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 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.

Comparison of the coagulation potential of lyophilized blood plasma virus-inactivated by two different methods.

2021 ◽  
Author(s):  
И.А. Кривов ◽  
А.А. Рагимов ◽  
Э.Л. Салимов
Keyword(s):  
Comparative Evaluation ◽  
Room Temperature ◽  
Coagulation Factors ◽  
Fresh Frozen Plasma ◽  
Activated Partial Thromboplastin Time ◽  
Plasma Virus ◽  
Coagulation Parameters ◽  
Normal Limits ◽  
Fresh Frozen ◽  
Frozen Plasma

Введение. Свежезамороженная плазма (СЗП) — один из самых распространённых компонентов крови, применяемых сегодня в клиниках при оказании медицинской помощи при кровотечениях и тяжёлых коагулопатиях. В отличие от вирусинактивированной замороженной плазмы, сублимированная (лиофилизированная) плазма может храниться при комнатной температуре, и восстановление перед переливанием обычно требует меньших временных затрат. Цель исследования: оценить коагуляционный потенциал лиофилизированной плазмы, полученной из вирусинактивированной плазмы, инактивированной 2 способами: с использованием метиленового синего + видимый свет и рибофлавина + ультрафиолетовое облучение спектра B. Материалы и методы. Проведен анализ 100 образцов иофилизированной плазмы, вирусинактивированной двумя методами. Изучали влияние лиофилизации на уровень факторов свертывания и показатели свертываемости в вирусинактивированной плазме. Для сравнительной оценки в качестве контроля были проанализированы 150 образцов СЗП. Результаты. При использовании обоих технологий инактивации в лиофилизированной вирусинактивированной плазме установлено снижение содержания факторов V и VIII как по отношению к СПЗ, так и по отношению к физиологической норме. Лиофилизация вирусинактивированной плазмы различными методами привела к некоторому увеличению показателей свёртывания крови — протромбинового времени и активированного частичного тромбопластинового времени. Остальные показатели оставались в нормальных пределах. Существенных различий в показателях между образцами плазмы, инактивированной различными методами, выявлено не было. Заключение. По клиническим свойствам вирусинактивированная лиофилизированная плазма может служить альтернативой СЗП, однако для уточнения всесторонних аспектов её применения необходимы дополнительные исследования. Introduction. Fresh frozen plasma (FFP) is one of the most common blood components used today in clinics for medical care of bleeding and severe coagulopathies. Unlike virus-inactivated frozen plasma, sublimated (lyophilized) plasma can be stored at room temperature, and recovery before transfusion usually requires less time. Objectives: to assess the coagulation potential of lyophilized plasma obtained from virus- inactivated plasma inactivated by 2 methods: using methylene blue + visible light and riboflavin + ultraviolet radiation of spectrum B. Materials/Methods. Analysis of 100 samples of lyophilized plasma, virus-inactivated by 2 methods, was carried out. The effect of lyophilization on the level of coagulation factors and coagulation parameters in virus-inactivated plasma was studied. For comparative evaluation, 150 samples of FFP were analyzed as a control. Results. Using both technologies for inactivation of lyophilized virus- inactivated plasma, a decrease in the content of V and VIII factors was found both in relation to the FFP and in relation to the physiological norm. Lyophilization of virus-inactivated plasma by various methods led to a slight increasing in blood coagulation parameters — prothrombin time and activated partial thromboplastin time. The rest of the parameters remained within normal limits. There were no significant differences in parameters between plasma samples inactivated by different methods. Conclusions. According clinical properties, virus- inactivated lyophilized plasma can serve as an alternative to FFP, but more studies are needed to clarify the comprehensive aspects of its use.

Prophylactic correction of the international normalized ratio in neurosurgery: a brief review of a brief literature

2011 ◽  
Vol 114 (1) ◽  
pp. 9-18 ◽  
Author(s):  
Kelly L. West ◽  
Cory Adamson ◽  
Maureane Hoffman
Keyword(s):  
Bleeding Risk ◽  
International Normalized Ratio ◽  
Fresh Frozen Plasma ◽  
Coagulation Cascade ◽  
Bleeding Tendency ◽  
Invasive Procedures ◽  
Clinical Tool ◽  
Clotting Factors ◽  
Fresh Frozen ◽  
Frozen Plasma

Prophylactic fresh-frozen plasma (FFP) transfusion is often undertaken in hemodynamically stable patients with a minimally elevated international normalized ratio (INR) prior to invasive procedures, despite little evidence in support of this practice. The authors review the current literature in an attempt to clarify best clinical practice with regard to this issue. Although the activated partial thromboplastin time and prothrombin time–INR are useful laboratory tests to measure specific clotting factors in the coagulation cascade, in the absence of active bleeding or a preexisting coagulopathy, their utility as predictors of overall bleeding risk is limited. Several studies have shown an imperfect correlation between mild elevations in the INR and subsequent bleeding tendency. Furthermore, FFP transfusion is not always sufficient to achieve normal INR values in patients who have mild elevations (< 2) to begin with. Finally, there are risks associated with FFP transfusion, including potential transfusion-associated [disease] exposures as well as the time delay imposed by laboratory testing and transfusion administration prior to initiation of procedures. The authors propose that the current concept of a “normal” INR value warrants redefinition to make it a more meaningful clinical tool. Based on their review of the literature, the authors suggest that in a hemodynamically stable patient population there is a range of mildly prolonged INR values for which FFP transfusion is not beneficial, and is potentially harmful.

