Central Journal of Hematology & Transfusion Bringing Excellence in Open Access Case Report *Corresponding author Vincent Hennaux, Biological Hematology Department, Hospital Institute Famenne Ardenne Condroz, IFAC- Successful Major Surgical VIVALIA, 21 rue du vivier, 6900 Marche-en-Famenne, Belgium, Tel: 32-84-245524; Fax: 32-84-374820; Email: Procedures in a Severe FXI Submitted: 22 February 2016 Accepted: 07 April 2016 Deficient Patient Using Fresh Published: 12 April 2016 ISSN: 2333-6684 Copyright Frozen Plasma: A Case Report © 2016 Hennaux et al. 1 1 2 Vincent Hennaux *, Stéphanie d’Otreppe , Benoit Guillaumie , OPEN ACCESS Jean-Luc Jacquemin3 and Celine Rousie4 1Department of Biological Hematology, Hospital Institute Famenne Ardenne Condroz, Keywords Belgium • FXI deficiency 2Department of Orthopedic Surgery, Hospital Institute Famenne Ardenne Condroz, Belgium • Surgery 3Department of Anesthesia, Hospital Institute Famenne Ardenne Condroz, Belgium • Fresh Frozen Plasma 4Department of General Surgery, Hospital Institute Famenne Ardenne Condroz, Belgium • OctaplasLG • aPTT Abstract Replacement therapy is desirable in case of surgical procedures in patients with severe FXI deficiency or milder deficiencies with a disposition to severe bleeding. Either FXI concentrates or FFP can be used in this setting. We report here the positive outcome of a series of consecutive surgical procedures in an aged patient with severe FXI deficiency treated with FFP and monitored with aPTT. In the two most recent major surgeries, the total dose of FXI administered pre-operatively was around 29 IU/kg (=mL/kg) during the first surgical procedure and around 19 IU/kg during the second one. Surgical hemostasis was judged “adequate” during these two major surgical procedures. APTT was close to upper normal value prior surgery and maintained ≤ 60 seconds at least 72 hours post-op. In the last major surgery both a PTT and FXI levels were monitored. FXI in-vivo recovery was found to be above 2%/IU/kg. Pre-op FXI plasma value was 45%, reaching the recommended value of 45 IU/dL for major surgeries. The post-op FXI plasma level was then rapidly below this value without any associated bleeding tendency. The clinical and aPTT monitoring were indeed not indicative of any additional transfusion need during the post-op period. So, FXI substitutive therapy using FFP monitored by aPTT was a safe and efficient procedure for planned consecutive surgical procedures. Clinical and aPTT-based laboratory monitoring of the patient led to a lesser intensive post-operative transfusion protocol than the protocol followed if the patient’s monitoring would have been based on the recommended FXI plasma levels. ABBREVIATIONS phenotypes unrelated to patient’s FXI plasma levels [1,2]. In case aPTT: activated Partial Prothrombin Time; cm: centimeter; disposition to severe bleeding, it is generally considered indicated dL: deciliter; FXI: coagulation factor XI; FFP: Fresh Frozen of severe FXI deficiency (FXI≤20%) or milder deficiencies with a Plasma; Hgb: hemoglobin; IU: international unit; Kg: kilogram; [3]. Substitutive treatment modalities include administration Max: maximum; Min: minimum; mL: milliliter to correct at least partially the FXI deficiency before surgery INTRODUCTION bleedingof FFP or tendency. FXI concentrate. Considering Treatment the long biological with antifibrinolytics, half-life of FXI We report here the successful completion of multiple surgical desmopressin and/or fibrin sealant can also help controlling the hours allow correcting the bleeding tendency with a limited risk ® patient substituted with Fresh Frozen Plasma (FFP) (Octaplas of(around volume 60 overload. hours), pre-operative While measurement administration of thrombin of FFP generation over 48 procedures in a® severe coagulation factor XI (FXI) deficient and OctaplasLG , Octapharma) and followed-up with activated by TGA (Thrombin Generation Assay) could probably be the most Partial Thrombin Time (aPTT) and/or FXI assays. predictive test to perform for monitoring FXI deficient patients FXI deficiency is a rare bleeding disorder with bleeding during the course of a surgical procedure [4,5] this test deserves Cite this article: Hennaux V, d’Otreppe S, Guillaumie B, Jacquemin JL, Rousie C (2016) Successful Major Surgical Procedures in a Severe FXI Deficient Patient Using Fresh Frozen Plasma: A Case Report. J Hematol Transfus 4(1): 1042. Hennaux et al. (2016) Email: Central Bringing Excellence in Open Access further standardization and is not feasible in routine. Follow-up of FXI levels is recommended while considered not correlated to bleeding risk and not easily available in routine. So, correction of aPTT is frequently used for the monitoring of patients’ haemostatic potential. CASE