2003; baxter - Supplements - Haematologica

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[Round Table on Immune Tolerance Treatment]High infection rate in portsused for immune tolerance.The Spanish Registryreview paperhaematologica 2003; 88(suppl. n. 12):42-44http://www.haematologica.org/free/immunotolerance2001.pdfJ. TUSELL, A. VILLAR, MF. LOPEZ-FERNANDEZ, S. HAYA,D. BRITO, R. SOSA, F. RODRIGUEZ-MARTORELLSpanish Ports Study Group, SpainThere is no doubt that port-a-caths (ports)are an extremely useful tool in the currenttreatment of hemophilia. Many hemophiliacchildren with problems of venous access areundergoing a home prophylactic treatment programmeor have completed immune toleranceinduction (ITI) treatment thanks to thesedevices. Their use is widespread in a number ofcountries. However, they are not free of certaincomplications, of which the most frequent areinfections and thromboses.In Spain, port use in hemophilia has most frequentlybeen for ITI programmes in patients withinhibitors. The results presented here, from theSpanish Registry, are for these pediatric patients,in whom a high infection rate has been observed.AimsTo evaluate port use for facilitating ITI treatmentto factor VIII in a pediatric population ofhemophiliacs with inhibitors. To evaluate thepresence of infections and their potential influcencein achieving tolerance.MethodsCompletion of a questionnaire on port insertion,use, and associated complications. Thequestionnaire is distributed to all treatment centresin the country for patients with bleeding disorders.It is completed when a port is inserted,after which it is updated yearly.ResultsThirty ports have been used in 21 severehemophilia A patients with inhibitors, in orderto carry out ITI treatment. Nine patients requireda second port. Insertion was at an average age of4.2, ranging from 16 months to 16 years. Theinhibitor titer at the moment of insertion wasbetween 1 and 789 BU, with an average of 92BU. Factor VIIa was used for surgical coverage in16 cases, in a further 8 cases factor VIII was usedand in 6 other cases activated prothrombincomplex concentrates (aPCC) were used. On 22occasions the ports were inserted in the subclavianvein, 6 were inserted in the external jugular,and 2 more in the saphenous. There wereone or more infections in 20 out of the 20 ports(66%). The rate of first infection per 1000 daysof use and per port was 2.24. The most frequentgerms, on order of frequency, were Staphylococcusepidermidis, Staphylococcus aureus, Pseudomonas,Klebsiella, Proyteus and Escherichia Coli.In nine cases, removal and replacement of theport was required due to the persistence of theinfection, despite local and systemic antibiotictreatment. Two of the second ports also becameinfected. Of the 19 patients who completed theITI treatment, 14 successfully achieved tolerancewhile the other 5 failed. Out of the 19 cases, theport presented infections in 9 while 10 remainedinfection free. Of the 5 cases in which tolerancewas not achieved, 4 presented infections. Of the10 cases free from infection, tolerance wasachieved in 9 and there was one failure (Table 1).DiscussionThere is a wide range of results (expressed inpercentage of infected ports) in relation to portinfections in hemophilia, varying from 10 to60%, and in the infection rate per 1000 days ofport use, varying from 0.14 to 0.76 (Table 2). 1–15It must be born in mind that some studies areTable 1. Success and failure of ITI in relation to the presenceof infections in ports.Patient (%) Infection (%) No infection (%)Tolerance 14 (74) 5 (36) 9 (64)No tolerance 5 (26) 4 (80) 1 (20)Completed ITI 19 9 10haematologica vol. 88(supplement n. 12):september 2003
IV International Workshop on Immune Tolerance in Hemophilia43Table 2. Percentage of port infections and infection rateper 1000 days of use, as published in literature.Table 3. Published percentage of infected ports used inhemophilic patients with inhibitor.% Rate%Rothschild 1 10 0.15Miller 2 13 0.14Ljung 3 17 0.19Manco-Johnson 4 22_Perkins 5 33 0.7Collins 6 34_Ragni 7 38_Warrier 8 40_Shapiro 9 45 0.26Geraghty 10 45_Blanchette 11 47 0.7Liesner 12 48 0.67Bollard 13 48_Santagostino 14 50 0.73Tusell 15 60 0.76Ljung 17 50Ragni 7 52Collins 6 58Tusell* 66Ljung 18 71Liesner 12 83Average 63Average 36 0.46still at a preliminary stage, so the number ofpatients and ports are low and the follow-upperiod short, causing values to vary over time andwith the addition of more patients. The resultsshould therefore be interpreted with caution. Onsome occasions, as in the Spanis register, the highincidence is due to port being used for ITI inpatients with inhibitors. It is well known that inthese cases there is a much higher infection rate,probably because treatment involves greater useof the port and there is no normalization inhemostasis. This leads to frequent hematomaswhere the skin is punctured, which, in turn,facilitates the presence of infections at this siteand the entrance of germs into the system. 16There is also a wide range of results (50-83%) inthe much higher percentages of infections inthese cases (Table 3).Although the serious consequences of theseinfections have not been described, they mustnot be underestimated, as they are bacterialprocesses, some of which are potentially very seriousgram-negative germs, 19 resistant to local andsystemic treatment, and, in many cases, requiringthe removal and replacement of the port.The role of infection in inhibitor developmentand their influence in the results of ITI are a subjectof some debate. 20 In the Spanish register, ofthe 5 patients who did not achieve tolerance, 4had presented infections in the port-a-cath, andof the 10 who did not suffer infections in theports, 9 achieved tolerance. Although the numberof cases is low, a negative effect of infectionson the results of immune tolerance can still bedetected. The inhibitor titer before and afterinfections may also be worth observing. Thrombosisrepresents a further complication withports and one that is of growing concern.Although no cases have yet been referred to theSpanish register, a prevalence of up to 18% 21 hasbeen published and there is a general increase incases, particularly when the period of use is prolongedfor more than 4 years.ConclusionsThere is no doubt that port-a-caths are a genuinelyefficacious tool in current hemophiliatreatment and especially in ITI. However, thehigh rate of complications - particularly infections- associated with port use in patients withinhibitors, as observed in the Spanish and otherstudies, needs to be evaluated. Infections maynegatively influence the results of ITI and someof them are potentially serious. It may be wise toexhaust the possibilities of administration viaperipheral veins before taking a decision onwhether to implant a port for ITI. Should implantationbe required then the most thorough antisepticmeasures must be taken.References1. Rotschild C, Mercks J, Torchet MF, Pertuiset N. Experiencewih implantable venous access devices inhaemophilia children treated intensively: a low rate ofcomplications. Haemophilia 2000; 6:276.2. Miller K, Buchanan GR, Zappa S, Cochran C, LaufenbergJ, Medeiros D, et al. Implantable venous accessdevices in children with hemophilia: a report of lowinfection rates. J Pediatr 1998; 132/934-8.3. Ljung R, van den Berg M, Petrini P, Tengborn L, ScheibelE, Kekomaki R, et al. Port-A-Cath usage in children withhaemophilia: experience of 53 cases. Acta Paediatr1998; 87:1051-4.4. Manco-Johnson M, Nuss R, Abshire T, Richardson L,Shapiro A, Valentinos L. Port infections in children withhaemophilia. The Hemophilia Joint Preservation StudyGroup. Haemophilia. 2000; 6:12a[abstract].5. Perkins JL, Johnson VA, Osip JM, Christie BA, Nelsonhaematologica vol. 88(supplement n. 12):september 2003
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2003; baxter - Supplements - Haematologica

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