Catheter-related deep venous thrombosis in children with hemophilia

Abstract

Central venous catheters (CVCs) are a common adjunct to hemophilia therapy, but the risk of CVC-related deep venous thrombosis (DVT) in hemophiliacs is not well defined. In a previous study, 13 patients with CVCs had no radiographic evidence of DVT. However, recent abstracts and case studies demonstrate that DVT does occur. Therefore, this study sought to determine the frequency of DVT in children with hemophilia and long-term CVCs and to correlate venographic findings with clinical features. All hemophilia patients with tunneled subclavian CVCs in place for 12 months or more were candidates for evaluation. Patients were examined for physical signs of DVT and questioned about catheter dysfunction. Contrast venograms were obtained to identify DVT. Fifteen boys with severe hemophilia were evaluated, including 9 from the initially studied group of 13. Eight patients had evidence of DVT, 5 of whom previously had normal venograms.

Catheter-related thrombosis in children with hemophilia A: evidence of a multifactorial disease

“…In one other patient thrombophilia screening awaits parental consent. Similar to patient number 2 in Journeycake et al, 1 the heterozygous factor V 20210GϾA mutation was present in 2 of our 7 patients. Additionally, 4 patients were treated with factor (F) VIII/vWF concentrates, and 1 child each received purified FVIII, recombinant VIIa, purified FVIII, and recombinant FVIII, respectively.…”

“…Although bleeding symptoms correlate with the levels of the remaining factor activity, it is reported that some hemophilic subjects with factor VIII levels below 1% do not all bleed with the same severity, and in rare cases of severe HA, thrombotic episodes have also been reported in childhood. [1][2][3][4][5][6][7][8] Since the reported symptomatic vascular accidents in hemophiliacs are, in the majority of cases, related to central venous lines (CVLs), we have read with interest the paper by Journeycake et al 1 Their data demonstrated that hemophiliacs with tunneled subclavian CVLs in place for more than 48 months had abnormal venograms. 1 In addition, 5 of 15 hemophiliacs (33.3%) had symptomatic deep venous thrombosis (DVT) related to the CVLs; 3 further patients (20%) with signs of DVT on contrast venography had no clinical problems.…”

“…[1][2][3][4][5][6][7][8] Since the reported symptomatic vascular accidents in hemophiliacs are, in the majority of cases, related to central venous lines (CVLs), we have read with interest the paper by Journeycake et al 1 Their data demonstrated that hemophiliacs with tunneled subclavian CVLs in place for more than 48 months had abnormal venograms. 1 In addition, 5 of 15 hemophiliacs (33.3%) had symptomatic deep venous thrombosis (DVT) related to the CVLs; 3 further patients (20%) with signs of DVT on contrast venography had no clinical problems. Because CVLs are a common adjunct to therapy of severe hemophiliacs, we would like to add some additional information on risk factors that, besides the CVLs themselves, are of importance in the development of CVLassociated vascular accidents in these patients.…”

Enhanced pharmacokinetic properties of a glycoPEGylated recombinant factor IX: a first human dose trial in patients with hemophilia B

“…8,11,12 The need for frequent dosing, especially in pediatric patients, presents challenges in terms of repeated venous access that may necessitate the placement of indwelling catheters that is associated with frequent complications such as thromboses and infections. 13,14 Furthermore, frequent dosing interrupts daily activities and therefore, lack of compliance with a frequent injection regimen is one of the most commonly cited reasons for failure of prophylaxis. 15 Similar to patients with mild or moderate hemophilia B, patients with severe hemophilia B would also benefit from a single effective dose, instead of multiple doses, when they experience breakthrough bleeding episodes during prophylaxis.…”

Thrombophilia in Children: Who to Test, How, When, and Why?

“…23 The majority of CVC-VTE are sub-clinical and not accompanied by a swollen, painful, or erythematous extremity. [24][25][26] In a prospective, multicenter cohort study of children with acute lymphoblastic leukemia (ALL), 29 of 85 (34%) developed CVC-VTE within the first month after CVC placement, but only 1 of these 29 patients had clinical symptoms. No patient tested positive for the factor V Leiden or prothrombin gene mutation.…”