A Relatively Low Frequency of Bleeding in Patients with Severe Factor XI Deficiency Who Undergo Surgery Involving Tissues with Low Fibrinolytic Activity Supports on Demand Rather Than Prophylactic Use of Plasma.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4036-4036
Author(s):  
Ophira Salomon ◽  
David M. Steinberg ◽  
Uri Seligsohn
Keyword(s):  
Fibrinolytic Activity ◽  
Oral Surgery ◽  
Low Frequency ◽  
Fresh Frozen Plasma ◽  
Bleeding Tendency ◽  
Factor Xi ◽  
Factor Xi Deficiency ◽  
On Demand ◽  
Number Of Patients ◽  
Fresh Frozen

Abstract Severe factor XI (FXI) deficiency is an injury related bleeding tendency. Fresh frozen plasma (FFP) or FXI concentrate are usually used for achieving hemostasis in such patients undergoing surgery but this can lead to inhibitor formation in 33% of patients with FXI level <1 U/dL (Blood101:4783, 2002). Based on observations in a limited number of patients with severe FXI deficiency, we previously suggested that surgery at tissues with known fibrinolytic activity is accompanied by bleeding more frequently than in other sites (NEJM325:153, 1991). In this study we retrospectively assessed the frequency of post surgical bleeding in 165 unrelated patients with severe FXI deficiency who were not treated by hemostatic means. Criteria for bleeding included local hematoma, need for surgical arrest of bleeding, or use of blood components. Bleeding occurred in 7/57 (12.3%) patients operated at tisues with low fibrinolytic activity, and in 29/48 (60.4%) patients operated at fibrinolytic sites (Table). Bleeding following circumcision (a non-fibrinolytic site) was accompanied by bleeding only in 1/64 (1.6%) and following oral surgery in 55/110 patients (50%). The relatively low frequency of bleeding following surgery at non-fibrinolytic sites advocates on demand rather than prophylactic replacement therapy when such surgery is performed. SITE OF SURGERY N BLEEDERS % BLEEDERS Non-fibrinolytic Appendicectomy 22 1 1.6 Gastric 3 0 0 Cholecystectomy 5 1 20 Herniotomy 13 3 23 Hysterectomy 5 2 40 Orthopedic 9 0 0 Fibrinolytic Tonsillectomy 33 20 60.6 Nose 12 7 58.3 Prostatectomy 3 2 66.6 Circumcision 64 1 1.6 Oral Tooth extractions 100 49 49 Gum 10 6 60

The Use of Factor XI Concentrates (Hemoleven, LFB) in Patients with Congenital Factor XI Deficiency and a Known Bleeding Tendency.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 1796-1796
Author(s):  
Vickie McDonald ◽  
Savidge F. Geoffrey ◽  
Savita Rangarajan ◽  
Mike Mitchell
Keyword(s):  
Fresh Frozen Plasma ◽  
Incidence Rates ◽  
Treatment Modalities ◽  
Bleeding Tendency ◽  
Normal Range ◽  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Unit Dose ◽  
The Mean ◽  
Congenital Factor

