Major Surgery in a Subject with Factor V Deficiency

1971 ◽  
Vol 25 (03) ◽  
pp. 438-446 ◽  
Author(s):  
E. J Melliger ◽  
F Duckert
Keyword(s):  
Intercellular Space ◽  
Correct Diagnosis ◽  
Postoperative Bleeding ◽  
Major Surgery ◽  
Factor V ◽  
Factor V Deficiency ◽  
Disappearance Time ◽  
Fresh Plasma ◽  
One Year

SummaryA further case of parahaemophilia is reported. One year after the correct diagnosis had been made the patient had to undergo cholecystectomy which was performed under prophylactic substitutive treatment with fresh plasma at a factor V level of 31 %. A minimal factor V level of 11 to 12% was maintained throughout the first week after operation. There was no abnormal postoperative bleeding. The half disappearance time of factor V was found to be about 12 h. Infusion of equivalent amounts of fresh plasma supplied a higher yield of factor V in the patient’s plasma before operation than postoperatively what may be explained by an increased diffusion of factor V into the intercellular space resulting from a postoperatively increased capillar permeability. The results are compared with those of other authors.

Management of Single Uncomplicated Dental Extractions and Postoperative Bleeding Evaluation in Patients With Factor V Deficiency: A Local Antihemorrhagic Approach

2018 ◽  
Vol 76 (11) ◽  
pp. 2280-2283 ◽  
Author(s):  
Pier Carmine Passarelli ◽  
Paolo De Angelis ◽  
Guido Pasquantonio ◽  
Paolo Francesco Manicone ◽  
Fernando Verdugo ◽  
...  
Keyword(s):  
Postoperative Bleeding ◽  
Factor V ◽  
Factor V Deficiency

Acquired Inhibitors of Factor V

1978 ◽  
Vol 39 (03) ◽  
pp. 663-674 ◽  
Author(s):  
Donald I Feinstein
Keyword(s):  
Physicochemical Properties ◽  
Prothrombin Time ◽  
Partial Thromboplastin Time ◽  
Laboratory Data ◽  
Major Surgery ◽  
Factor V ◽  
Igg Antibodies ◽  
Factor V Deficiency ◽  
Close Relationship ◽  
Polyclonal Igg

SummaryThe clinical and laboratory data on the 12 patients with an acquired inhibitor to factor V have been reviewed. The degree of clinical bleeding in these patients varied from none to severe, and in most patients the inhibitor was transient. The combination of a markedly prolonged partial thromboplastin time and Quick prothrombin time and failure of normal plasma to correct these tests, in the presence of a normal thrombin and prothrombin and proconvertin time, seems to be pathognomonic for a factor V inhibitor. The inhibitors have physicochemical properties of immunoglobulins and a few have been characterized as polyclonal IgG antibodies or a mixture of IgM and IgG antibodies. The etiology and pathophysiologic mechanism of their development is unknown, but there seems to be a close relationship to major surgery. When tested with inhibitor plasma, the plasmas from 9 patients with hereditary factor V deficiency from 7 unrelated families did not contain factor V antibody-neutralizing material.

scholarly journals Acquired Inhibitors of Factor V

1977 ◽  
Author(s):  
Donald I. Feinstein
Keyword(s):  
Temporal Relationship ◽  
Major Surgery ◽  
Severe Bleeding ◽  
Factor V ◽  
Inhibitor Activity ◽  
Physiochemical Properties ◽  
Hereditary Factor ◽  
Factor V Deficiency ◽  
Antigenic Material ◽  
Close Temporal Relationship

Twelve patients with an acquired inhibitor of Factor V have been reported thus far in the literature. Of these, only one occurred in a patient with hereditary Factor V deficiency. Six patients received streptomycin in close temporal relationship to the appearance of the inhibitor. Six of the patients had been previously transfused, including four of those who received streptomycin. In nine of the eleven spontaneously occurring inhibitors, major surgery preceded the appearance of the inhibitor. The degree of clinical bleeding in these patients varied. One patient had no bleeding, six patients had mild to moderate bleeding, and four patients had severe bleeding. The inhibitor disappeared in less than eight weeks in seven patients, whereas in one patient it persisted for more than two years. Most of these inhibitors have the physiochemical properties of antibodies. Six of the spontaneous inhibitors appeared to be IgG, whereas in two patients inhibitor activity was found in both IgM and IgG fractions. Three inhibitors have been typed with light chain antisera and all contained both kappa and lambda chains. Plasmas from seven patients with hereditary Factor V deficiency have been tested with three of these inhibitors for inactive factor V antigenic material and none has been detected. In addition, plasma from a patient with hereditary factor V deficiency has been tested with heterologous factor V antibody and no antigenic material has been found. Thus hereditary factor V deficiency probably represents a deficiency of factor V molecules, rather than the synthesis of a defective molecule.

