scholarly journals Extensive catastrophic thromboses from elevation of factor VIII

2020 ◽  
Vol 10 (3) ◽  
Author(s):  
Jacqueline Kropf ◽  
Sarah Cheyney ◽  
Josselin Vachon ◽  
Philip Flaherty ◽  
Mai Vo ◽  
...  
Keyword(s):  
Factor Viii ◽  
Cause Of Death ◽  
Rapid Onset ◽  
High Plasma ◽  
Clinical Syndrome ◽  
Renal Infarction ◽  
Factor Viii Level ◽  
Progressive Dyspnea ◽  
Viii Level ◽  
St Elevation

Catastrophic thrombotic syndrome, otherwise known as thrombotic storm (TS) is an extreme prothrombotic clinical syndrome that presents as rapid onset of multiple thromboembolic events affecting a large variety of vasculature. In recent studies, there has been a correlation of high plasma levels of factor VIII with thrombotic events. We present the case of a young man who exhibited multi-organ failure due to thrombotic storm. A 38-year-old male presented to the emergency department for progressive dyspnea and was diagnosed to have pulmonary embolism. The patient developed respiratory distress requiring intubation and was diagnosed with both an ST-elevation myocardial infarction and right cerebral infarction during the hospital course. The patient expired and autopsy revealed the cause of death to be myocardial, cerebral and renal infarction from widespread vascular thrombosis. Autopsy revealed cause of death to be elevated factor VIII associated thrombotic coagulopathy. Factor VIII level upon autopsy was 375% (55-200%). Although TS is rare, it can be lifethreatening if not recognized early. Survival depends on the prompt initiation and duration of anticoagulation.

scholarly journals Further Experience on the Effect of High Pre-Donation Factor VIII Levels in Blood Donors on the Factor VIII Content of Small-Pool Fractions

1977 ◽  
Author(s):  
H. Beeser ◽  
H. Eqli
Keyword(s):  
Factor Viii ◽  
High Plasma ◽  
Activity Levels ◽  
Factor Viii Activity ◽  
Factor Viii Level ◽  
Plasma Factor ◽  
Viii Level ◽  
Factor Viii Concentrates ◽  
Small Pool ◽  
Concentrate Preparation

Because of the well known wide normal range of the factor VIII activity between 60 to 170% I man, selecting of donors with high activity levels would be of advantaae for the preparation of factor VIII concentrates. This is especially true for preparing small-pool fractions, as for technical reasons the final product cannot be controlled for its factor VIII content. In preliminary investigations, we reported on elsewhere, high factor VIII activity in donors estimated before a donation had been rarely reproducible before a second donation after 8-12 weeks. So as a preliminary result of finding a donor’s factor VIII level varying from donation to donation selecting of plasmas with high factor VIII content for concentrate preparation could only be establishedby re-estimating the activity before each donation. Proceeding in this way would be much too troublesome. To get more reliable information whether a healthy subject’s high factor VIII plasma level is distinctly varying or rather constant we assayed the plasma of 200 donors with factor VIII activity > 120% two times more before donation. The results confirmed our preliminary findings, especially the fact that a high plasma factor VIII activity in experienced donors was rarely reproducible when re-estimated before a second and third donation. As a consequence selecting of donors with high factor VIII procoaqulant activity for preparing small-pool factor VIII concentrates is impracticable.

A case of renal infarction associated with elevated factor VIII level

2011 ◽  
Vol 76 (09) ◽  
pp. 250-254 ◽  
Author(s):  
O. Asif Siddiqui ◽  
A. Al-Absi ◽  
A. Showkat
Keyword(s):  
Factor Viii ◽  
Renal Infarction ◽  
Factor Viii Level ◽  
Viii Level

Laboratory Detection of Female Carriers of Canine Hemophilia

1964 ◽  
Vol 12 (02) ◽  
pp. 368-376 ◽  
Author(s):  
B. J Parks ◽  
K. M Brinkhous ◽  
P. F Harris ◽  
G. D Penick
Keyword(s):  
Factor Viii ◽  
Partial Thromboplastin Time ◽  
Screening Test ◽  
Factor Viii Level ◽  
Viii Level ◽  
Antihemophilic Factor ◽  
Female Carriers

SummaryFemales known to be heterozygous for canine hemophilia had a plasma antihemophilic factor (AHF, factor VIII) level of about 50%, as determined by bioassay and by the effectiveness of their transfused plasma in raising the AHF levels of hemophilic dogs. Determination of the plasma AHF should serve to identify transmitter females prior to appearance of affected progeny in litters. Lyon’s hypothesis appears to apply to our findings.The simple partial thromboplastin time (PTT) test was prolonged in heterozygous females. Modifications of the test, by the addition of thrombin, a serum accelerator preparation, or kaolin, gave consistently longer PTT values for heterozygotes than for normal dogs. The PTT appears useful as a screening test for carriers of canine hemophilia.