Abstract Traditional treatment modalities for FXI deficiency (UK prevalence 400 cases) include antifibrinolytics, desmopressin, fresh frozen plasma (FFP) and FXI concentrates but there has been reluctance to use FXI concentrates because of reported incidence rates of thrombosis up to 10%. Concerns over the safety and efficacy of FFP, with additional viral inactivation steps possibly leading to reduced FXI recoveries, have led us to increase our use of FXI concentrates. We aimed to assess the indications, dosage, recovery, efficacy and safety of Hemoleven, a plasma derived, purified and virally inactivated FXI concentrate, which also contains heparin and antithrombin, in patients with congenital factor XI deficiency. A retrospective study was performed using hospital notes and laboratory records of all patients who had received Hemoleven over a 2-year period. Eleven patients (6 male, 5 female) had been treated with a median age of 38 years (range 7–74) and mean baseline FXI:C levels of 25.4U/dl (3–50). All patients received Hemoleven as prophylaxis for surgery or dental work and had all previously had excess bleeding when surgically challenged. One patient died of a condition unrelated to FXI treatment. Pre- and post-FXI:C levels were available for a total of 60 treatment episodes of which 25 were 1000-unit doses and 35 were 2000-unit doses. The mean increase in FXI:C per 1000-unit dose was 25.4 U/dl (12.4–43.9) while the mean increase in FXI:C per 2000-unit dose was 50.5 U/dl (11.8–106.5). This is consistent with the manufacturer’s data. Ten minute post infusion FXI:C levels were above the normal range (73–133 U/kg) in 8% of patients given 1000 units and 11% of patients given 2000 units but below the normal range in 24% of patients who received 1000 units and 20% of patients who received 2000 units. 90% of treatment episodes led to FXI:C levels above the usual treatment target of 65 U/dl. Genetic analysis of 9/11 patients showed that 2 were homozygous (one type II and one type III), 6 were heterozygous for other recognised mutations and one had no mutation identified but apparent absence of RNA from one allele demonstrated in a relative by qRT-PCR. No excess bleeding or inhibitor development was recorded even in one patient who had had a poor haemostatic response with FFP. There were no episodes of arterial or venous thrombotic complications within this group and no clinical or laboratory evidence of DIC following treatment. In summary, treatment with factor XI concentrates gave consistent increments in FXI:C at the doses given and achieved good haemostasis with no episodes of thrombosis in this study, even in patients over the age of 60y. While the risk of prion transmission is still unknown, use of FXI concentrates is not associated with the risks of fluid overload and TRALI that are seen with FFP. We acknowledge that the study includes small numbers of patients however the cohort of patients with a bleeding diathesis in this condition is small. We conclude that Hemoleven appears to be an effective and reliable treatment for patients with FXI:C deficiency but should be given in the context of FXI:C level monitoring in order to detect those patients who may develop high levels and possible thrombosis.

Correlation of a Condensed Bleeding Score with New Diagnosis of a Congenital Bleeding Disorder in Patients Referred to a Tertiary Centre

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 1226-1226
Author(s):  
Deepa Ranjani Jayakody Arachchillage ◽  
Tina Biss ◽  
John Hanley ◽  
Kate Talks
Keyword(s):  
Laboratory Tests ◽  
Conflicts Of Interest ◽  
Bleeding Disorder ◽  
Bleeding Tendency ◽  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Number Of Patients ◽  
Abnormal Bleeding ◽  
Bleeding Score

Abstract Abstract 1226 The performance and utility of a condensed bleeding score (Bowman et al, J Thromb Haemost., 2008;6:2062) in relation to the diagnosis of a congenital bleeding disorder in new referrals to a regional haemostasis clinic over an 8 month period is presented. Between November 2010 and June 2011, 50 patients over the age of 16 (median age, 31 years; range, 16–79), including 32 females, were referred for investigation of a possible congenital bleeding disorder following detection of abnormal coagulation results and/or presentation with a bleeding history. A bleeding score was performed as part of their initial assessment. 12(24%) patients were from local referral and 38(76%) patients were referred from other hospitals in the region for further investigation of a suspected bleeding disorder. Basic coagulation tests (activated partial thromboplastin time (APTT), prothrombin time Clauss fibrinogen and platelet count) were normal in the referred patients from other centres. 50% (6/12) of the local referrals were for investigation of a prolonged APTT detected on routine coagulation screening prior to major surgery. The median bleeding score was 6 with a range of −1 to 14 (Table 1). The presence of a congenital bleeding disorder was confirmed in 31 of the 50 patients (62%), including 19/31 (61%) of the female patients and 12/31(39%) of the males. Correlation of an abnormal bleeding score (score ≥ 4) with diagnosis of a congenital bleeding disorder was only seen for diagnosis of type 1 Von Willebrand Disease (VWD) (Table 2). Analysis of the cases with low scores and abnormal results identified two groups of patients; firstly, those who had not yet had a significant haemostatic challenge, and secondly, those in whom the abnormal coagulation results were explained by a non-haemostatically significant reduction in a coagulation factor level (e.g. FVII, 15%; dysfibrinogenaemia; F XII deficiency). These clinically insignificant laboratory abnormalities explain the discrepancy between the number of patients with abnormal laboratory tests (35) and the number of patients diagnosed with a congenital bleeding disorder (31).Table 1Bleeding score (range)Number of patients with normal lab resultsNumber of patients with abnormal lab results−1 to +1382–44105–74128–102311–1422Total1535Table 2DiagnosisNumber of patientsMedian bleeding scoreAge rangeType 1 VWD116 (4–10)17–51Type 2 VWD48 (5–13)17–36Factor XI123 (1–8)17–76Platelet function defect46 (2–9)17–57 Compared to previous reports the range of scores found with this assessment tool was narrow and could not exclude patients from further laboratory assessment. However the condensed bleeding score has only been validated prospectively for the diagnosis of type 1 VWD and all patients in this cohort who were diagnosed with type 1 VWD had an abnormal bleeding score (≥ 4). This observation supports the role of this scoring system in the assessment of patients for type 1 VWD. The use of the condensed bleeding score in assessing patients with suspected factor XI deficiency is difficult due to the lack of a phenotypic relationship between residual factor XI activity and a bleeding tendency. Furthermore, although factor XI deficiency is a rare congenital bleeding disorder in our cohort of patients 12/31(39%) were diagnosed with factor XI deficiency. This may explain the overall lack of correlation between bleeding score and diagnosis of a congenital bleeding disorder. Patients who have an abnormal bleeding score but normal laboratory tests need consideration of further investigations before concluding they are normal. The possibility of an acquired bleeding disorder should be considered. A thorough drug history is also important as one of the patients with a bleeding score of 14 was taking a non-steroidal anti-inflammatory drug. The use of the condensed bleeding score in the detection of congenital bleeding disorders other than type 1 VWD requires further validation in a larger number of patients. Disclosures: No relevant conflicts of interest to declare.