PRESENTATION preoperativeThe (female) assessment patient’s of FXIbleeding deficiency risk (isolatedwas diagnosed prolonged in aPTT)2006 (FXI=4%)prior to aat mastectomy the age of for66 yearsbreast at adenocarcinoma. the occasion of Thethe patient was successfully operated under FFP treatment. In without problem. October 2012, a total hip replacement was performed under FFP Figure 1 Evolution of Hgb and aPTT over time and transfusion of blood be performed because of neoplasm. aPTT was measured during components. Green arrows indicate transfusion: pre-op (2FFP+1PRC; In March 2013, a sigmoidectomy with lateral ileostomy had to (Stago) according to the routine validated standard operating 5FFP; 4FPP) and post-op (1FFP+1PRC) FFP = Octaplas. procedurethe perioperative of the laboratory.period using At STA-Cephascreenhospital entry, patient’s 10 reagent aPTT was 101 seconds (normal laboratory range: 25-35 seconds)®, and hemoglobin (Hgb) 10.1 g/dL (normal laboratory range: of11-14 92 kg) g/dL). preceding She received the operation 11 units and of one200 redmL cellFFP concentrate. (Octaplas Octapharma) over the 48 hours (total 24 mL/kg for a weight substitutive therapy and allowed eliminating the presence of a FXIPre-operative inhibitor. Onmonitoring the day of of the aPTT operation, indicated aPTT the was efficacy 37 seconds of the starting the operation. The day after surgery, her aPTT was and Hgb was 10.9g/dL, which was considered satisfactory for as well as 2 units of red blood cell concentrate because of low 40 seconds and 1 additional unit of plasma was administered Figure 2 aPTT and FXI over time and transfusion of blood components. hemoglobin (8.3g/dL). Post-transfusion aPTT was 38 seconds; Yellow arrows indicates OctaplasLG® transfusion (2 units and then 6 units) pre-op. onpost-transfusion peri-operative Hgb bleeding was 9.5 and g/dL. hemostasis Six days after has thebeen operation judged “adequate”her aPTT was with 63 no seconds. excessive Efficacy blood oflosses the observed.transfusion (Figure protocol 1) summarizes the biological follow-up and the transfusion protocol used for this surgical procedure. AfterFXI values OctaplasLG below ®5%. administration, aPTT was 91 aPTTsec (Normal was within values: normal 25-37 sec) and FXI plasma level was 2% prior to plasma transfusion. implantation of a Port Chamber (port-a-cath) with no bleeding One month later, the patient received 8 units of FFP for the values (34 sec) and FXI plasma level reached 45%. Considering the total dose of FXI administered (1,600 IU) and the patient’s problem. In November 2013, reimplantation of the lateral of FFP. body weight (85kg), and the observed increased of FXI plasma ileostomy was performed successfully with transfusion of 8 units conductedlevel (45%-2%=43%), without any theabnormal overall bleeding. observed No in-vivo additional recovery plasma in replacement) was planned for August. FXI inhibitor was checked transfusionour patient andis 2.28%/IU/kg. no other blood The products surgical were procedure transfused has beenperi- andIn was June found 2014, negative a new majorat that surgical moment procedure besides (totalthe pasthip operatively. Surgical hemostasis was judged “adequate” by the repeated administrations of FFP. At this occasion, transfusion surgical team. Peri-operative aPTT and FXI values are presented (OctaplasLGefficacy has ®been, Octapharma) monitored duringhave beenthe perioperative administered period to the by DISCUSSIONin the figure 2 below. both aPTT and FXI plasma levels. 8 units (1,600 mL) of plasma Replacement therapy is desirable in case of surgical validatedpatient over standard 48 hours operating prior to surgery.procedure aPTT of wasthe measuredlaboratory. using FXI STA-Cephascreen 10 reagent (Stago) according to the routine amongprocedures others, in patientsthe risk withof inhibitor severe FXIappearance, deficiency the or surgical milder CK PREST reagents (Stago) according to the validated standard proceduredeficiencies shouldwith a dispositionbe absolutely to severe indicated bleeding. and Because planned of, operatingplasma levels procedure were measured in place inusing the laboratory.STA-deficient The XI procedure and STA- presence of FXI inhibitor is recommended especially in case of meticulously in severe FXI deficient patients. Testing for the implies duplicate testing and specific dilutions to measure J Hematol Transfus 4(1): 1042 (2016) 2/4 Hennaux et al. (2016) Email: Central Bringing Excellence in Open Access the aPTT-based laboratory and clinical monitoring performed butsevere should deficiency be used, and as recommendedhistory of transfusion. in their Summary FXI concentrates, of Product if does5 days not is indicateconsidered the toneed be usuallyfor maintaining sufficient this [3,7].