Desmopressin‐induced thrombocytopenia in type i platelet discordant von willebrand disease
- 1 May 1993
- journal article
- case report
- Published by Wiley in American Journal of Hematology
- Vol. 43 (1) , 5-9
- https://doi.org/10.1002/ajh.2830430103
Abstract
Thrombocytopenia after desmopressin (DDAVP) infusion is usually observed in patients with type IIB von Willebrand disease (vWD). No other subtypes of vWD with thrombocytopenia after DDAVP have been reported so far. We describe here the occurrence of thrombocytopenia after DDAVP in a 39 year old male and his son with phenotypic characteristics of type I vWD, 'platelet discordant subtype.' After DDAVP, the abnormal ristocetin cofactor/von Willebrand factor antigen ratio in plasma was not corrected and the bleeding time remained markedly prolonged. Platelet count dropped 30 min after DDAVP (from 279 to 96 × 103/μL in the propositus and from 298 to 116 × 103/μL in his affected son) and returned to normal at 60 min. Platelet clumping was evident on peripheral blood smears obtained after infusion. These cases indicate that after DDAVP thrombocytopenia can occur in vWD other than type IIB.Keywords
This publication has 11 references indexed in Scilit:
- Replacement Therapy with Virus—Inactivated Plasma Concentrates in von Willebrand DiseaseVox Sanguinis, 1992
- Clinical indications for desmopressin (DDAVP) in congenital and acquired von Willebrand diseaseBlood Reviews, 1991
- Platelet von Willebrand factor assay: results using two methods for platelet lysisThrombosis Research, 1990
- Von Willebrand Factor Antigen Is Less Sensitive than Ristocetin Cofactor for the Diagnosis of Type I von Willebrand Disease -Results Based on an Epidemiological InvestigationThrombosis and Haemostasis, 1990
- Hyper-responsiveness to DDAVP for patients with type I von Willebrand's disease and normal intra-platelet von Willebrand factorEuropean Journal of Haematology, 1988
- von Willebrand disease "Vicenza" with larger-than-normal (supranormal) von Willebrand factor multimersBlood, 1988
- Subunit composition of plasma von Willebrand factor. Cleavage is present in normal individuals, increased in IIA and IIB von Willebrand disease, but minimal in variants with aberrant structure of individual oligomers (types IIC, IID, and IIE).Journal of Clinical Investigation, 1986
- Heterogeneity of type I von Willebrand disease: evidence for a subgroup with an abnormal von Willebrand factorBlood, 1985
- Platelet Aggregation Induced by I-Desamino-8-D-Arginine Vasopressin (dDAVP) in Type IIb von Willebrand's DiseaseNew England Journal of Medicine, 1983
- Heightened Interaction between Platelets and Factor VIII/von Willebrand Factor in a New Subtype of von Willebrand's DiseaseNew England Journal of Medicine, 1980