Successful perioperative management of factor XI deficiency with administration of fresh-frozen plasma in a subdural hematoma patient

2016 ◽  
Vol 16 (1) ◽  
pp. 143-144
Author(s):  
Yoko Edahiro ◽  
Kunimoto Ichikawa ◽  
Hiroharu Suzuki ◽  
Hajime Yasuda ◽  
Michiaki Koike ◽  
...  
Keyword(s):  
Subdural Hematoma ◽  
Perioperative Management ◽  
Fresh Frozen Plasma ◽  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Fresh Frozen ◽  
Frozen Plasma

Clinical experience of factor XI deficiency: the role of fresh frozen plasma and factor XI concentrate

Haemophilia ◽  
1995 ◽  
Vol 1 (4) ◽  
pp. 227-231 ◽  
Author(s):  
P.W. COLLINS ◽  
E. GOLDMAN ◽  
P. LILLEY ◽  
K. J. PASI ◽  
C. A. LEE
Keyword(s):  
Clinical Experience ◽  
Fresh Frozen Plasma ◽  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Fresh Frozen ◽  
Frozen Plasma

scholarly journals Peri- and Postpartum Management of Patients With Factor XI Deficiency

2019 ◽  
Vol 25 ◽  
pp. 107602961988026 ◽  
Author(s):  
Gloria F. Gerber ◽  
Kelsey A. Klute ◽  
John Chapin ◽  
James Bussel ◽  
Maria T. DeSancho
Keyword(s):  
Pregnant Women ◽  
Medical Center ◽  
Academic Medical Center ◽  
Fresh Frozen Plasma ◽  
Neuraxial Anesthesia ◽  
Factor Xi ◽  
Antifibrinolytic Agents ◽  
Factor Xi Deficiency ◽  
Severe Deficiency ◽  
Fresh Frozen

Factor XI (FXI) deficiency is an uncommon autosomal disorder with variable bleeding phenotype, making peripartum management challenging. We describe our experience in pregnant women with FXI deficiency and identify strategies to minimize the use of hemostatic agents and increase utilization of neuraxial anesthesia. Electronic records of 28 pregnant women with FXI deficiency seen by a hematology service in an academic medical center from January 2006 to August 2018 were reviewed. Data on bleeding, obstetric history, peripartum management, and FXI activity were collected. Partial FXI deficiency was defined as >20 IU/dL and severe <20 IU/dL. Median FXI activity was 42 IU/dL (range <1-73 IU/dL), and median activated partial thromboplastin time was 32.2 seconds (range: 27.8-75 seconds). There were 64 pregnancies: 53 (83%) live births and 11 (17%) pregnancy losses. Postpartum hemorrhage occurred in 9 (17%) pregnancies. Antifibrinolytic agents and fresh frozen plasma were used only in women with severe deficiency (42% with bleeding and 17% with no bleeding phenotype, respectively). Neuraxial anesthesia was successfully administered in 32 (59%) deliveries. Most women with FXI deficiency have uncomplicated pregnancies and deliveries with minimal hemostatic support. Neuraxial anesthesia can be safely administered in most women.

scholarly journals Acquired Factor XI Deficiency with Lupus Anticoagulant in a Pregnant Woman Diagnosed by the Eruptions and Pain in Fingers