Congenital Combined Deficiency of Factor VIII (Antihaemophilic Globulin) and Factor V (Proaccelerin) in Two Siblings

1967 ◽  
Vol 17 (01/02) ◽  
pp. 194-204 ◽  
Author(s):  
F Gobbi ◽  
E Ascari ◽  
U Barbieri
Keyword(s):  
Factor Viii ◽  
Mutant Gene ◽  
Factor V ◽  
Dominant Type ◽  
Factor Viii Deficiency ◽  
Factor V Deficiency ◽  
Common Precursor ◽  
The Family ◽  
Fresh Plasma ◽  
Combined Deficiency

SummaryTwo cases of congenital combined deficiency of factor VIII (antihaemophilic globulin) and factor V (proaccelerin) in 2 sibilings (a female and a male) born of non-consanguineous parents are reported.Mild isolate defect of factor V was demonstrated in the mother and in 2 maternal aunts, while pure factor VIII deficiency was found in a male relative on the maternal side.Infusion of normal fresh plasma lead in both cases to a parallel rise of both factors, while infusion of haemophilic plasma lead to a rise of factor V only, thus excluding the presence in the haemophilic plasma of a common precursor to both factors.The genetic study of the family seems to suggest that the two defects are inherited according to different patterns, two genes being responsible for the two defects. Factor V deficiency seems inherited according to an autosomal incompletely dominant type of heredity, while factor VIII deficiency is due to a sex-linked mutant gene.Genie interaction, inversion of the dominance or early inactivation of the normal X-chromosome in a carrier are the possible explanations for the severe factor VIII deficiency in the proposita.

Severe Factor V Deficiency with Prolonged Bleeding Time

1974 ◽  
Vol 32 (02/03) ◽  
pp. 538-548 ◽  
Author(s):  
Kees Bkeederveld ◽  
Eric A. van Royen ◽  
Jan W. ten Cate
Keyword(s):  
Aortic Coarctation ◽  
Glass Bead ◽  
Bleeding Time ◽  
Dominant Mode ◽  
Congenital Defects ◽  
Factor V ◽  
Prolonged Bleeding ◽  
Prolonged Bleeding Time ◽  
Factor V Deficiency ◽  
Fresh Plasma

SummaryA patient with two congenital defects, i.e. a severe factor V deficiency and an aortic coarctation is described. Arguments for a dominant mode of inheritance of the factor V deficiency are presented. A coinciding haemostatic defect consisting of a prolonged bleeding time, which could be corrected by fresh plasma and cryoprecipitate, and a decreased platelet retention in the glass bead test disappeared after surgical correction of the coarctation.Extensive platelet studies failed to show any further defect.

Molecular Characterization of a Type I Quantitative Factor V Deficiency in a Thrombosis Patient that Is “Pseudo Homozygous” for Activated Protein C Resistance

1997 ◽  
Vol 77 (02) ◽  
pp. 252-257 ◽  
Author(s):  
Joan F Guasch ◽  
Ruud P M Lensen ◽  
Rogier M Bertina
Keyword(s):  
Protein C ◽  
Dna Analysis ◽  
Activated Protein C ◽  
Type I ◽  
Factor V ◽  
Sequencing Analysis ◽  
Apc Resistance ◽  
Quantitative Factor ◽  
Factor V Deficiency ◽  
Fv Leiden

SummaryResistance to activated protein C (APC), which is associated with the FV Leiden mutation in the large majority of the cases, is the most common genetic risk factor for thrombosis. Several laboratory tests have been developed to detect the APC-resistance phenotype. The result of the APC-resistance test (APC-sensitivity ratio, APC-SR) usually correlates well with the FV Leiden genotype, but recently some discrepancies have been reported. Some thrombosis patients that are heterozygous for FV Leiden show an APC-SR usually found only in homozygotes for the defect. Some of those patients proved to be compound heterozygotes for the FV Leiden mutation and for a type I quantitative factor V deficiency. We have investigated a thrombosis patient characterized by an APC-SR that would predict homozygosity for FV Leiden. DNA analysis showed that he was heterozygous for the mutation. Sequencing analysis of genomic DNA revealed that the patient also is heterozygous for a G5509→A substitution in exon 16 of the factor V gene. This mutation interferes with the correct splicing of intron 16 and leads to the presence of a null allele, which corresponds to the “non-FV Leiden” allele. The conjunction of these two defects in the patient apparently leads to the same phenotype as observed in homozygotes for the FV Leiden mutation.