Factor-VIII Inhibitor in Less Severely Affected Haemophilic Patients

1975 ◽  
Author(s):  
E. G. D. Tuddenham ◽  
A. L. Bloom ◽  
J. C. Giddings ◽  
C. A. Barrett
Keyword(s):  
Factor Viii ◽  
Factor Viii Inhibitor ◽  
Factor Viii Level ◽  
Replacement Treatment ◽  
Viii Level ◽  
Viii Inhibitor ◽  
In Vivo Recovery ◽  
Better Than

The occurrence of factor VIII inhibitor in five mild or moderately affected liaemophilic patients is described. In four patients the inhibitor inactivated endogenous factor VIII an dtemporarily converted them to severely affected haemophiliacs with factor VIII level of 0%. In the fifth patient, a brother of one of the others, the inhibitor although more potent did not inactivate the patient’s own factor VIII and did not completely inactivate normal factor VIII in vitro. This patient responded to treatment with factor-VIII concentrate but the in-vivo recovery was reduced. The patient’s plasma was tested against a panel of normal donors but it inactivated factor VIII in each to a similar extent and no evidence for normal factor-VIII groups was obtained. In the other patients the response to replacement treatment was also better than that usually seen in severely affected haemophilic patients with inhibitor. In the two related patients the inhibitors have so far persisted but in the unrelated patients the inhibitors eventually disappeared and did not always recur with subsequent therapy. The incidence of factor- VIII inhibitor in less severe haemophiliacs (factor VIII > 3% ) in this centre is 6% suggesting that the complication is more frequent in this type of patient than hitherto recognised.

Hematogenous thrombophilia as an additional risk factor for cerebral circulation disorders

2021 ◽  
Author(s):  
И.В. Куртов ◽  
С.П. Кривова ◽  
Р.К. Хайретдинов ◽  
И.Л. Давыдкин
Keyword(s):  
Risk Factor ◽  
Factor Viii ◽  
Blood Coagulation ◽  
Genetic Polymorphisms ◽  
Additional Risk Factor ◽  
Factor Viii Level ◽  
Aged Patients ◽  
Additional Risk ◽  
Viii Level ◽  
The Impact

Введение. Гематогенная тромбофилия служит дополнительным фактором риска нарушений мозгового кровообращения (НМК). Цель исследования: изучить влияние повышенного уровня фактора VIII и нарушений в системе фибринолиза на НМК у пациентов с сочетанной патологией системы свертывания крови. Материалы и методы. Обследовано 20 пациентов с перенесенными ишемическими инсультами в возрасте от 36 до 56 лет. Определяли показатели плазменного, тромбоцитарного звеньев гемостаза, системы фибринолиза, а также генетические полиморфизмы системы гемостаза. Результаты. У всех пациентов выявлены различные сочетания генетических полиморфизмов, связанных с изменениями антикоагулянтного звена гемостаза, а также дефекты системы фибринолиза и коагуляционного звена гемостаза, что является дополнительным фактором риска НМК. Заключение. У пациентов молодого и среднего возраста, перенесших острое НМК по ишемическому типу, необходимо исследование системы свертывания крови, включающее определение содержания фактора VIII в крови, показателей системы фибринолиза и уровня гомоцистеина. Background. Hematogenous thrombophilia is an additional risk factor for cerebral circulatory disorders (ССD). Objectives: to study the impact of increased factor VIII level and disturbances of fibrinolysis system on ССD in patients with combined pathology of blood coagulation. Patients/Methods. We examined 20 patients with ischemic strokes aged from 36 to 56 years. The parameters of plasma hemostasis, platelet function, fibrinolysis system, as well as the genetic polymorphisms of hemostasis system were determined. Results. All patients had different combinations of genetic polymorphisms associated with anticoagulant hemostasis changes, as well as defects in fibrinolysis system and coagulation hemostasis that is an additional risk factor for ischemic stroke. Conclusions. In young and middle-aged patients with history of acute ischemic ССD, it is necessary to study blood coagulation, including the determination of blood factor VIII level, fibrinolysis system parameters and homocysteine content.

scholarly journals Clinical Severity, Personality Characteristics and Maternal Attitudes in Hemophiliac Children