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Rie Nakajima ◽  
Atsuko Togo ◽  
Yasuhira Kanno ◽  
Masaru Hayashi ◽  
Kanako Mitsuzuka ◽  
...  
Keyword(s):  
Lupus Anticoagulant ◽  
Fresh Frozen Plasma ◽  
Factor Xi ◽  
Excessive Bleeding ◽  
Factor Xi Deficiency ◽  
Glycoprotein I ◽  
Starting Point ◽  
Tertiary Center ◽  
Fresh Frozen ◽  
Dermatoses Of Pregnancy

We report a case of acquired factor XI deficiency with lupus anticoagulant (LA) in a 28-year-old primigravida who presented with finger pain and eruptions on her palms and fingers during the 3rd trimester of pregnancy. The patient complained of pain and reddening of the fingers at 30 weeks of gestation. She was referred to our tertiary center with a diagnosis of preeclampsia and suspected collagen disease at 35 weeks of gestation. Erythema was seen on the fingers and palms, and she presented with pain and cryesthesia on the fingers. Laboratory investigations revealed an activated partial thromboplastin time of 51 s (normal, 23–40 s), although it was normal during the 30th and 34th gestational weeks, LA with an anticardiolipin-beta2-glycoprotein I complex antibody, and low level of clotting XI activity (25 U/mL). On week 37 day 0 of gestation, the patient presented with severe hypertension. An urgent Cesarean section was performed after transfusion of two units of fresh frozen plasma. There was no excessive bleeding during the surgery or the postpartum period. The symptoms on her fingers and palms gradually improved after surgery. Our case indicates that dermatoses of pregnancy may become a starting point for the diagnosis of autoimmune diseases and coagulation abnormalities. When a patient presents with an atypical symptom, as in our case, the possibility of various diseases should be considered.

Production and Therapeutic Use of a Factor XI Concentrate from Plasma

1992 ◽  
Vol 67 (03) ◽  
pp. 314-319 ◽  
Author(s):  
P H B Bolton-Maggs ◽  
R T Wensley ◽  
P B A Kernoff ◽  
C K Kasper ◽  
L Winkelman ◽  
...  
Keyword(s):  
Parvovirus B19 ◽  
Virus Transmission ◽  
Liver Function Tests ◽  
Fresh Frozen Plasma ◽  
Bleeding Tendency ◽  
Factor Xi ◽  
Excessive Bleeding ◽  
Human Donor ◽  
Factor Xi Deficiency ◽  
The Mean

SummaryFactor XI deficiency is an uncommon bleeding disorder usually manifested by excessive bleeding after surgery or trauma. Until recently the only effective therapy has been fresh-frozen plasma (FFP) infusion. We describe the efficacy and safety of a new factor XI concentrate produced from human donor plasma by a modification of the method used for antithrombin III concentrate. The mean recovery of factor XI in the circulation measured on 62 occasions was approximately 91% of the injected dose, and the mean half-disappearance-time was 52 h. The concentrate was used for 31 invasive procedures in 30 patients, including 16 patients who had a definite bleeding tendency on previous occasions, with normal haemostasis being achieved in all but 1. Only 1 patient (previously experiencing allergy to FFP) experienced adverse effects during infusion. Monitoring of liver function tests and viral antibody status in suitable patients has shown no evidence of transmission of hepatitis viruses, HIV-1 or parvovirus B19.We conclude that this concentrate provides effective treatment for patients with factor XI deficiency. Preliminary results suggest safety from virus transmission, but this needs to be established in further studies of previously untreated patients.

scholarly journals Rosenthal's Disease (Hemophilia C or factor XI Deficiency) Revealed by Chronic Epistaxis: The First Observation in Sub-Saharan Africa.

2019 ◽  
Vol 3 (2) ◽  
pp. 14-17
Author(s):  
Essohana PADARO ◽  
Irenée MD KUEVIAKOE ◽  
Yao LAYIBO ◽  
Kossi AGBETIAFA ◽  
Hèzouwè MAGNANG ◽  
...  
Keyword(s):  
Coagulation Factors ◽  
Fresh Frozen Plasma ◽  
Sub Saharan Africa ◽  
Factor Xi ◽  
Systematic Analysis ◽  
Factor Xi Deficiency ◽  
Prolonged Aptt ◽  
Fresh Frozen ◽  
Sub Saharan ◽  
The Right