Comparison Between a One-Point Dilute Phospholipid APTT and the Dilute Russell Viper Venom Time for Verification of Lupus Anticoagulants

1992 ◽  
Vol 67 (06) ◽  
pp. 672-678 ◽  
Author(s):  
Barbara M Alving ◽  
Charles F Barr ◽  
Lawrence E Johansen ◽  
Douglas B Tang
Keyword(s):  
Partial Thromboplastin Time ◽  
Antiphospholipid Antibodies ◽  
Test System ◽  
Bovine Brain ◽  
Activated Partial Thromboplastin Time ◽  
Clotting Time ◽  
Lupus Anticoagulants ◽  
Normal Plasma ◽  
Prolonged Aptt ◽  
Higher Sensitivity

SummaryIn the present study, the dilute Russell viper venom time (RVVT) and the dilute phospholipid activated partial thromboplastin time (PL-APTT), which are two assays used for the verification of lupus anticoagulants (LA), were modified to increase standardization. The modified assays were then compared with respect to sensitivity and specificity in detecting LA in plasmas from 72 patients with a prolonged APTT. Modifications included utilizing a single dilution of phospholipid that was either bovine brain thromboplastin (Thrombofax) or liposomes comprised of phosphatidylcholine/phosphatidylserine, and expressing the results as a ratio of the clotting times of the mixture of patient and normal plasma/clotting time of normal plasma. In the RWT, the correlation coefficient between assay results for liposomes and Thrombofax was 0.88 and in the PL-APTT, the correlation was 0.68. A positive test for LA was defined as a ratio of ≥1.3 for the PL-APTT with liposomes and ≥1.2 for the PL-APTT with Thrombofax and the RWT with Thrombofax or liposomes. Regardless of the phospholipid source in the test system, the PL-APTT demonstrated higher sensitivity and the RWT showed greater specificity in detecting patient plasmas that contained antiphospholipid antibodies.

The influence of acetaldehyde and glycosaminoglycans upon Factor Xa- and Factor X-deficient plasma

2002 ◽  
Vol 80 (9) ◽  
pp. 879-886 ◽  
Author(s):  
Arthur S Brecher ◽  
Eric L Hommema
Keyword(s):  
Blood Coagulation ◽  
Partial Thromboplastin Time ◽  
Factor Xa ◽  
Activated Partial Thromboplastin Time ◽  
Clotting Factor ◽  
Factor X ◽  
Chondroitin Sulfates ◽  
Prolonged Aptt ◽  
Subsequent Addition ◽  
Three Components

The comparative effects of glycosaminoglycans and acetaldehyde (AcH) – glycosaminoglycan (GAG) mixtures upon Factor Xa- (FXa) and Factor X-deficient plasma (FXDP) have been studied by activated partial thromboplastin time (APTT) studies. Heparin at 0.025, 0.030, 0.04, and 0.05 U statistically prolonged the APTT when pre-incubated with FXa at 37°C for 3 min prior to addition to FXDP and subsequent addition of Ca2+. Upon addition of 0.25, 0.375, and 0.5 µg heparin-6000 (H6k) to FXa, significant increases in APTT were observed. Similarly, profound increases in APTT were observed when 0.5, 0.75, and 1.0 µg heparin-3000 (H3k) was added to FXa. The chondroitin sulfates (CSA, CSB, CSC) had far less impact upon APTT with the FXa–FXDP system. In examining the effects of AcH–GAG mixtures upon the clotting factor, it was observed that 44.3 and 443 mM AcH synergistically prolonged the APTT in a statistically significant manner regardless of the order of premixing the three components. Hence, AcH may play a role in prolonging APTT in alcoholics. It synergistically prolonged APTT in concert with GAGs and FXa at the AcH levels used in this study. The effect of the GAGs upon FXDP is far less than its effect upon FXa.Key words: Factor Xa, acetaldehyde, heparin, glycosaminoglycans, blood coagulation.

