently encountered bleeding disorder and that DDAVP is effective in achieving hemostasis for the management of acute bleeding and may be useful prior to surgical procedures.
-
References
- 1 Stein MN, Hoak JC. Nonthrombocytopenic vascular disorders. In: Colman RW, Hirsch J, Marder VJ, Salzman EW. (Eds.) Hemostasis and Thrombosis. p 557 J B Lippincott; Philadelphia: 1982
- 2 Mannucci PM. Desmopressin (DDVAP) for the treatment of disorders of hemostasis. In: Progress in Hemostasis and Thrombosis. Coller BS. (Ed.) Vol.8 pp 19-45 Grune and Stratton; New York: 1986
- 3 Richardson DW, Robinson AG. Desmopressin. Ann Intern Med 1985; 103: 228-239
- 4 Mielke CH, Kaneshiro MM, Mahler IA. et al. The standardized normal Ivy bleeding time and its prolongation by aspirin. Blood 1969; 34: 204-215
- 5 Toomey KC, Kim HC, Kosmin M, Saidi P. Clinical trial of a new disposable bleeding-time device. Am J Clin Pathol 1986; 85: 610-613
- 6 Langdell RD, Wagner RH, Brinkhous KM. Effect of antihemophilic factor on one stage clotting tests. J Lab Clin Med 1953; 41: 637
- 7 Laurell CB. Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodies. Anal Biochem 1966; 15: 45-52
- 8 MacFarlane DE, Stibbe J, Kirby FP, Zucker MB, Grant RA. A method assessing von Willebrand’s factor (ristocetin cofactor). Thrombos Diathes Haemorrh 1975; 34: 306-308
- 9 Ruggeri ZM, Zimmerman TS. Variant von Willebrand’s disease: Characterization of two subtypes to analysis of multimeric composition of factor VHI/von Willebrand factor in plasma and platelets. J Clin Invest 1980; 65: 1318-1325
- 10 Brosstad F, Kjdnniksen I, Rpnning B. et al Visualization of von Willebrand factor multimer by enzyme-conjugated secondary antibody. Thromb Haemostas 1986; 55: 276-278
- 11 Kobrinsky NL, Israel ED, Gerrard JM. et al. Shortening of bleeding time by l-deamino-8-d-arginine vasopressin in various bleeding disorders. Lancet 1984; i: 1145-1148
- 12 Mannucci PM, Ruggeri ZM, Pareti FI. et al. l-desamino-8-arginine vasopressin: a new pharmacologic approach to the management of haemophilia A and von Willebrand’s disease. Lancet 1977; i: 86-172
- 13 Ludlam CA, Peake IR, Allen N. et al. Factor VIII and fibrinolytic response to deamino-8-arginine vasopressin in normal subjects and dissociate response in some patients with haemophilia and von Willebrand’s disease. Br J Haematol 1980; 45: 499-511
- 14 Nilsson IM, Holmberg L, Abert M. et al. The release of plasminogen activator and factor VIII after injection of DDAVP in healthy subjects and patients with von Willebrand’s disease. Scand J Haematol 1980; 24: 351-359
- 15 de la Fuente B, Kasper CK, Rickies FR. et al. Response of patients with mild and moderate hemophilia A and von Willebrand’s disease to treatment with desmopressin. Ann Intern Med 1985; 103: 6-14
- 16 Cash JD, Gader AM A, da Costa J. The release of plasminogen activator and factor VIII by LVP, AVP, DDAVP, ATIII and OT in man. Br J Haematol 1974; 27: 363-364
- 17 Mannucci PM, Remuzzi G, Pusineri F. et al. Deamino-8-d-arginine vasopressin shortens the bleeding time in uremia. N Engl J Med 1983; 308: 8-12
- 18 Kentro TB, Lottenberg R, Kitchens CS. Clinical efficacy of desmopressin acetate for hemostatic control in patients with primary platelet disorders undergoing surgery. Amer Haematol 1987; 24: 215-219
- 19 Barnhart MI, Chen S, Lusher JM. DDAVP: Does the drug have a direct effect on the vessel wall?. Thromb Res 1983; 31: 239-253
- 20 Schulman S, Johnsson H, Edberg N, Blomback N. DDAVP-induced correction of prolonged bleeding time in patients with congenital platelet function defects. Thromb Res 1987; 45: 165-174
- 21 Burroughs AK, Mathews K, Qadiri M. et al. Desmopressin and bleeding time in patients with cirrhosis. Brit Med J 1985; 291: 1377-1381
- 22 Nieuwenhuis HK, Akkerman J-WN, Sixma JJ. Patients with a prolonged bleeding time and normal Aggregation tests may have storage Pool deficiency: Studies on one hundred six patients. Blood 1987; 70: 620-623