scholarly journals Case Report on Management of Combined Factor V and factor VIII Deficiency during Pregnancy

2021 ◽  
pp. 1-2
Author(s):  
Dr. Kirti Solanki ◽  
Dr. Swati Kochar ◽  
Dr. Shweta Choudhary ◽  
Dr. Priyanka Gaur ◽  
Dr. Krishna Krishna
Keyword(s):  
Factor Viii ◽  
Post Partum ◽  
Autosomal Recessive Disorder ◽  
Fresh Frozen Plasma ◽  
Factor V ◽  
Bleeding Tendency ◽  
Factor Viii Deficiency ◽  
Risk Of Bleeding ◽  
Increased Risk ◽  
Fresh Frozen

Combined factor V and factor VIII deficiency (CF5F8D) is a rare autosomal recessive disorder associated with mild to moderate risk of bleeding tendency. These patients have an increased risk of bleeding after surgical procedures. Pregnant women are at increased risk of having a miscarriage, placental abruption, or post partum hemorrhage. Management of these patients requires the replacement of deficient factors. We are reporting a case of management of a 31-year old second gravida female with combined factor V and factor VIII deficiency, who was transfused with fresh frozen plasma before and during labor to prevent bleeding episodes.

scholarly journals INFLUENCE OF ABO BLOOD GROUP ON FACTOR VIII AND FACTOR V LEVELS IN FRESH FROZEN PLASMA

2020 ◽  
Vol 42 ◽  
pp. 404-405
Author(s):  
C.M. Wink ◽  
J.S. Palaoro ◽  
D. Glimm ◽  
A.A.C. Araujo ◽  
C.S.R. Araujo
Keyword(s):  
Factor Viii ◽  
Blood Group ◽  
Fresh Frozen Plasma ◽  
Abo Blood Group ◽  
Factor V ◽  
Fresh Frozen ◽  
Frozen Plasma

scholarly journals Clinical guidelines for cryosupernatant transfusions

2020 ◽  
Vol 65 (3) ◽  
pp. 351-359
Author(s):  
G. M. Galstyan ◽  
T. V. Gaponova ◽  
F. S. Sherstnev ◽  
A. A. Kupryashov ◽  
N. I. Olovnikova ◽  
...  
Keyword(s):  
Factor Viii ◽  
Antithrombin Iii ◽  
Fresh Frozen Plasma ◽  
Blood Component ◽  
Factor Viii Inhibitor ◽  
Factor V ◽  
Fresh Frozen ◽  
Viii Inhibitor ◽  
Von Willebrand ◽  
Frozen Plasma

Introduction. Cryosupernatant is blood component. Cryosupernatant is the supernatant plasma removed during the preparation of cryoprecipitate. Aim. To provide information on the composition and methods of production, storage, transportation and clinical use of Cryosupernatant. General fi ndings. In comparison with fresh frozen plasma (FFP) and cryoprecipitate, Cryosupernatant plasma is depleted in factor VIII, fi brinogen factor von Willebrand (VWF). Cryosupernatant is defi cient in high molecular weight multimers of VWF, but contains VWF metalloproteinase. The concentrations of factor V, antithrombin III, albumin and immunoglobulins are the same as in FFP and cryoprecipitate. The indications for Cryosupernatant transfusions are massive blood loss in patients with factor VIII inhibitor, plasma exchange in patients with thrombotic thrombocytopenic purpura. For children the doses of Cryosupernatant should be 10-15 mL/kg.

scholarly journals Successful Pregnancy in a Patient with Combined Deficiency of Factor V and Factor VIII

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Ahmed Ghassan El Adib ◽  
Farah Majdi ◽  
Mohamed Othmane Dilai ◽  
Hamid Asmouki ◽  
Ahlam Bassir ◽  
...  
Keyword(s):  
Factor Viii ◽  
Fetal Monitoring ◽  
Fresh Frozen Plasma ◽  
Factor V ◽  
Successful Pregnancy ◽  
Fresh Frozen ◽  
Factor Viii Concentrates ◽  
Low Levels ◽  
Frozen Plasma ◽  
Combined Deficiency

Inherited combined factor V and factor VIII deficiency (F5F8D) is autosomal recessive transmission disorder. Epistaxis, postsurgical bleeding, and menorrhagia are the most common symptoms. The risk of miscarriage and placental abruption is consequent. We report a case of successful pregnancy in a patient with F5F8D. 20-year-old woman, born of consanguineous parents, third gestate, first parity, two miscarriages, admitted for child birth of a spontaneous pregnancy estimated at 38 weeks and was diagnosed with F5F8D. At admission, patient was hemodynamically stable, with good obstetric conditions. The biologic results showed low levels of PT (52%), factor V (7%), and factor VIII (5%), and the activated partial thromboplastin time was prolonged (68,6%). Parturient was admitted in intensive care unit, maternal and fetal monitoring was performed. Fresh frozen plasma (FFP) and factor VIII concentrates were perfused at the induction of labor. Analgesia used fentanyl titration. The delivery gave birth to a newborn male, with Apgar 10/10 and 3000 g. The puerperium was simple without any important bleeding. Laboratory tests for the newborn were acceptable. Little literature is available on this subject and there are no guidelines available concerning pregnancy; we chose to prescribe a combination of factor VIII concentrate and FFP in pre-, per- and postpartum. The same protocol was successfully used in a patient before dental extraction and prostatectomy. Vaginal delivery is possible, as our case. Management by multidisciplinary team is recommended.

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.

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.

scholarly journals The long and short of it: using the new factor products

Hematology ◽  
2015 ◽  
Vol 2015 (1) ◽  
pp. 26-32 ◽  
Author(s):  
Amy Dunn
Keyword(s):  
Half Life ◽  
Fresh Frozen Plasma ◽  
Recurrent Bleeding ◽  
Prophylactic Therapy ◽  
Improved Outcomes ◽  
Increased Risk ◽  
Plasma Factor ◽  
Fresh Frozen ◽  
Bleeding Episodes ◽  
Viral Attenuation

Abstract Hemophilia A (HA) and B (HB) are classified as mild (>5%-40%) moderate (1%-5%) and severe (<1%) disease based on plasma factor activity. Severity of bleeding is commensurate with baseline factor levels in general; however, heterogeneity of bleeding in patients is well described. Recurrent bleeding with painful and disabling musculoskeletal complications is the largest source of morbidity for persons with hemophilia (PWH) but treatment advances through the years has led to improved outcomes. In the early 20th century, only whole blood and fresh frozen plasma (FFP) was available to treat bleeding episodes. In 1959, cryoprecipitate was discovered and became an option for treatment of HA in 1965. In the 1970s plasma fractionation led to the first standard half-life (SHL) concentrates. These products ushered in the use prophylactic therapy to prevent bleeding episodes. However, viral contamination slowed the use of prophylaxis until the 1980s when viral attenuation steps increased the safety of plasma concentrates. In the 1990s recombinant concentrates were developed and prophylactic therapy is increasing widely yet not yet universally used. However even with frequent SHL concentrate infusions outcomes are not optimal as PWH spend the majority of time with factor levels below the normal range and are at increased risk for bleeding. In 2014, the first extended half-life (EHL) products were approved for use and have begun to change the landscape of hemophilia care. Challenges of EHL implementation include patient selection, product selection, dose and schedule of infusions, monitoring for safety, efficacy and outcomes, and managing economic aspects of care.

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