1-Desamino-8-D-Arginine Vasopressin (DDAVP) Infusion in Type IIB von Willebrand’s Disease: Shortening of Bleeding Time and Induction of a Variable Pseudothrombocytopenia

1990 ◽  
Vol 64 (01) ◽  
pp. 117-120 ◽  
Author(s):  
Alessandra Casonato ◽  
M Teresa Sartori ◽  
Luigi de Marco ◽  
Antonio Girolami
Keyword(s):  
Monoclonal Antibody ◽  
Platelet Count ◽  
Arginine Vasopressin ◽  
Calcium Concentration ◽  
Sodium Citrate ◽  
Bleeding Time ◽  
Blood Collection ◽  
Von Willebrand's Disease ◽  
Blood Samples ◽  
Von Willebrand’S Disease

SummaryWe have investigated the effects of 1-desamino-8-D-arginine vasopressin (DDAVP) infusion on platelet count and bleeding time in 4 patients with type IIB von Willebrand’s disease (vWd). Three of four patients showed a normalization of the bleeding time within 1 h after the infusion, while bleeding time was not modified in the fourth. In accordance with the literature, thrombocytopenia was observed after DDAVP infusion, but this thrombocytopenia was due to the anticoagulants used for blood collection. In two patients (F. I., G. F.) no thrombocytopenia was observed when platelets were counted by fingerstick method but there was a 20% platelet decrease in blood samples collected in sodium citrate and a 50% decrease in samples collected in EDTA. Dramatic falls in platelet counts (70–95%) were observed in the additional two patients (C. A., D.Z.) after DDAVP infusion, when both sodium citrate or EDTA were used as anticoagulants. In the latter two patients there was also a 50% decrease in platelet count when the fingerstick method was used. The decrease in the patient’s platelet count in EDTA samples after DDAVP infusion could be prevented, in part, by the previous additions of an anti GPIb monoclonal antibody and an anti GPIIb-IIIa monoclonal antibody.Thus, the thrombocytopenia observed in the four IIB vWd patients studied after DDAVP infusion seems to be, at least partially, a pseudothrombocytopenia depending on the calcium concentration in the blood samples and the availability of GPIb and GPIIb-IIIa receptors. These findings and the normalization of the bleeding time observed in three of the four patients has led us to reconsider the possible use of DDAVP in the treatment of our IIB vWd patients.

Effect Of Prednisone On Platelet Function In Patients With Bleeding Disorders

1981 ◽  
Author(s):  
R McKenna ◽  
F Bachmann ◽  
O Pichairut ◽  
B Whittaker
Keyword(s):  
Platelet Count ◽  
Platelet Function ◽  
Bleeding Time ◽  
Platelet Factor ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Before And After ◽  
History Of ◽  
The Mean ◽  
One Year

There is considerable controversy regarding the effect of Prednisone on the hemostatic mechanism of normal people versus patients with bleeding diatheses. We administered Prednisone 15 mg TID to patients with a positive history of a bleeding disorder, and evaluated the bleeding time and other in-vitrc tests of platelet function prior to and between the 5th and 7th day after Prednisone.Eleven patients were admitted into this study over a one year period. All patients had a history of excessive bruising, epistaxis, bleeding after dental extractions, and gastrointestinal or other bleeding in various combinations. Two out of the eleven had template bleeding times of greater than 15 minutes both before and after the Prednisone. These two patients were subsequently proven to have von Willebrand’s disease by the washed platelet ristocetin assay. In the remaining 9 patients, the pre-Prednisone bleeding time was 9.3 ±3.7 minutes (x ± 1 S.D.) whereas the post-Prednisone bleeding time was 5.8 ±3.6 minutes (x ±1 S.D.). These results were significant(td=3.83;df:7;p=0.007).Platelet aggregation in response to exogenous ADP (1 μM, 3 μM) Sigma bovine tendon collagen (1.8 mg/ml F) and epinephrine (5.5 × 104M), platelet retention in a glass bead column or platelet factor 3 availability did not improve or worsen after Prednisone therapy. The mean platelet count of 328,000±94,000 (x ±1 S.D.) was significantly (p=0.05) higher than the mean pre-Prednisone platelet count of 268,000±77,000 (x ±1 S.D.).In conclusion, we have shown that large doses of Prednisone appear to shorten the bleeding time in patients with significant defects in the primary hemostatic mechanism. However the bleeding time improvement is not evident in patients with von Willebrand’s disease.