Liver Cirrhosis Manifested by Dysfibrinogenemia

1997 ◽  
Vol 3 (2) ◽  
pp. 102-103 ◽  
Author(s):  
Takeshi Wajima
Keyword(s):  
Liver Cirrhosis ◽  
Gastrointestinal Bleeding ◽  
Bleeding Time ◽  
Degradation Products ◽  
Liver Function Tests ◽  
Fresh Frozen Plasma ◽  
Activated Partial Thromboplastin Time ◽  
Thrombin Time ◽  
Fresh Frozen ◽  
Frozen Plasma

We report a case in which liver cirrhosis was manifested by dysfibrinogenemia before the diagnosis of liver cirrhosis was made. A patient developed gastrointestinal bleeding and hepatic encephalopathy although liver function tests, prothrombin time (PT), activated partial thromboplastin time (aPTT), bleeding time, fibrin (ogen) degradation products (FDP), and platelet counts were normal. However, thrombin time (TT) was prolonged. The gastrointestinal bleeding (GI bleeding) was successfully treated with cryoprecipitate and fresh frozen plasma (FFP). Key Words: Dysfibrinogenemia—Liver disease—Thrombin time.

Spurious Prolongation of the Activated Partial Thromboplastin Time

1977 ◽  
Vol 38 (04) ◽  
pp. 0900-0908 ◽  
Author(s):  
Robert A. Okpara ◽  
Joann Carabello ◽  
H James Day
Keyword(s):  
Partial Thromboplastin Time ◽  
Laboratory Data ◽  
Activated Partial Thromboplastin Time ◽  
Rate Of Change ◽  
Maximal Rate ◽  
Middle Stage ◽  
Normal Plasma ◽  
Prolonged Aptt ◽  
Tissue Thromboplastin ◽  
Abnormal Tissue

SummaryThe clinical and laboratory data of 8 patients (4 males and 4 females) with circulating anticoagulant were presented. Based on prolonged APTT, failure to correct the APTT with 50 % normal plasma and abnormal tissue thromboplastin inhibition test, the inhibitor was identified as “middle stage” – or the “lupus anticoagulant”. Thrombokinetics showed the maximal rate of change in optical density (VmaxΔOD) of plasma, resulting from clot formation to be significantly less in the plasma of patients with the inhibitor than in normal plasma. This was not completely corrected by mixing the patients’ plasma with 50% normal plasma.

Evaluation Of Contact Activation Of The Intrinsic Coagulation System In Evacuated Tubes: Chromogenic Substrate Assay Equivalent To The Non-Activated Partial Thromboplastin Time

1981 ◽  
Author(s):  
D S Edlefson ◽  
P A Aiyappa
Keyword(s):  
Partial Thromboplastin Time ◽  
Glass Surface ◽  
Activated Partial Thromboplastin Time ◽  
Coagulation System ◽  
Blood Collection ◽  
Chromogenic Substrate ◽  
Contact Activation ◽  
Thrombin Activity ◽  
Evacuated Tubes ◽  
Blood Collection Tubes

Plasma was collected and stored in plastic tubes and commercially available blood collection tubes to study the effect of the type of surface on contact activation. To determine if activation had occurred, a sensitive chromogenic substrate assay was used. A non-activated partial thromboplastin reagent was employed with a synthetic tripeptide chromogenic substrate to measure the thrombin activity. The results indicated that the evacuated tubes showed substantial contact activation as evidenced by the increased levels of thrombin generated, compared to the plasma stored in plastic. Total siliconization of the glass surface eliminated contact activation. The chromogenic substrate assay equivalent to the non-activated Partial Thromboplastin Time offers a sensitive, easy and faster method for studying contact activation.

Factor XIII: Congenital Deficiency Factor XIII, Acquired Deficiency, Factor XIII A-Subunit, and Factor XIII B-Subunit

2014 ◽  
Vol 138 (2) ◽  
pp. 278-281 ◽  
Author(s):  
Anita Tahlan ◽  
Jasmina Ahluwalia
Keyword(s):  
Factor Xiii ◽  
Recurrent Miscarriage ◽  
Fresh Frozen Plasma ◽  
Screening Tests ◽  
Bleeding Tendency ◽  
Thrombin Time ◽  
Congenital Deficiency ◽  
Fresh Frozen ◽  
B Subunits ◽  
Fxiii Deficiency

Factor XIII (FXIII) is a transglutaminase consisting of 2 catalytic A subunits and 2 noncatalytic B subunits in plasma. The noncatalytic B subunits protect the catalytic A subunits from clearance. Congenital FXIII deficiency may manifest as a lifelong bleeding tendency, abnormal wound healing, and recurrent miscarriage. Acquired FXIII deficiency, with significant reductions in FXIII levels, has been reported in several medical conditions. The routine screening tests for coagulopathies—prothrombin time, activated partial thromboplastin time, and thrombin time—do not show abnormalities in cases of FXIII deficiency. A quantitative, functional, FXIII activity assay that detects all forms of FXIII deficiency should be used as a first-line screening test. Treatment consists of recombinant FXIII or FXIII concentrate. If these are unavailable, then fresh-frozen plasma and cryoprecipitates may be used. Factor XIII has a long half-life; therefore, the patients can lead near-normal lives with regular replacements. Patients with acquired FXIII deficiency with inhibitors need immunosuppressive therapy in addition to factor replacements.

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