Active and inactive factor VII in Dubin-Johnson syndrome with factor-VII deficiency, hereditary factor-VII deficiency and on coumadin administration.
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Combined factor VII and factor VIII deficiency due to a casual association of heterozygosis for factor VII deficiency and hemophilia A.
A patient with combined factor VII and factor VIII deficiency is discussed. The propositus is a 21-year-old male who presented a mild bleeding tendency. The patient appears to be a hemophilia and at the same time heterozygote for factor VII deficiency. This conclusion is based on the fact that heterozygosis for factor VII deficiency was present in the father and in other relatives of the paternal side. On the contrary, no factor VII deficiency was present in the maternal side of the family. However, the maternal grandfather was known to have been a bleeder and the propositus' mother, his sister and his aunt had low-normal factor VIII levels and were probably hemophilia A carriers. This type of combined factor VII and factor VIII deficiency appears to be due to the casual association of two independently segregating defects.
Adult↗
[Hereditary heterozygote factor VII deficiency].
Hereditary Factor VII deficiency is one of the rare congenital coagulopathies. Prolonged prothrombin time (PT) with normal partial prothrombin time (PTT) may be an indicator for Factor VII deficiency. A family with hereditary heterozygous Factor VII deficiency is presented in whom no symptoms of a bleeding disorder were clinically detectable. A discussion of the therapeutic options follows.
Blood Coagulation Tests↗
[A new case of homozygous congenital factor VII deficiency. Associated factor X deficiency in some members of the family].
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Blood Coagulation Tests↗
Characterization and comparison of immune-depleted and hereditary factor-VII-deficient plasmas as substrate plasmas for factor VII assays.
The functional activity of coagulation factors usually is determined using bioassays dependent on substrate plasmas deficient in the factor being measured. These are obtained most often from patients with hereditary deficiency and are frequently poorly characterized. The authors characterized seven commercially available hereditary Factor-VII-deficient substrate plasmas in terms of Factor VII activity and antigen level, and then compared them with a Factor-VII-deficient plasma prepared by immunoadsorption. All of the hereditary Factor-VII-deficient plasmas contained significant quantities of Factor VII antigen (120-217 ng/mL; normal, 250-600 ng/mL). One of the commercial substrate plasmas showed significant factor VII coagulant activity with a human thromboplastin. The sensitivity of the Factor VII assay system was dependent on both the source of Factor-VII-deficient plasma and the origin of the thromboplastins; human thromboplastin was more sensitive than rabbit thromboplastin, and immune-depleted plasma gave the most sensitive assay with each thromboplastin. These results indicate that immunoadsorbed plasma may be a superior reagent for coagulation factor assays.
Blood Coagulation Tests↗
Hereditary factor VII deficiency in pregnancy: peripartum treatment with factor VII concentrate.
This report describes pregnant sisters with severe factor VII deficiency and peripartum management with factor VII concentrate. In this family, two affected members had previously developed severe postpartum hemorrhage when managed with fresh frozen plasma. Therapy-related complications owing to plasma infusion included viral disease transmission and pulmonary edema. Therefore, to lessen the risks of complications, specific factor therapy was initiated shortly before parturition in both patients. Factor VII concentrate was administered prior to delivery and every 6 hr for the next 72 hr to keep trough levels at approximately 10%. Based on peak and trough levels, the mean factor VII half-lives were determined to be 3.6 and 5.7 hr. Use of the concentrate was associated with uncomplicated delivery and minimal postpartum bleeding.
Factor VII↗
Factor VII deficiency and pregnancy.
Hereditary factor VII deficiency is very rare in pregnancy (one in 500,000). However, obstetricians should consider this diagnosis whenever the prothrombin time is prolonged while the activated partial thromboplastin time is normal. The factor VII level increases in normal pregnancy, but the effect of pregnancy upon the factor VII level in deficient individuals is unknown. We report two cases of factor VII deficiency in pregnancy. In both, factor VII levels were 15% or less in the third trimester and were lower postpartum, suggesting that pregnancy does elevate factor VII in deficient individuals as well. Fresh frozen plasma is the treatment of choice. If the level is very low, fresh frozen plasma can be given prophylactically; otherwise, it should be given if blood loss becomes excessive.
Adolescent↗
Atherosclerosis and coronary bypass surgery in hereditary factor VII deficiency.