Plasma Contact Activation and Decrease of Factor V Activity on Negatively-Charged Polyelectrolytes

1984 ◽  
Vol 51 (01) ◽  
pp. 061-064 ◽  
Author(s):  
M C Boffa ◽  
B Dreyer ◽  
C Pusineri
Keyword(s):  
Time Dependent ◽  
Initial Contact ◽  
Factor Xii ◽  
Factor V ◽  
Contact Activation ◽  
Polyelectrolyte Complexes ◽  
Plasma Factor ◽  
Fresh Plasma ◽  
Direct Adsorption

SummaryThe effect of negatively-charged polymers, used in some artificial devices, on plasma clotting and kinin systems was studied in vitro using polyelectrolyte complexes.Contact activation was observed as an immediate, transient and surface-dependent phenomenon. After incubation of the plasma with the polymer a small decrease of factor XII activity was noticed, which corresponded to a greater reduction of prekallikrein activity and to a marked kinin release. No significant decrease of factor XII, prekallikrein, HMW kininogen could be detected immunologically. Only the initial contact of the plasma with the polyelectrolyte lead to activation, subsequently the surface became inert.Beside contact activation, factor V activity also decreased in the plasma. The decrease was surface and time-dependent. It was independent of contact factor activation, and appeared to be related to the sulfonated groups of the polymer. If purified factor V was used instead of plasma factor V, inactivation was immediate and not time-dependent suggesting a direct adsorption on the surface. A second incubation of the plasma-contacted polymer with fresh plasma resulted in a further loss of Factor V activity.

“In vitro” correction of the severe factor V deficiency-related coagulopathy by a novel plasma-derived factor V concentrate

Haemophilia ◽  
2018 ◽  
Vol 24 (4) ◽  
pp. 648-656 ◽  
Author(s):  
C. Bulato ◽  
C. Novembrino ◽  
M. Boscolo Anzoletti ◽  
L. Spiezia ◽  
S. Gavasso ◽  
...  
Keyword(s):  
Factor V ◽  
Factor V Deficiency

scholarly journals An Unsual Site about Small Cells Osteosarcoma Parietal Region and Classique Osteosarcoma Occipital Region Mimicking Meningioma with Literature Review Management and Outcome

2019 ◽  
Vol 2 (1) ◽  
Keyword(s):  
Correct Diagnosis ◽  
Small Cell ◽  
Small Cells ◽  
Long Bones ◽  
Unusual Site ◽  
First Case ◽  
Review Management ◽  
Similar Materials ◽  
One Year ◽  
Age Range

Introduction: Primary osteosarcoma (OS) is the second most common primary bone malignancy, the first being multiple myeloma. OS occurs in the second decade, with a predilection for ends of long bones. Head and neck involvement is seen in 2-9% with extragnathic craniofacial bones in 1–2% of cases. Small Cell OS (SCO) constitutes 1.3-4% of all OS, skeletal distribution and age range being similar. Materials and Methods: We report two rare osteosarcoma and we done the review of the literature about the management and the outcome about intracranial osteosarcoma in our department of neurosurgery. Results: It is two osteosarcoma cases about a 72-year-old man and one 49-year-old man who both mimiking first meningioma. The first case is an unusual site parietal and the second case is occipital. The both benefited surgery with excision and exam of histology confirm diagnosis. But the first case died 15 days after surgery in intensive unit care and the second cases died after one year, he benefited surgery and chemotherapy. Conclusion: Small cell osteosarcoma (SCO) is an extremely uncommon entity that mainly involves the metaphysics of long bones and, rarely, the skull. Histopathology is the key to establishing the correct diagnosis, including sub typing for appropriate management and prognostication, as radiological features are not specific.

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