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.

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

U.S. Factor XI Patients: Is There a Safer Treatment? Yes - Hemoleven.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4005-4005
Author(s):  
Louis M. Aledort ◽  
Jenny Goudemand
Keyword(s):  
Surgical Intervention ◽  
Infectious Agent ◽  
Fresh Frozen Plasma ◽  
Major Surgery ◽  
Transmission Factor ◽  
Factor Xi ◽  
Spontaneous Bleeding ◽  
Fresh Frozen ◽  
Frozen Plasma ◽  
The U.S

Abstract Despite donor screening and newer PCR testing, human-derived fresh frozen plasma (FFP) is not without the potential for viral transmission. Factor XI-deficient patients are plentiful in the U.S. (estimate ~1500–2000). Although there is discordance between phenotype and genotype, with substantially fewer “bleeders” than those with deficiency, and spontaneous bleeding is rare, surgical intervention frequently demands replacement therapy. The English F XI concentrate, virally inactivated, had thrombosis as a complication, and heparin was added, but is no longer available in the U.S. An LFB factor XI concentrate (Hemoleven), doubly viral-inactivated, with heparin and ATIII is available internationally, but not in the U.S. A review of 56 cases treated in France, Spain, Switzerland, Canada, and Israel reveal that for minor and major surgery it is both safe and effective. Between 1992 and 2004, data for the use of Hemoleven for either prophylaxis, minor and major surgery was collected. In all, hemostasis was excellent. In three, concentrate was used because of anaphylaxis with FFP. Three had laboratory evidence of DIC and, of these, one had a PE. In addition, these three cases received excessive dosage. Twelve patients had recovery and half-life studies for this concentrate. T1/2 was 45.5 ± 7.9h and recovery was 1.85 ± 0.38%/u/kg. These data are evidence that a safer and effective therapy for F XI patients exist outside of the U.S. We should not have to wait for a new infectious agent transmitted by FFP before we make it available in the U.S.. We should have learned something from HIV, when DDAVP was used everywhere, but not licensed here.

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 &lt;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

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.

Das Smith-Lemli-Opitz-Syndrom

2005 ◽  
Vol 5 (04) ◽  
pp. 178-182
Author(s):  
Wieland Kiess ◽  
Manuela Schulz ◽  
Sabine Liebermann ◽  
Roland Pfäffle ◽  
Peter Bührdel ◽  
...  
Keyword(s):  
Fresh Frozen Plasma ◽  
Therapeutische Möglichkeiten ◽  
Fresh Frozen ◽  
Frozen Plasma

ZusammenfassungDas Smith-Lemli-Opitz-Syndrom wird durch einen Defekt des letzten Schrittes der Cholesterolbiosynthese, den Mangel an 7-Dehydrocholesterolreduktase, verursacht. Die Akkumulation der Metaboliten 7-Dehydrocholesterol und 8-Dehydrocholesterol, die die wichtigsten biochemischen Marker für die Diagnose der Erkrankung darstellen, sowie der Mangel an Cholesterol können zu multiplen kongenitalen Anomalien führen. Die Ursache des Enzymmangels sind Mutationen innerhalb des DHCR7-Gens, welches auf Chromosom 11q13 lokalisiert ist. Therapeutische Möglichkeiten bestehen in der Gabe von Cholesterol und im Notfall Fresh Frozen Plasma (FFP); der therapeutische Nutzen von Statinen befindet sich zurzeit in der klinischen Erprobung.

The Recovery of Factor VIII from Fresh-Frozen, Indated and Outdated Human Plasma

1976 ◽  
Vol 36 (01) ◽  
pp. 071-077 ◽  
Author(s):  
Daniel E. Whitman ◽  
Mary Ellen Switzer ◽  
Patrick A. McKee
Keyword(s):  
Factor Viii ◽  
Polyacrylamide Gel Electrophoresis ◽  
Sodium Dodecyl ◽  
The United States ◽  
Fresh Frozen Plasma ◽  
Red Cross ◽  
Random Samples ◽  
Fresh Frozen ◽  
Factor Viii Concentrates ◽  
Frozen Plasma

SummaryThe availability of factor VIII concentrates is frequently a limitation in the management of classical hemophilia. Such concentrates are prepared from fresh or fresh-frozen plasma. A significant volume of plasma in the United States becomes “indated”, i. e., in contact with red blood cells for 24 hours at 4°, and is therefore not used to prepare factor VIII concentrates. To evaluate this possible resource, partially purified factor VIII was prepared from random samples of fresh-frozen, indated and outdated plasma. The yield of factor VIII protein and procoagulant activity from indated plasma was about the same as that from fresh-frozen plasma. The yield from outdated plasma was substantially less. After further purification, factor VIII from the three sources gave a single subunit band when reduced and analyzed by sodium dodecyl sulfate polyacrylamide gel electrophoresis. These results indicate that the approximately 287,000 liters of indated plasma processed annually by the American National Red Cross (ANRC) could be used to prepare factor VIII concentrates of good quality. This resource alone could quadruple the supply of factor VIII available for therapy.

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