A 66-year-old man with homozygous deficiency of factor VII (less activity than 4 percent of normal) had a minimal hemorrhagic tendency and severe coronary atherosclerosis, and underwent aortocoronary saphenous vein bypass surgery. Although plasma factor VII coagulant activity and cross-reacting material were markedly reduced, comparable amounts of factor VII antigen were detected in peripheral blood mononuclear cells of both the patient and of a normal subject by Western blotting techniques. Accelerated coagulation was observed following brief exposure of the patient's phytohemagglutinin-stimulated peripheral blood mononuclear cells to low concentrations of ambient factor VII in vitro. Evidence indicates that factor VII plays a role in vivo in both hemostasis and atherogenesis and it might be assumed that factor VII deficiency would both predispose to excessive bleeding and forestall atherosclerosis. However, these observations suggest that factor VII-mediated thrombin generation may proceed by partitioning of small amounts of factor VII on tissue factor-expressing cells and that factor VII contained within monocytes may facilitate tissue factor-induced coagulation by these cells. These features may provide efficient coagulation activation despite a deficiency of the plasma coagulant protein. The current results may explain, at least in part, the minimal bleeding tendency, and also the occurrence of thrombosis and atherosclerosis in certain persons with factor VII deficiency.
Aged↗
Combined factor V and factor VII deficiency. Report of a case with a record on combined defects and considerations on the relevance of partial deficiency of coagulation factors.
A patient suffering from cardiochalasia was found to be partially deficient in both coagulation factors V and VII. No bleeding tendency had been noticed. A family study showed that the father had factor VII deficiency with normal factor V, while the mother and 2 sisters had a reduced level of factor V and normal factor VII. Thus, the combined deficiency was due to chance association of two distinct independently segregating genetic defects. While a number of combinations of coagulation factor deficiency have been previously described, this, to be best of our knowledge, is the first instance of combined deficiency of factor V and VII reported so far.
Adolescent↗
Factor VII deficiency and surgery. Is preoperative replacement therapy necessary?
A patient with congenital factor VII deficiency (factor VII was 12%) had gynecologic surgery performed without prophylactic blood-product replacement therapy. Blood loss was not excessive. A review of 12 additional patients with factor VII deficiency who underwent surgery without replacement therapy showed that surgical bleeding was uncommon and that there was no relationship between factor VII levels and hemorrhage. It is proposed that patients who bleed may be those who also have a prolonged bleeding time or who have ingested aspirin shortly before surgery. It is all proposed that replacement therapy be available for use if required, but that its routine preoperative use is probably unnecessary in this disorder.
Adolescent↗
Congenital factor VII deficiency. A report of four new cases.
Four new cases with congenital homozygous factor VII deficiency are described. Factor VII levels were reduced to less than 1%, 3%, 8% and 10%, respectively. The incidence and severity of bleeding symptoms were well correlated with the measured factor VII activity. In the severe case of factor VII deficiency (less than 1%) a home treatment program was started because of severe recurrent hemarthroses. This entailed transfusions of 20 U/kg body weight prothrombin complex or factor VII concentrate in case of acute bleeding approximately every three weeks. These transfusions have been carried out successfully without any problems. In contradiction, two brothers with hypoproconvertinemia (factor VII 8% and 10% respectively) reached an age of more than 70 years. Despite replacement therapy postoperative bleeding followed one appendectomy, whereas no postoperative bleeding followed patients requiring Achilles tendon lengthening and an above knee amputation and only slight bleeding followed a tonsillectomy. Based on our experience we suggest that in patients with factor VII deficiency of less than 10%, when undergoing surgery, should be maintained a minimal factor VII activity of 10--15% during the first three postoperative days.
Adolescent↗
Treatment of congenital factor VII deficiency with a new concentrate.
A new factor VII concentrate, made from ACD plasma by a process involving successive absorptions of cryoprecipitate supernatant on DEAE Sephadex and of the resulting supernatant on A1(OH)3, was administered to 10 patients with severe factor VII deficiency. 5 patients received only one dose for treatment of a single bleeding episode, the remaining 5 were given multiple infusions (47) for spontaneous hemorrhages or for the prevention of surgical bleeding. In vivo factor VII recovery ranged from 43 to 126% (average 88%) of the assayed in vitro activity of the concentrate. A dose of 0.5 u/kg was found to produce a 1% rise of the plasma factor VII levels. The mean half-life on injected factor VII as assessed in 7 kinetic studies was 205 min (range 168--234). Spontaneous bleeding was easily controlled by the concentrate and major surgical procedures (two tonsillectomies) could be performed without complications. 1 patient developed HBSAg positive hepatitis, but otherwise no serious side effects were observed. Factor VII concentrate reduced the risk of precipitating circulatory overload associated with the use of plasma and avoids the unnecessary rise of factor II, IX and X which follows prothrombin complex concentrates.
Adolescent↗
Acquired factor VII deficiency associated with aplastic anaemia: correction with bone marrow transplantation.