1977 ◽  
Author(s):  
L.L. Ackerman ◽  
J. Verrecchio ◽  
D.S. Charney ◽  
E.M. Strickler
Keyword(s):  
Factor Viii ◽  
Personality Characteristics ◽  
Clinical Severity ◽  
Personality Questionnaire ◽  
Factor Viii Level ◽  
Future Studies ◽  
Maternal Attitudes ◽  
Child Relationship ◽  
Viii Level ◽  
Mother Child Relationship

The interrelationships of severity of hemophilia, personality characteristics, and maternal attitudes were evaluated in 21 hemophiliac boys and 17 of their mothers. The 8 to 11 year old boys were given the Children’s Personality Questionnaire and those between 12 and 18, the Junior-Senior High School Personality Questionnaire. The mothers were administered the Mother-Child Relationship Evaluation which measured maternal attitudes of four scales; Acceptance (A), Overprotection (OP), Overindulgence (OI), and Rejection (R).Results were analyzed by comparing children with Factor VIII levels >1% (N=10) to those whose levels were <1% (N=ll) as determined on more than one occasion. There were no significant differences between the two groups in terms of personality characteristics or maternal attitudes.Results were also analyzed by comparing those children (N=6) who had accepting mothers (A> 60, OI < 30, OP < 30, R < 30) with those of non-accepting mothers (N=ll). The children of the non-accepting mothers (mean Factor VIII levels 3.0%) were significantly more sober (P<.02), and internally restrained (P <.01) and tended to be less intelligent and less emotionally stable than the children of accepting mothers (mean Factor VIII level 1.5%).These findings suggest that personality characteristics may be more closely related to maternal attitudes than severity of disease. Future studies should identify and focus on determinants of maternal attitudes.

Aberration of Coagulation Parameters with a Small Dose of Hydroxyethyl Starch in a Patient with Underlying Factor XII Deficiency: A Case Report.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4058-4058
Author(s):  
Dimitrios Scarvelis
Keyword(s):  
Case Report ◽  
Factor Viii ◽  
Small Dose ◽  
Factor Xii ◽  
Thrombin Time ◽  
Factor Viii Level ◽  
Coagulation Parameters ◽  
Factor Xii Deficiency ◽  
Underlying Factor ◽  
Viii Level

Abstract Background: Hydroxyethyl starch (HES) is a commonly used intravascular volume expander in the peri-operative period. The use of such volume expanders has been demonstrated to cause significant abnormalities of laboratory tests of hemostasis by a number of mechanisms including a reduction of factor VIII activity. In Canada, Pentaspan® is commonly used, and its shorter half life has been reported to cause less coagulation abnormalities than older HES formulations. A recent study has demonstrated that in patients receiving Pentaspan®, a volume of up to 45ml/kg per 24hrs will not cause significant changes to coagulation parameters (Arellano et al. Anaesth Analg2005;100:1846–53). Following is a report of a coagulopathy caused by much lower doses of Pentaspan®. Case report: A 53 year old male was scheduled to undergo elective aortic valve replacement for severe aortic stenosis. He had no previous history of a bleeding disorder and one week pre-operatively he had normal coagulation parameters with an activated partial thromboplastin time (aPTT) of 33 seconds (normal range 24–36 sec). On the day of surgery, after sternotomy was performed, the patient experienced an allergic reaction to morphine and developed transient hypotension necessitating a small dose of phenylephrine and 500cc of Pentaspan®. The patient recovered, but subsequent to this, the activated clotting time (ACT) was &gt;300sec in the absence of heparin and the aPTT was 68sec. Other coagulation parameters included a thrombin time of 14sec (upper limit normal (ULN) 16sec), a fibrinogen of 3.7g/L and a heparin anti-Xa level of &lt;0.1 U/cc. The patient did not experience clinical bleeding. The case was aborted and further hematological investigated was initiated. Two hours post-operatively, coagulation parameters revealed the following: Factor VIII activity 0.89 U/cc, factor XI 0.78U/cc, Factor XII 0.28 U/cc, aPTT 62sec. The following day, the aPTT was 32sec and subsequent to this the patient was given a small dose (250cc) of Pentaspan®. One hour post-infusion, the aPTT had risen to 88 seconds. Factor levels were repeated two days later, and this time revealed a factor VIII level of 2.08 U/cc, with factor XII, XI and factor IX levels being similar to those on the day of the surgery. The aPTT on this day was normal at 34 seconds. This case report demonstrates that a small dose of the HES Pentaspan® caused a significant abnormality in the coagulation testing for this patient with underlying factor XII deficiency. It is hypothesized that normally his elevated factor VIII level compensated for the diminished factor XII level, resulting in a normal aPTT, but that after a small dose of Pentaspan®, the factor VIII level decreased sufficiently so the that the underlying factor XII deficiency was unmasked. Conclusion: An underlying factor deficiency can result in a laboratory coagulopathy (this case with factor XII deficiency) or potentially a clinical coagulopathy when even small doses of Pentaspan® are used. 1 week pre-op Pentaspan 500cc given intra-op ↓ 10 minutes post Pentaspan 2 hours post Pentaspan Post-operative day 1: pre Pentaspan Post-operative day 1: 1 hour post 250cc Pentaspan Post-operative day 3 Factor VIII 0.89 U/cc 2.08 U/cc Factor IX 1.09 U/cc Factor XI 0.78 U/cc 0.86 U/cc Factor XII 0.28 U/cc 0.26 U/cc Apt 33 sec 68 sec 62 sec 32 sec 88 sec 34 sec INR 1.1 1.1 1.1 1.1 1.3 1.2 Fibrinogen 3.7 g/L Thrombin Time 14 sec Heparin anti-Xa level &lt;0.1 U/cc

