scholarly journals Successful use of BPL Factor X concentrate in a child with severe factor X deficiency

2013 ◽  
Vol 1 (2) ◽  
pp. 8-10 ◽  
Author(s):  
Kate Khair ◽  
Poornima Kumar ◽  
Mary Mathias ◽  
Jemma Efford ◽  
Ri Liesner
Keyword(s):  
Central Venous Access ◽  
Coagulation Factor ◽  
Venous Access ◽  
Fresh Frozen Plasma ◽  
Factor X ◽  
Post Dose ◽  
Thrombotic Risk ◽  
Factor X Deficiency ◽  
Fresh Frozen ◽  
The Right

Abstract Introduction: Severe factor X deficiency is a rare serious bleeding disorder historically treated with fresh frozen plasma (FFP) and more recently with prothrombin complex concentrate (PCC) which contains activated factors II, VII, IX and X. The infusion volume of PCC is smaller than FFP, but there is a risk of thromboembolic complications given the presence of activated forms of vitamin K-dependent factor concentrates when treating an isolated coagulation factor deficiency. Methods: We describe the case of a nine-year-old girl of consanguineous origin with co-existent congenital merosin deficient muscular dystrophy and severe factor X deficiency treated with twice-weekly PCC prophylaxis via an indwelling central venous access device (CVAD). Infusion occlusion of her fifth CVAD occurred 24-months post-insertion; thrombus within the right subclavian and brachiocephalic veins was seen on radiological imaging. She started peripheral treatment with BPL Factor X concentrate as infusion volumes were smaller and given her immobility further thrombotic risk was predicted to be reduced. A sixth CVAD was inserted seven months later and BPL Factor X prophylaxis was continued. Results:BPL Factor X concentrate was effective in maintaining trough levels of 13IU/ml 72-hours post-dose, with no intercurrent bleeding episodes or further problems in terms of occlusion of her portacath. Further radiological screening has not been undertaken. Conclusion: BPL Factor X has been shown to be a safe and effective alternative to PCC for treatment of severe factor X deficiency in this case.

scholarly journals Inherited Moderate Factor X Deficiency Presenting as Cardiac Tamponade

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Tamer Othman ◽  
Ayman Abdelkarim ◽  
Karen Huynh ◽  
An Uche ◽  
Jennifer Lee
Keyword(s):  
Cardiac Tamponade ◽  
Clinical Manifestations ◽  
Missense Variant ◽  
Fresh Frozen Plasma ◽  
Factor X ◽  
Pericardial Window ◽  
Extensive Experience ◽  
Management Guidelines ◽  
Factor X Deficiency ◽  
Fresh Frozen

Factor X deficiency is a rare bleeding disorder that varies in the severity of its clinical manifestations. The symptoms of this disorder can occur at any age, although most severe cases appear in childhood. The rarity of this condition has not allowed for the establishment of evidence‐based management guidelines, and thus, individuals afflicted with factor X deficiency are treated based on limited literature and the opinions of clinicians with extensive experience. In this case report, we discuss a unique presentation of a 38-year-old male who was found to have cardiac tamponade as a result of his newly diagnosed inherited moderate factor X deficiency. This was discovered by obtaining a factor X activity assay and confirmed with genetic testing which demonstrated a missense variant on the factor X gene on chromosome 13. His management involved correction of his factor X deficiency with fresh frozen plasma, a pericardiocentesis, and placement of a pericardial window. He has been asymptomatic and without hemorrhagic episodes for the 10 months following his discharge.

Treatment of Amyloidosis Associated Factor X Deficiency

1976 ◽  
Vol 35 (02) ◽  
pp. 377-381 ◽  
Author(s):  
Joel A. Spero ◽  
Jessica H. Lewis ◽  
Ute Hasiba ◽  
Lawrence D. Ellis
Keyword(s):  
Fresh Frozen Plasma ◽  
Factor Ix ◽  
Bleeding Complications ◽  
Primary Amyloidosis ◽  
Factor X ◽  
Associated Factor ◽  
Factor X Deficiency ◽  
Fresh Frozen ◽  
Frozen Plasma

SummaryThis is the tenth patient in thirteen years to be reported with the findings of an isolated factor X deficiency associated with primary amyloidosis. A favorable response to factor IX concentrate was manifested by temporary clinical and laboratory correction of her diathesis. This mode of treatment, therefore, provides an approach to therapy for bleeding complications in this group of patients who have previously failed to respond to fresh frozen plasma.