Study of a Caucasian Family with von Willebrand’s Disease in Association with Vascular Telangiectasia and Hemoglobinopathy

1979 ◽  
Author(s):  
W. Hanna ◽  
C. McCarroll ◽  
J. Chen ◽  
T. McDonald ◽  
D. Lin ◽  
...  
Keyword(s):  
Platelet Count ◽  
Platelet Aggregation ◽  
Factor Viii ◽  
Bleeding Time ◽  
Family Members ◽  
Inherited Disorders ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Normal Platelet ◽  
Coagulant Activity

This family carries multihematological inherited disorders; namely, von Wille-brand’s, vascular telangiectasia and hemoglobinopathy. Family members were studied by quantifying the following: Factor VIII pro-coagulant activity, Factor VIII related antigen, Factor VIII inhibitors, platelet adhesion, platelet aggregation (with ristocetin, collagen and ADP), bleeding time, platelet count, partial thromboplastin time, prothrombin time, hemoglobin electrophoresis, hemoglobin finger-printing, sickling preparation and the presence of telangiectasia.The affected members of this family with von Willebrand’s express their disease in a variable tendency to bleeding from almost clinically asymptomatic cases to cases with severe bleeding tendency.One member of this family had to have a hysterectomy at the age of 20 to control the abnormal uterine bleeding after conservative treatment failed. All affected members with von Willebrand’s disease had a normal platelet count, prolonged bleeding time, decreased Factor VIII pro-coagulant activity and related antigen, negative aggregation using the ristocetin co-factor for von Willebrand’s, defective platelet adhesiveness to glass beads, and normal platelet aggregation to collagen and ADP. Some members have vascular telangiectasia in the mucous membranes. An incidental finding was the presence of an abnormal hemoglobin S in some family members.Supported in part by the Cumberland Chapter of the National Hemophilia Foundation.

The Spectrum of von Willebrand’s Disease

1967 ◽  
Vol 18 (01/02) ◽  
pp. 040-056 ◽  
Author(s):  
E. J Walter Bowie ◽  
P Didisheim ◽  
J. H Thompson ◽  
C. A Owen
Keyword(s):  
Factor Viii ◽  
Bleeding Time ◽  
Severe Bleeding ◽  
Platelet Adhesiveness ◽  
Platelet Activity ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
The Third ◽  
Generation Test

SummaryPatients (from 5 kindreds) with variants of von Willebrand’s disease are described. In one kindred the depression of factor VIII was moderate (20 to 40% of normal) and transfusion of 500 ml of normal plasma led to an increase higher than anticipated and to an almost normal level of factor VIII 17 to 24 hrs later. This represents the usual type of von Willebrand’s disease.In the second kindred the concentration of factor VIII was less than 2 % of normal in the son and daughter, who had severe bleeding and hemarthroses.The third kindred was characterized by reduction of factor VIII and a long bleeding time as well as by a serum defect in the thromboplastin-generation test comparable to that seen in patients with hemophilia B, yet with normal levels of factors IX, X, and VII. The severity of the serum defect, the positive result with the Rumpel-Leede test, and the reduced platelet activity in the thromboplastin-generation test are all compatible with the diagnosis of thrombopathy or ‘‘thrombopathic hemophilia.” In two other kindreds, one patient had a long bleeding time and normal levels of factor VIII and another had a normal bleeding time and decrease of factor VIII. The last patient had the type of response to transfusion usually seen in von Willebrand’s disease.In four kindreds, platelet adhesiveness in vivo was found to be strikingly abnormal (virtually absent).It would appear, therefore, that von Willebrand’s disease forms a spectrum, and whether the kindreds reported simply reflect variations of a single genetic disease state or represent separate entities will be answered only by clarification of the underlying etiology of that disease.

