[Acquired deficiency of coagulation factor VIII in post partum with transplacental passage].
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Biomedical subjects
Publications and source records attributed to M E Briquel.
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A new generation of techniques can be applied to the selective removal of some well-defined molecules from plasma. This is the field of plasma treatment. We investigated the selective removal of LDL cholesterol, by double filtration and dextran sulfate cellulose column plasmapheresis, from 3 to 4 liters of plasma from 4 hypercholesterolemic patients. The extraction rate of Apo A1 lipoprotein in dextran sulfate was less than 25% and Apo B was between 50 and 90%. Cascade filtration gave an extraction rate for Apo A1 between 25 and 50%; the extraction rate for Apo B was about the same as that found using the dextran sulfate procedure. Both techniques decreased the concentrations of coagulation factors (V, VIII and fibrinogen). In conclusion, we obtained good removal of LDL cholesterol with both systems. Dextran sulfate (from Kaneka) was more selective but very expansive. However, we observed side effects with this product that probably result from the release of dextran sulfate from the column.
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The evaluation of Dideco's Double Ring reveals a sensitive improvement on the Single Ring. This system provides a platelet concentrate of over 4x 10(11) platelets in 75 minutes. It is reasonable to envisage yet shorter and more efficient processes, as suggested by VALBONESI et al. However, considering the platelet transfusion requirements, it would be interesting to have a plastic 5-day preservation closed system.
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Blood lymphocyte proliferative responses to mitogens were studied in 65 patients with haemophilia (haemophilia A: 54 patients, haemophilia B: 11 patients) in parallel with 39 male control subjects. As a group, patients with haemophilia did not demonstrate abnormal proliferative responses to phytohaemagglutinin (PHA), Concanavalin A (ConA) and pokeweed mitogen (PWM) when compared with healthy controls. When the patients were analysed according to their seropositivity for antibody to human immunodeficiency virus (HIV), those who were positive had significantly decreased PHA, ConA and PWM responses. Haemophiliac patients with T4+/T8+ ratios less than 1 had reduced proliferative responses to PHA, ConA and PWM when compared to patients with ratios greater than 1. No significant difference in mitogen responses were found when the patients were analysed according to the presence or absence of palpable lymphadenopathy. Those patients with haemophilia A who had received more than 5 x 10(4) units of factor VIII during the two years preceding the study showed no significant difference in PHA, ConA and PWM responses when compared to patients receiving less.
Tolerance and efficacy of single i. v. injections of PK 10169 (1 mg/kg) during installation were evaluated in 10 patients with stable chronic renal insufficiency treated with 3 sessions of hemodialysis weekly (sessions of 4 and 5 hours). Tolerance was good in all cases. Efficacy was globally satisfactory (no complete coagulation) but by the 4th hour in 7 out of 30 sessions coagulation was apparent in the bubble trap with elevation of residual blood volume, recurring in the same patient but not affecting result of dialysis. In these cases anti-Xa activity was reduced from the 2nd hour and at end of dialysis, with a markedly elevated FPA level. For most patients it is possible to envisage 5-hour sessions, but in those few susceptible to develop coagulation repeated clinical surveillance of all circuit elements is necessary.
Heparin induced thrombocytopenia is characterized by often dramatic evolution of thrombotic arterial and venous complications. These occurred or are worsened in curative or preventive heparin therapy and the treatment remains delicate. The authors report 32 observations of thrombocytopenia complicated with thromboembolic events; seven deaths are to mention. The tests of platelet aggregation with standard heparin and platelets poor patient's plasma confirm the diagnosis in 28 cases of 30 very early studied and in 2 cases around the 6th day only after the stop of standard heparin. The choice of anticoagulant therapy is carried out the negative tests of platelet aggregation with low molecular weight heparin (L.M.W.H.) (CY 216, CY 222 Choay, PK 10109 Pharmuka). In L.M.W. heparin therapy, clinical and biological improvement is obtained in 26 cases of 30 treated cases. In three cases, the rapid climbing of platelet countings is not present although negative tests of platelet aggregation with selected L.M.W.H. In one case, after a initial climbing of platelet countings, the thrombocytopenia recurs rapidly with L.M.W. heparin after a operation. The immediate or secondary passage with K-antivitamins, platelet antiaggregant stabilized medium and long term's evolution.
A case of female hemophilia with a 46,XXr/45,X karyotype and signs of Turner syndrome, has been followed for the past 10 years. One of her brothers also has hemophilia A. A study with polymorphic DNA probes located in the Xq27-qter region has enabled us to demonstrate that the ring chromosome is of paternal origin and that the factor VIII gene region is deleted. The hemizygous state allowed expression of the hemophilia A mutation, present on the morphologically normal X chromosome, inherited from her carrier mother.