Prevalence of Thrombophilia in Patients with Retinal Vascular Occlusion

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 5341-5341
Author(s):  
Maria Teresa De Sancho ◽  
Karen Powell-Boone ◽  
Karen Carlson
Keyword(s):  
Risk Factors ◽  
Factor Viii ◽  
Factor V Leiden ◽  
Vascular Occlusion ◽  
Factor V ◽  
Factor Viii Level ◽  
Female Hormones ◽  
Retinal Vascular Occlusion ◽  
Viii Level ◽  
Prothrombin Gene Mutation

Abstract Background: Retinal vascular occlusion (RVO), both arterial (RAO) and venous (RVO) is an important cause of visual loss. Inherited and acquired thrombophilia are considered important risk factors in the development of RVO. Additionally, cardiovascular risk factors such as hypertension, diabetes, hyperlipidemia, smoking and use of female hormones may play a role. Objective: To determine the prevalence of thrombophilia in patients with RVO. Methods: We reviewed the medical records of all patients with documented RVO who were referred to our hematology clinic at a tertiary care center between January 2001 and June 2008 for thrombophilia evaluation. Thrombophilia evaluation included testing for factor V Leiden, prothrombin gene mutation G20210A, deficiencies in antithrombin, protein C and S, presence of antiphospholipid antibodies (aPLas), hyperhomocysteinemia and increased levels of lipoprotein (a), factor VIII, IX and XI. Serum protein electrophoresis and immunofixation were also performed. Environmental risk factors analyzed included use of female hormones, pregnancy and puerperium, smoking, hypertension, hyperlipidemia and diabetes. Results: A total of 24 patients were identified; 10 were males and 14 females. The mean age was 62 years (range, 31 to 86 years). Seven patients had RAO and 17 RVO. Of the 24 patients, 19 (79%) had thrombophilia. Of these, 10 had only one thrombophilia and 9 had ≥2 thrombophilia. Of the 10 patients with a single thrombophilia, 3 patients had aPLas, 3 had increased lipoprotein (a) 2 had factor V Leiden, 1 hyperhomocysteinemia, and 1 had increased factor VIII level. Of the nine patients with combined thrombophilia: 3 had hyperhomocysteinemia and increase factor VIII level, 2 prothrombin gene mutation and positive lupus anticoagulant, 1 positive aPLas, hyperhomocysteinemia and a monoclonal gammopathy, 1 aPLas and increased factor VIII, 1, hyperhomocysteinemia and protein S deficiency and 1 hyperhomocysteinemia and polycythemia vera. In 5 patients, no thrombophilia was identified. Of the 24 patients, 11 had hypertension, 9 had hyperlipidemia, 3 were smokers, 3 were using female hormones and 1 was pregnant. Treatment included aspirin 81 mg (n= 9), aspirin &gt;81 mg (n= 5), warfarin (n= 3), folic acid (n=2), aspirin and clopidogrel (n=1), clopidogrel (n=1), no treatment (n=4), and unknown (n=1). Conclusions: Over three quarters (79%) of patients with RVO had an underlying thrombophilia with aPLas, hyperhomocysteinemia and increased factor VIII level being the most prevalent. Our findings suggest that testing for thrombophilia particularly for aPLas, homocysteine and factor VIII levels may be useful in patients with RVO

Effect of corticosteroids on factor VIII level

1967 ◽  
Vol 71 (6) ◽  
pp. 878-880 ◽  
Author(s):  
Virginia C. Canale ◽  
Margaret W. Hilgartner ◽  
Carl H. Smith ◽  
Philip Lanzkowsky
Keyword(s):  
Factor Viii ◽  
Factor Viii Level ◽  
Viii Level
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