EDTA‑dependent pseudothrombocytopenia in child (clinical case report)

2021 ◽  
pp. 51-54
Author(s):  
N. A. Sokolova ◽  
M. I. Savina ◽  
O. S. Shokhina
Keyword(s):  
Platelet Count ◽  
Clinical Case ◽  
Sodium Citrate ◽  
Ethylenediaminetetraacetic Acid ◽  
Hemorrhagic Diathesis ◽  
Platelet Dysfunction ◽  
Rare Presentation ◽  
Blood Samples ◽  
Low Platelet Count ◽  
Aggregation Of Platelets

Ethylenediaminetetraacetic acid (EDTA)-dependent pseudothrombocytopenia is the phenomenon of a spurious low platelet count due to antiplatelet antibodies that cause platelet clumping in blood anticoagulated with EDTA. The aggregation of platelets in EDTA-dependent pseudothrombocytopenia is usually prevented by other anticoagulants, such as sodium citrate. EDTA-dependent pseudothrombocytopenia has never been associated with hemorrhagic diathesis or platelet dysfunction. In this article, a 2,5-year-old boy with EDTA-dependent pseudothrombocytopenia is presented because of rare presentation. We report that EDTA can induce platelet clumping, and thus spuriously low platelet counts. However, aggregation of platelets was not detected in blood samples with sodium citrate, and platelet count was normal.

Comparison of the O’Brien Filter Test and the PFA-100 Platelet Analyzer in the Laboratory Diagnosis of von Willebrand’s Disease

2000 ◽  
Vol 84 (07) ◽  
pp. 88-92 ◽  
Author(s):  
Agota Schlammadinger ◽  
Adrienne Kerenyi ◽  
Laszlo Muszbek ◽  
Zoltan Boda
Keyword(s):  
Bleeding Time ◽  
Second Phase ◽  
Bleeding Tendency ◽  
High Shear ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Time Determination ◽  
Filter Test ◽  
Type 3

SummaryVon Willebrand’s disease (vWD) is the most common congenital haemorrhagic diathesis, characterized by the quantitative or qualitative disorder of von Willebrand factor (vWF). A number of methods have been used for the diagnosis of the disease, and the bleeding time determination is widely accepted as a screening test in spite of its low sensitivity. Our aim was to evaluate and compare the performance of two high shear systems (the O’Brien filter test and the PFA-100 device) in the screening and diagnosis of vWD. Thirty patients (n=13 type 1 with mild symptoms, n = 9 type 1 with severe symptoms, n = 2 type 2A, n = 3 type 2B and n = 3 type 3 vWD) and twenty controls were investigated. In mild vWD the platelet retention in the second phase of the filter test with citrated blood showed the highest sensitivity (91.6%). The sensitivity of the PFA-100 method with collagen-epinephrine cartridges in this group was 76.9%, while the bleeding time was prolonged only in 15.4% of the cases. In severe type 1, in type 2A and type 3 all functional tests reflected the bleeding tendency of the patients. In type 2B disease the bleeding time was prolonged only when the patient was thrombocytopenic, but both high shear systems revealed the disease independently of the presence of thrombocytopenia. The overall sensitivity of the bleeding time determination was 50% compared to the 80-90% sensitivity of the O’Brien filter test and the PFA-100 system. The sensitivity values of the filter test and the PFA-100 device with collagen-epinephrine cartridges were in the same range, but the collagen-ADP cartridges showed a lower (65.5%) sensitivity, though the results were specific and had high positive predictive value. We conclude that both high shear systems are suitable for the screening of vWD, and that they are superior to the traditional bleeding time determination in case of mild disease or type 2B vWD.

scholarly journals DDAVP in type IIa von Willebrand's disease

Blood ◽  
1986 ◽  
Vol 67 (2) ◽  
pp. 465-468 ◽  
Author(s):  
HR Gralnick ◽  
SB Williams ◽  
LP McKeown ◽  
ME Rick ◽  
P Maisonneuve ◽  
...  
Keyword(s):  
Factor Viii ◽  
Protease Inhibitors ◽  
Von Willebrand Factor ◽  
Bleeding Time ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Time Period ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Ristocetin Cofactor