A Rare Case of Congenital Factor X Mild Deficiency Presenting as Life-Threatening Hemorrhagic Shock in a Neonate

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S151-S151
Author(s):  
Rachelle Mendoza ◽  
Tahmineh Haidary ◽  
Steven Kang
Keyword(s):  
Bleeding Event ◽  
Coagulation Factor ◽  
Hemodynamic Instability ◽  
Fresh Frozen Plasma ◽  
Bleeding Disorder ◽  
Activity Level ◽  
Factor X ◽  
Factor X Deficiency ◽  
Life Threatening ◽  
Congenital Factor

Abstract Introduction Congenital factor X deficiency is one of rarest bleeding disorders, occurring in 1 out of 1 million births. Its rarity limits its consideration in newborns presenting with hemodynamic instability. It is autosomal recessive and seen frequently in the consanguineous population. Patients with factor X deficiency are classified into three groups, based on factor X activity level: severe (<1%), moderate (1%-4%), and mild (6%-10%). Severe deficiency presents with bleeding diathesis early in life. Methods A 3-day-old male newborn, delivered at term via spontaneous vaginal delivery, presented in a well-baby clinic for a routine bilirubin check. Family history was negative for bleeding disorder or consanguinity. Nurse noted persistent blood oozing at heel stick site and oral, nasal, and umbilical stump bleeding. The patient immediately developed respiratory distress and shock. Sepsis, vitamin K deficiency, and congenital metabolic syndrome were considered. Liver function, WBC count, and other chemistry were normal, and cultures were negative. Hemoglobin was low and platelet count was elevated. PT (30.3 seconds) and aPTT (53.3 seconds) were both prolonged. Mixing patient’s sample with normal plasma corrected PT (13.1 seconds) and aPTT (25.7 seconds), indicating a factor deficiency. Results Coagulation factor assays revealed normal levels of factors VII, VIII, IX, and II. Factor X activity (12.9%) was low. The patient’s condition improved after multiple pRBC and plasma transfusions. He was placed on a daily 25-mL/kg dose of fresh-frozen plasma, which maintained his PT at 17.6 to 19.1 seconds and aPTT at 34.1 to 48.0 seconds. Factor X level increased to 20% after plasma transfusion. Conclusion Congenital bleeding disorder should be considered for neonates presenting with bleeding and shock. Factor X deficiency is suspected when both PT and aPTT are prolonged and corrected with mixing studies. Although factor levels of 10% to 40% are considered adequate for hemostasis, our patient with 12% factor X activity presented with a life-threatening bleeding event.

Immunological Studies on the Blood Coagulation Factor X and Its Warfarin-induced Precursor

1974 ◽  
Vol 31 (01) ◽  
pp. 040-051 ◽  
Author(s):  
Gustav Gaudernack ◽  
Åse Gladhaug Berre ◽  
Bjarne Østerud ◽  
Hans Prydz
Keyword(s):  
Coagulation Factor ◽  
Factor X ◽  
Intrinsic Pathway ◽  
Coagulation Activity ◽  
Factor X Deficiency ◽  
Warfarin Treatment ◽  
The Cross ◽  
Monospecific Antisera ◽  
Purified Antigen ◽  
Congenital Factor

SummaryMonospecific antisera against the human coagulation factor X have been raised in rabbits by injections of purified antigen. Such antiserum was used to study the cross-reacting material without factor X activity which is present in the blood of warfarin-treated patients and animals as well as to study the changes in factor X during coagulation. One patient with congenital factor X deficiency was also studied.A complete identity was found between factor X in Macaca mulatta and human blood. During warfarin treatment antigenically cross-reacting material appeared in plasma. This was not adsorbed on BaSO4, and inhibited the coagulation activity of normal factor X.Both this material, normal factor X and the cross-reacting material in plasma from a patient congenitally deficient in factor X gave rise to split products during coagulation by the intrinsic pathway, i. e. all of them served as substrates for the intrinsic activator of factor X.

