Prolonged Bleeding Time in Children and Young Adults with Hemophilia

PEDIATRICS ◽  
1980 ◽  
Vol 66 (6) ◽  
pp. 951-955
Author(s):  
George R. Buchanan ◽  
Christine A. Holtkamp

Of 49 patients with hemophilia A or B, who had not received replacement transfusions for at least 72 hours, ten were demonstrated to have a prolonged value for the bleeding time. None had evidence of von Willebrand's disease or prior aspirin ingestion, and only one was found to have an associated intrinsic platelet defect. It appears that the bleeding time, generally thought to be a measurement of platelet-subendothelium interaction, may also sometimes be prolonged in congenital coagulation disorders in which platelet function is normal.

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1137-1137
Author(s):  
Clara PW Klerk ◽  
Djamila E Issa ◽  
Johannes C Baayen ◽  
Sonja Zweegman

Abstract Abstract 1137 Introduction Various haemostatic abnormalities have been reported in association with use of the anti-epileptic drug valproic acid (VPA). When neurosurgical procedures were performed in patients on VPA, our center performed extensive haemostatic screening preoperatively. However, the clinical importance of abnormal findings is unclear. In the current report we evaluate the need for elaborate haemostatic investigations and make recommendations for clinical practice. Materials and Methods We reviewed medical records of all neurosurgical patients on VPA in our university clinic from February 2006 through May 2011 and recorded outcome of the extensive haemostatic screening, any perioperative measures to prevent extensive blood loss and the occurrence of bleeding complications during or after surgery. Results A total of 65 consecutive patients using VPA had a neurosurgical procedure performed and underwent preoperative haemostatic screening. In 34 patients persistent laboratory abnormalities were found and the proportion was similar in the 13 patients who reported a bleeding tendency as in the 52 who did not. However, 5/6 patients who reported a clear time relation between start of VPA use and their bleeding tendency were found to have lab abnormalities. Five of the 65 patients had a mild thrombocytopenia (range 100–150 *10E9/L). 17/63 patients had a prolonged bleeding time of which 5 had normalized on repeat testing. 9/65 had a decreased von Willebrand cofactor activity. Only 2 of these had a prolonged bleeding time. Twelve patients had abnormal platelet function tests but in 3 these normalized on repeat testing. CT and PT were normal in all patients and fibrinogen was marginally lowered in 14/65 (lowest 1.2 g/L). Factor XIII was tested in 57 patients and no clinically relevant lowering was perceived (lowest 0.47 IU/mL). Clinical consequences of the laboratory results were left to the treating haematologist. Additional perioperative measures were taken in 15 patients (stopping VPA in 3, administering DDAVP in 12 and tranexaminic monotherapy in one). Two of 65 patients had a bleeding complication: in one no bleeding time was performed but all other tests were normal. Despite tranexaminic acid perioperatively she developed a large subdural hematoma and seizures. None of the other patients with normal lab values had a bleeding complication. The second patient had had uncomplicated non-neurological surgery during VPA use and was found to have a mildly abnormal ATP/ADP ratio. Because of his uncomplicated history no additional measures were taken and he developed a large epidural hematoma. Conclusion In our cohort of 65 neurosurgical patients using VPA, none had clinically relevant abnormalities in PT, aPTT, thrombocyte count, fibrinogen or fXIII level. Therefore, these tests appear unnecessary in the preoperative screening of patients on VPA. However, even in the presence of a normal bleeding time, clinically relevant lowering of von Willebrand factor, and abnormalities in platelet function should actively be sought after. In these cases, we recommend treatment with DDAVP perioperatively. Unlike others (Kreuz et al, Epilepsia 1992), we found DDAVP to be safe and effective in our neurosurgical population. Whether an isolated prolonged bleeding time in absence of demonstrated platelet abnormalities necessitates additional perioperative measures should be the subject of further study. In our cohort the majority of patients with an unexplained prolonged bleeding time received DDAVP perioperatively. Disclosures: No relevant conflicts of interest to declare.


1977 ◽  
Author(s):  
S. M. Gerritsen ◽  
J. W. N. Akkerman ◽  
J. J. Sixma

Four patients with Hermansky-Pudlak Syndroora (HPS)— storage pool deficiency, albinism and ceroid containing bone marrow macrophages — from one family, one unrelated hps patient and one patient with storage pool deficiency (SPD) were alternately treated with cryoprecipitate from 16 donors or an equal amount of human albumin. Prior to infusion of cryoprecipitate template bleeding times were all longer than 20 min. Within 2 hrs after infusion bleeding times decreased 70% of initial values. This affect lasted for at least 6 hrs but had disappeared after 24 hrs. Infusion of albumin had no effect. Similar results were obtained in 2 additional cases of SPD that were treated with cryoprecipitate only. The abnormal platelet function tests and the biochemical abnormalities remained unchanged. Infusion of cryoprecipitate protected 4 SPD patients from bleeding during surgery. Infusion of cryoprecipitate may prevent bleeding in spd patients. Its mechanism is still obscure.


1962 ◽  
Vol 08 (03) ◽  
pp. 511-523
Author(s):  
G. J. H den Ottolander ◽  
A Bleijenberg

SummaryA prolonged bleeding time can be due to a vascular, plasmatic, or thrombocytic cause. On the basis of investigations in 8 patients with a prolonged bleeding time, these different forms are discussed.There appeared to be at least two plasmatic factors one of which is the bleeding factor of Nilsson. The demonstration of a thrombocytic cause is sometimes only possible with transfusions of normal platelet suspensions. A patient is described with a prolonged bleeding time due to an acquired thrombo-pathia, resulting from excessive haemorrhages and exchange transfusions.


Blood ◽  
1960 ◽  
Vol 15 (6) ◽  
pp. 807-829 ◽  
Author(s):  
GIOVANNI RACCUGLIA ◽  
JAMES V. NEEL ◽  
Ruth T. Davidson ◽  
Mary Jane Ussery

Abstract 1. A kindred of 311 individuals, many members of which are affected by a hemorrhagic diathesis, has been described. 2. The variability in the manifestations of this diathesis is extreme. In its fullest expression the disease is characterized by a prolonged bleeding time with evidence of a morphologic defect in the platelets, and a deficiency in antihemophilic globulin. Some possibly affected individuals exhibit only a prolonged bleeding time, while, on the other hand, the clinically most severely affected individual, with AHF levels on several occasions of 5 to 10 per cent, has not been observed by us to have a prolonged bleeding time, although his platelets are morphologically abnormal. 3. Genetic analysis suggests that the hemorrhagic tendency is determined by a single dominant gene of variable penetrance and expressivity. 4. No satisfactory explanation can be developed on the basis of these studies for the association between platelet abnormality and AHF deficiency. More specifically, it is impossible to conclude whether the platelet defect is precursor to the AHF deficiency, or whether—as on a priori grounds seems less likely—this is an example of true genetic pleiotropy. 5. The terminologic chaos which afflicts the literature on hemorrhagic diatheses characterized by a prolonged bleeding time is discussed in the light of the findings in this one large kindred, and suggestions are advanced for minimizing confusion based on terminology alone.


Sign in / Sign up

Export Citation Format

Share Document