Abstract 1-D-Amino(8-D-arginine)-vasopressin (DDAVP) infusion in three patients with type IIa von Willebrand's disease (vWD) resulted in a normalization of the factor VIII coagulant, factor VIII-related antigen, and von Willebrand factor (vWF) (ristocetin cofactor) activities and the bleeding time. The normalization of these hemostatic parameters persisted for four hours. Over the same time period there was a marked increase in the quantity of the vWF multimers when blood was collected in the presence of protease inhibitors. The vWF multimers present were even larger than the normal. When blood was collected in the absence of protease inhibitors, a smaller increase in the plasma vWF multimers was observed and fewer of the intermediate and larger vWF multimers were seen; multimers larger than those present in normal plasma were not visualized. The platelet vWF multimers and activities did not change with or without inhibitors. These studies suggest that there is a subgroup of patients with type IIa vWD who respond to DDAVP with complete normalization of their hemostatic abnormalities and whose vWF is sensitive to proteolysis.

scholarly journals The Aspirin Bleeding Time - A Screening Test for Evaluation of von Willebrand’s Disease

1977 ◽  
Author(s):  
Marie J. Stuart ◽  
Merrill Miller ◽  
Joel Wolk ◽  
Fredrick Davey
Keyword(s):  
Screening Test ◽  
Patient Population ◽  
Bleeding Time ◽  
Bleeding Diathesis ◽  
Repeated Testing ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Group 4 ◽  
The Mean ◽  
Platelet Defects

In an attempt to elucidate the usefulness of the Bleeding Time (BT) post Aspirin (ASA) ingestion this test was done with other tests of coagulation in 30 controls and 77 patients evaluated for a possible bleeding diathesis. Coagulation studies included PT, PTT, TT, VIII AHF & AGN, IX, XI, XIII platelet retention and aggregation, and modified Ivy BTs pre and 2 hrs. post 600 mgms ASA. The mean control BT in 25 normals was 3.6'±3.2' (3SD). Following ASA the BT was 6.4'±4.1' (3SD). 5/30 “controls” without bleeding histories had abnormal BTs post ASA. In this group, 4 were proven to have unrecognized von Willebrand’s disease (VWD) and one a platelet defect. Of the 74 patients studied, 28 had initial BTs that were abnormal (23 with VWD and 5 with platelet defects). Of the remaining 46 with initially normal BTs, 26 had abnormal BTs post ASA. 13/26 had VWD at their first evaluation. In 9/26, however, the abnormal BT post ASA was the only abnormality at initial evaluation. On repeated testing, these patients were also shown to have VWD. 4/26 with abnormal BTs post ASA were found to have platelet abnormalities. The remaining 20 patients had normal BTs pre and post ASA. 16/20 revealed no hemostatic abnormality. In 4 eventually proven to have VWD, the BT post ASA was normal. The use of the BT post ASA raised the sensitivity of the BT as a screening test from 48% to 93% in the abnormal patient population. VWD appears to be the most common symptomatic/ asymptomatic bleeding disorder. The BT post ASA is a valuable screen in the evaluation of hemostatic defects i.e., mainly VWD, as also the occasional patient with a thrombocytopathy.

scholarly journals Antibody-induced von Willebrand's disease: a newly defined inhibitor syndrome

Blood ◽  
1976 ◽  
Vol 48 (3) ◽  
pp. 393-405 ◽  
Author(s):  
RI Handin ◽  
V Martin ◽  
WC Moloney
Keyword(s):  
Bleeding Time ◽  
Factor Vii ◽  
Human Igg ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Laboratory Evidence

Abstract A patient is described with clinical and laboratory evidence of von Willebrand's disease (VWD) in association with lymphosarcoma. He consistently had a bleeding time of over 20 min; factor VIIAHF, VIIAGN, and VIIVWF under 20%; and severe, diffuse hemorrhage, Following transfusion with cryoprecipitate, the patient had the expected rise in VIIAGN and VIIAHF. The patient's plasma contained an inhibitor which prevented aggregation of normal platelets by ristocetin, but which did not interfere with the measurement of VIIAGN or inactivate VIIAHF activity. The inhibitor was present in serum heated at 56 degrees C for 30 min, was present in a purified IgG fraction of serum, and was precipitated by anti-human IgG. The antibody did not interact directly with washed platelets, but appeared to interact with that portion of the factor VII protein that supports ristocetin aggregation (VIIIVWF). The data provide the first explanation for the pathophysiology of the acquired von Willebrand's syndrome.

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