Perioperative management of coagulation

2006 ◽  
Vol 26 (S 02) ◽  
pp. S3-S14 ◽  
Author(s):  
P. Innerhofer
Keyword(s):  
Coagulation Factor ◽  
Fresh Frozen Plasma ◽  
Laboratory Evaluation ◽  
Blood Component ◽  
Actual Case ◽  
Fresh Frozen ◽  
Coagulation Factor Concentrates ◽  
Hemostatic Therapy ◽  
Frozen Plasma

SummaryGuidelines of official societies for diagnosis and therapy of intraoperatively occurring hypocoagulability rely mainly on data of patients receiving whole blood transfusions. They recommend -provided that laboratory evaluation shows deficiency (values >1.5 fold normal)- administration of fresh frozen plasma, cryoprecipitate and platelet concentrates (platelet count <50 000 or <100 000/μl). This article describes the pathogenesis of coagulopathy in the light of the special intraoperative setting, emphasizes recent changes of blood component preparation, transfusion triggers, effects of volume therapy and challenges standard laboratory assays as reliable guide for intraoperative hemostatic therapy. The role of thrombelastographic monitoring is discussed as well as an alternative strategy to compensate deficiencies by the use of coagulation factor concentrates instead of or in addition to transfusion of FFP, a new concept which is illustrated by the presentation of an actual case report.

scholarly journals Solvent/detergent-treated plasma: a virus-inactivated substitute for fresh frozen plasma

Blood ◽  
1992 ◽  
Vol 79 (3) ◽  
pp. 826-831 ◽  
Author(s):  
B Horowitz ◽  
R Bonomo ◽  
AM Prince ◽  
SN Chin ◽  
B Brotman ◽  
...  
Keyword(s):  
Sindbis Virus ◽  
Animal Studies ◽  
Coagulation Factor ◽  
Fresh Frozen Plasma ◽  
Complete Inactivation ◽  
Virus Safety ◽  
Fresh Frozen ◽  
History Of ◽  
Triton X ◽  
Frozen Plasma

Abstract Fresh frozen plasma (FFP) is prepared in blood banks world-wide as a by- product of red blood cell concentrate preparation. Appropriate clinical use is for coagulation factor disorders where appropriate concentrates are unavailable and when multiple coagulation factor deficits occur such as in surgery. Viral safety depends on donor selection and screening; thus, there continues to be a small but defined risk of viral transmission comparable with that exhibited by whole blood. We have prepared a virus sterilized FFP (S/D-FFP) by treatment of FFP with 1% tri(n-butyl)phosphate (TNBP) and 1% Triton X-100 at 30 degrees C for 4 hours. Added reagents are removed by extraction with soybean oil and chromatography on insolubilized C18 resin. Treatment results in the rapid and complete inactivation of greater than or equal to 10(7.5) infectious doses (ID50) of vesicular stomatitis virus (VSV) and greater than or equal to 10(6.9) ID50 of sindbis virus (used as marker viruses), greater than or equal to 10(6.2) ID50 of human immunodeficiency virus (HIV), greater than or equal to 10(6) chimp infectious doses (CID50) of hepatitis B virus (HBV), and greater than or equal to 10(5) CID50 of hepatitis C virus (HCV). Immunization of rabbits with S/D-FFP and subsequent adsorption of elicited antibodies with untreated FFP confirmed the absence of neoimmungen formation. Coagulation factor content was comparable with that found in FFP. Based on these laboratory and animal studies, together with the extensive history of the successful use of S/D-treated coagulation factor concentrates, we conclude that replacement of FFP with S/D-FFP, prepared in a manufacturing facility, will result in improved virus safety and product uniformity with no loss of efficacy.

Study of coagulation factor activities in apheresed thawed fresh frozen plasma at 1–6°C for five days

2006 ◽  
Vol 21 (4) ◽  
pp. 224-226 ◽  
Author(s):  
Rameshwar S. Sidhu ◽  
Tuan Le ◽  
Brad Brimhall ◽  
Hannis Thompson
Keyword(s):  
Coagulation Factor ◽  
Fresh Frozen Plasma ◽  
Fresh Frozen ◽  
Frozen Plasma
Sign in / Sign up

Export Citation Format

Share Document