We studied a group of 64 patients undergoing cardiac surgery for the occurrence of post-transfusion hepatitis during a follow-up period of 5 months. They received blood units (packed red cells in saline-adenine-glucose medium and/or fresh frozen plasma exclusively) from 447 volunteer donors. Post-transfusion hepatitis was identified in 5 patients: 1 patient had cytomegalovirus hepatitis and the remaining 4 cases were defined, by exclusion, as non-A, non-B hepatitis (with prevalence and incidence rates of 80% and 6.25% respectively). We found no statistically significant differences between the numbers of transfused blood product units in patients who developed non-A, non-B hepatitis as compared to those who did not. Our analysis of the predictive effectiveness of alanine aminotransferase and anti-HBc antibodies screening in blood donors to prevent non-A, non-B post-transfusion hepatitis led to the following conclusions: we failed to confirm the association between anti-HBc in blood donors and enhanced risk of non-A, non-B hepatitis in recipients since no case developed among patients receiving blood products from anti-HBc positive donors. So, 20 donors (4.5%) would have been discarded without any reduction of the incidence of non-A, non-B hepatitis. we could not confirm nor exclude the possibility that screening donor blood for elevated alanine aminotransferase levels would have reduced the number of non-A, non-B hepatitis in recipients.
A case is reported of an 80 year old woman with old sarcoidosis and acquired factor VIIIc inhibitor undergoing orthopaedic surgery. This was successfully carried out after she had been given a total of 48,420 units of factor VIII concentrate: there were no haemorrhagic complications. The main pathological states in which acquired factor VIIIc inhibitor may be found are related: principal treatments available nowadays are discussed: immunosuppressors, human factor VIII, animal factor VIII, prothrombin complex concentrate, plasma exchange.
From 7 cases of abnormalities involving chromosome 13, the structural gene(s) coding for coagulation factors VII and X were located in the region 13q34-13qter. Gene-dosage effects for these coagulation factors seem to act in both directions, causing a decrease when there is monosomy of segment 13q34, but also, as has not been demonstrated before, an increase when there is trisomy of this same segment.
49 french haemophiliacs (haemophilia A: 41 patients; haemophilia B: 8 patients) were serologicaly tested for LAV antibodies: 10 patients (20.4%) were seropositive including 9 (21.9%) with haemophilia A and 1 (12,5%) with haemophilia B. Between seronegative and seropositive patients total lymphocyte and T-lymphocyte sub-populations counts were not significantly different. The mean serum IgG level was higher and palpable lymphadenopathy more frequently encountered among seropositive patients.
49 French haemophiliacs (haemophilia A: 41 patients; haemophilia B: 8 patients) were serologically testes for lymphadenopathy-AIDS virus antibodies: 10 patients (20.4%) were seropositive including 9 (21.9%) with haemophilia A and 1 (12.5%) with haemophilia B. Among haemophiliacs A, seropositive patients received significantly larger amounts of factor VIII concentrate during the 2 years preceding the study.
Blood T-lymphocyte subsets and serum immunoglobulin levels were studied in a group of 52 haemophiliacs (44 patients with haemophilia A and 8 patients with haemophilia B). None of the patients had AIDS or belonged to any AIDS high-risk group. Patients were exclusively treated with clotting fractions obtained from healthy volunteers in metropolitan France. As compared to a group of 52 normal donors, haemophiliacs had increased numbers of suppressor lymphocytes, which resulted in depressed helper/suppressor (H/S) ratios, and increased levels of serum IgG and IgA. 21 haemophiliacs (40,3%) had a H/S ratio less than 1.4. Among patients with haemophilia A a higher mean IgG level was found in patients presenting lymphadenopathy. Decreased mean H/S ratio and increased mean serum IgG level were found in patients receiving more than 50 000 U of factor VIII during the 2 years preceding the study. No striking difference in mean serum IgG, IgA and IgM levels was found in patients with haemophilia A when H/S ratios were higher and lower than 1.4 respectively. As AIDS and immunological abnormalities among haemophiliacs probably share a common viral origin, this study emphasize the need to discourage blood donation from donors who belong to any AIDS high-risk group, and to screen sera from the blood donor population for antibodies to LAV/HTLV III.
We have studied a group of 31 hemophiliac patients (hemophilia A: 26 patients, hemophilia B: 5 patients); 29 healthy men were used as controls. Hemophiliac patients had increased percentages of suppressor T-lymphocytes and depressed T4/T8 ratios. These abnormalities were found to be significantly correlated with the amount of F VIII used per year.
The authors describe the preparation of a first batch of intravenous cytomegalovirus (CMV) immune globulin at the Nancy Regional blood transfusion centre. Immune plasmas were selected from 3 640 healthy volunteer blood donors on the basis of CF antibody titers to CMV (Kolmer's method modified) of, at least, 1:8; plasmas from approximately 10% of the donors were therefore selected. The 68 liters of pooled immune plasma had à CF antibody titer of 1:16 (CMV antibody titers of 1: 10 000 and 1: 640 when tested in the ELISA assay and passive hemagglutination assay respectively). Intravenous immune globulin was produced from pooled plasma by Cohn fractionation and treatment with pepsin at pH 4; 4.8 liters of immune globulin were prepared and divided in 96 doses of 50 ml each. The final product was found to have a CMV antibody titer of 1: 32 (CF) 1: 50 000 (ELISA) or 1: 2 560 (passive hemagglutination). Recent reports on the preparation of CMV immune globulin are briefly reviewed.