Objective Rosenthal's disease (RD) is a rare constitutional hemorrhagic disorder defined by factor XI deficiency. It is clinically characterized by the presence of minimal haemorrhage. We report the first observation of RD in Togo. Observation Mrs. G. A., 45 years old with no particular pathological antecedents, was referred for anemia in a context of chronic epistaxis. It was a spontaneous anterior exteriorization epistaxis often of great abundance, rocking and which evolved episodically. The patient received several transfusions for anemia. The ear-nose-throat examination was normal and a sinus CT scan found only an inflammatory process of the right maxillary sinus. The blood count showed microcytic severe anemia (2,2g/dl). Hemostasis tests showed a prolonged aPTT (57,9 seconds). Clinical examination documented an anemic syndrome with dry skin. Iron deficiency was found. The hemostasis balance confirmed aPTT elongation. Coagulation factors activity showed normal VIII and IX level, but moderate decrease of factor XI (32%). The family survey was not possible (orphan patient). It is recommended the setting under fresh frozen plasma (FFP) in case of a new episode. Follow-up is in progress. Conclusion In the event of any hemorrhagic syndrome, the isolated elongation of the aPTT must lead to a systematic analysis of intrinsic pathway factors

Factor XI deficiency and aortic valve replacement: Perioperative management

2016 ◽  
Vol 25 (6) ◽  
pp. 450-452 ◽  
Author(s):  
Aristea Petroulaki ◽  
George Lazopoulos ◽  
Fotini Chaniotaki ◽  
Emmanuel Kampitakis ◽  
Dionysis Pavlopoulos ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Fresh Frozen Plasma ◽  
Coagulation Disorder ◽  
Factor Xi ◽  
Intraoperative Bleeding ◽  
Factor Xi Deficiency ◽  
Fresh Frozen ◽  
Thrombotic Complications

Severe factor XI deficiency (hemophilia C) is a rare coagulation disorder. A 73-year-old woman, a homozygote for factor XI deficiency, required aortic valve replacement. An initial dose of 15 U kg−1 of factor XI concentrate was administered preoperatively and on postoperative day 3. During surgery, concentrated red cells, fresh frozen plasma, platelets, tranexamic acid, and fibrinogen were transfused. Intraoperative bleeding and total chest drainage were minimal. Postoperatively, there was no need for further transfusions and no bleeding or thrombotic complications occurred. The patient was well 16 months after surgery.

scholarly journals A Rare Cause of Isolated Prolonged Activated Partial Thromboplastin Time: An Overview of Prekallikrein Deficiency and the Contact System

2021 ◽  
Vol 9 ◽  
pp. 232470962110121
Author(s):  
Ivy Riano ◽  
Klaorat Prasongdee
Keyword(s):  
Partial Thromboplastin Time ◽  
Normal Activity ◽  
Fresh Frozen Plasma ◽  
Activated Partial Thromboplastin Time ◽  
Recessive Trait ◽  
Bleeding Tendency ◽  
Contact Activation ◽  
Asymptomatic Patients ◽  
Prolonged Aptt ◽  
Fresh Frozen

Prekallikrein (PK) deficiency, also known as Fletcher factor deficiency, is a very rare disorder inherited as an autosomal recessive trait. It is usually identified incidentally in asymptomatic patients with a prolonged activated partial thromboplastin time (aPTT). In this article, we present the case of a 52-year-old woman, with no prior personal or family history of thrombotic or hemorrhagic disorders, who was noted to have substantial protracted aPTT through the routine coagulation assessment before a kidney biopsy. The patient had an uneventful biopsy course after receiving fresh frozen plasma (FFP). Laboratory investigations performed before the biopsy indicated normal activity for factors VIII, IX, XI, XII, and von Willebrand factor (vWF) as well as negative lupus anticoagulant (LA) screen. The plasma PK assay revealed low activity at 15% consistent with mild PK deficiency. The deficit of PK is characterized by a severely prolonged aPTT and normal prothrombin time (PT) in the absence of bleeding tendency. PK plays a role in the contact-activated coagulation pathway and the inflammatory response. Thus, other differential diagnoses of isolated prolonged aPTT include intrinsic pathway factor deficiencies and nonspecific inhibitors such as LA. We concluded that the initial evaluation of a prolonged aPTT with normal PT should appraise the measurement of contact activation factors and factor inhibitors. PK deficiency should be considered in asymptomatic patients with isolated aPTT prolongation, which corrects on incubation, with normal levels of the contact activation factors and factor inhibitors.

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