We report a patient with severe aplastic anaemia found to have a prolonged prothrombin time due to acquired factor VII deficiency. No evidence for a factor VII inhibitor or inactivator was demonstrable. Laboratory studies identified deficiency both of factor VII activity and factor VII antigen. The factor VII deficiency persisted from clinical presentation until approximately 50 d after allogeneic marrow transplantation when restoration of factor VII activity and antigen was noted. The patient's serum could be depleted of factor VII activity by in vitro incubation with Protein A bound to Sepharose, suggesting the presence of an IgG or IgG containing complex able to bind factor VII, but not neutralize its procoagulant activity. A dual specificity solid phase immunoassay identified a factor VII binding immunoglobulin which was detectable throughout the course of factor VII deficiency. The concordant appearance of this factor VII reactive immunoglobulin and the factor VII deficiency suggested the pathologic role of this immunoglobulin in the aetiology of the factor VII deficiency. This factor VII binding immunoglobulin may have induced rapid plasma clearance of the factor VII molecule or, alternatively, may have modified factor VII synthesis. The immunosuppressive therapy and subsequent lymphohaematopoietic engraftment following allogeneic marrow transplant was accompanied by complete resolution of the factor VII deficiency.
Adolescent↗
A CRM-Positive variant of factor-VII deficiency and the detection of heterozygotes with the assay of factor-like antigen.
Nine patients with severe factor-VII deficiency, belonging to seven pedigrees were studied for the presence of factor-VII-CRM with an inhibitor neutralization assay. The antibody, raised in rabbits, did not precipitate the antigen and could only be used in a fluid phase assay to measure the capacity of plasma to neutralize inhibitory activity directed against factor-VII activity. In one of these nine patients normal amounts of factor-VII-CRM could be demonstrated. The CRM + patient did not show a clinical picture at variance with that of the CRM-patients. The investigation into this CRM+ pedigree revealed heterozygosity in nine out of 12 persons when using the ratio between biological factor-VII activity and factor-VII-CRM as the criterion.
Adolescent↗
Factor VII deficiency first observed at preoperative routine clotting test.
Factor VII deficiency was first observed in a 24-year-old female in the routine clotting test for tooth extraction. Her factor VII was 22%, mildly below normal limits. Factor IX complex was not administered as replacement therapy when her impacted tooth was extracted. There was no sign of abnormal postoperative bleeding. As this disease was a rare disorder, the minimum safe level of factor VII for tooth extraction was unknown. We had a opportunity to study a patient with this disorder observed in the preoperative routine clotting test for tooth extractions.
Adult↗
A syndrome of factor VII deficiency and abnormal platelet release reaction.
A 15-year-old girl with severe factor VII deficiency and chronic arthropathy showed an excessively prolonged bleeding time. Further studies demonstrated low platelet adhesiveness and abnormal platelet aggregation with ADP, collagen and epinephrine. Release of 14C-serotonin was deficient after aggregation with ADP and epinephrine, but was normal with thrombin. Transfusion of plasma or prothrombin complex concentrate resulted in a partial or complete correction of the bleeding time, respectively, but had no effect on in vitro platelet function tests. Both parents and the only sister had factor VII activities of 42%-72% and factor VII antigen levels of 45%-66% of normal and may thus be heterozygotes with respect to factor VII deficiency. All three had normal bleeding times in spite of abnormal in vitro platelet functions. The observations are interpreted to mean that in this family with factor VII deficiency and abnormal platelet release reaction the platelet abnormality as such was not sufficiently severe to prolong the bleeding time unless the factor VII activity was also very low.
Adenosine Diphosphate↗
[Gastrectomy for a patient with congenital factor VII deficiency--a case report].
A 76-year-old man with congenital factor VII deficiency was admitted to our hospital for the treatment of gastric cancer. On admission, the hepaplastin test was prolonged reaching to 21% and factor VII activity was reduced to 8%. After preoperative heat-treated prothrombin complex concentrates loading test, factor VII levels had been maintained above 20% by every four-hour infusion of concentrates during operation and the first post operative day. Bleeding tendency didn't occur, but thrombosis occurred at the left femoral vein. Pre and postoperative replacement therapy is essential to the patient with congenital factor VII deficiency for the safety of operation.
Aged↗
[Anesthesia and factor VII deficiency].
A case is reported of a 17 year-old patient undergoing emergency internal fixation of a mandibular fracture after a road traffic accident. Routine preoperative blood analysis revealed an isolated deficiency in factor VII (33%), with a normal activated partial thromboplastin time and a reduced prothrombin level (50%). Because there was no previous history of an haemorrhagic diathesis, the surgical procedure was carried out without any factor VII replacement. The course of surgery was normal, with no abnormal blood loss. The possible causes of this deficiency, and its treatment are discussed.
Adolescent↗