[Factor V Leiden and myocardial infarction: a case, review of the literature with a meta-analysis]. / Facteur V Leiden et infarctus du myocarde: à propos d'un cas, revue systématique de la littérature et méta-analyse.

Mutation in blood coagulation factor V Leiden is the most frequently genetic polymorphism implied in venous thrombosis. A 57 year old man was hospitalised for acute myocardial infarction (MI). An emergency coronary angiography was performed, and no significant stenosis was observed. The haematologic check-up showed an heterozygous Leiden mutation of factor V. We report all publications about the relation between factor V Leiden and coronary thrombosis, and we performed a meta-analysis. We analysed the relation in general population and in subgroups, such as, younger and older, and patient with or without coronary stenosis. In global population, the meta-analysis did not found significant association between Factor V Leiden and myocardial infarction (OR = 1.25; IC = 0.97-1.58). In contrast, in patients less than < 55 years old after MI, Factor V Leiden prevalence was significantly higher than in control group (OR = 1.48; IC = 1.05-2.08). In addition, after MI without significant coronary stenosis Factor V Leiden prevalence was significantly higher than in normal patients (OR = 2.84; IC = 1.46-5.51). After MI, in patients without significant coronary stenosis, Factor V Leiden prevalence was significantly higher than in patients with significant coronary stenosis (OR = 3.26; IC = 1.67-6.36). Our study suggests that Factor V Leiden could be search after MI in young subjects and/or without significant stenosis.

Coagulation abnormalities in lacunar and cortical ischemic stroke are quite different.

In order to clarify the coagulation profile accompanying ischemic stroke, which may have implications on therapeutic strategies, we performed a prospective study to evaluate the hemostatic parameters in the first 24 h after the onset of cortical atherothrombotic infarct and lacunar infarction. Twenty-seven patients with cortical atherothrombotic infarction and 27 patients with lacunar infarction, diagnosed on clinical and CT-scan criteria, had blood samples taken within the first 24 h after onset of the stroke, and before anticoagulant treatment had been started. Levels of fibrinogen, von Willebrand factor, D-dimers, prothrombin factors 1 + 2, anti-thrombin III, and C-protein and S-proteins, were measured. Laboratory tests detected the following abnormalities: a protein C deficiency was observed in 1 case of cortical infarction and in 1 case of lacunar infarction; a decrease in S-protein was observed in 1 case of cortical infarction, and the presence of lupus anticoagulant in 4 cases (2 in cortical and 2 in lacunar infarction). Various degrees of coagulation activation were observed. Statistically significant activation of the coagulation was observed in the patients with cortical infarction, compared to normal patients adjusted for age: the levels of DDI were significantly raised (2298 +/- 2221 ng ml-1 vs. 750 +/- 400 ng ml-1) (p < 0.03) as were F1 + 2 levels (3.9 +/- 2.8 nmol l-1 vs. 1.5 +/- 0.9 nmol l-1). (p < 0.01). In the lacunar infarction group, there was a significant rise in F1 + 2 compared with normal patients adjusted for age (2.2 +/- 1.7 nmol l-1 vs. 1.5 +/- 0.9 nmol l-1) (p < 0.01), while the DDI level was in the normal range, when age was taken into account. In the cortical infarction group, we observed a significantly raised fibrinogen level (4.8 +/- 1.7 g l-1 vs. 3.7 +/- 1.0 g l-1) (p < 0.05) and von Willebrand factor level (271 +/- 104% vs. 178 +/- 103%) (p < 0.01) compared to the lacunar infarction group. In addition, we observed a significantly low level of S-protein in the cortical infarction group (105 +/- 29%) compared to the lacunar infarction group (127 +/- 28%) (p < 0.01). Confirmation of the role of enhanced thrombin activity in the pathogenesis of acute stroke may be an important determinant in its therapeutic management.

Extracorporeal circuit heparinization in selective low density lipoprotein apheresis: changes in patient hemostasis and low molecular weight heparin benefit.

Treatment by low density lipoprotein (LDL) apheresis using dextran sulfate columns (DSC) leads to hemostasis alterations with prolonged activated partial thromboplastin time (APTT) of more than 120 seconds. In order to explain this hypocoagulability, we studied hemostasis parameters both in patients and in the extracorporeal circulation (ECC). Hemostasis changes are first related to unfractionated heparin (UFH)--needed to avoid circuit coagulation--which leads to high residual heparinemia in the patient (more than 3 times the recommended level for therapeutic use). Second, the hypocoagulability is induced by a coagulation factor decrease (primarily factors V, VIII, and X) mainly due to an adsorption mechanism on dextran sulfate. Studies on samples from column inflow, outflow, and eluate confirm this mechanism. Low molecular weight heparin (LMWH) can be used in LDL apheresis on DSC without major changes in lipid removal or coagulation factors compared to UFH. The benefit of using LMWH is to reduce residual heparinemia into the therapeutic range.

Continuous hemofiltration with a low molecular weight heparin, enoxaparine: report on two cases.

The heparinization of multiple trauma patients during hemofiltration involves a high risk of bleeding. We report here two continuous hemofiltration cases -15 and 60 days in duration-using a low molecular weight heparin, enoxaparine. No major bleeding or clotting was observed. However, continuous infusion of the heparin is needed and the rate of delivery of the hemofiltration pump must be taken into consideration since it influences the clearance of the drug through the hemofiltration membrane. Anti-Xa activity measurement is not sufficient to adjust the heparin dose.

[Physiopathologic and clinical implications of protein C deficiencies]. / Implications physiopathologiques et cliniques des déficits en protéine C.

The protein C system--a complex physiological coagulation inhibition system--is a major regulator which controls thrombin production. Nowadays, its exploration is indispensable for the detection of constitutional or acquired protein C deficiency, side by side with a search for antithrombin III deficiency. This approach, based on the frequent association of recurrent thrombosis, mainly venous, in young subjects with congenital deficiencies, makes it possible to initiate, beside a family survey, an individual and efficient prophylaxis of recurrent thrombosis.

[Emergency management of abnormalities of preoperative blood coagulation tests]. / Conduite à tenir en urgence devant une anomalie du bilan d'hémostase préopératoire.

Usual coagulation tests--Platelet count, Prothrombin Time (PT), Activated Partial Thromboplastine Time (APTT)--detect most of coagulation abnormalities in preoperative situation. Emergency surgery remains possible without bleeding in most cases. Patient history of haemostatic responses is absolutely necessary to know in addition to coagulation tests. Platelets transfusion may often be avoid in thrombocytopenic patients with platelet count higher than 50,000 mm3. The PT and/or APTT perturbations need further laboratory investigations to estimate the bleeding risk which is not constant. The correction of an antithrombotic treatment is usually easy except for drugs which inhibit platelet aggregation.

Incidence of hematopoietic malignancies in a well-defined population in France.

A registration of hematopoietic malignancies was established on January 1, 1980, in order to determinate accurately the incidence and epidemiological features of these diseases in the department of Côte d'Or (population 477,908). Information was collected from various sources (public and private laboratories, hospitals, general practitioners and death certificates). Over a 3-year period (1980-1982), 401 new cases were diagnosed among residents of the department. The crude incidence rates were 32.9/100,000 for males and 23.0/100,000 for females. The corresponding age standardized rates (using world standard population) were 26.1 and 15.9, respectively. The sex ratio was 1.8:1. In males, chronic lymphocytic leukemias, Hodgkin's and non-Hodgkin's lymphomas are the most common hematopoietic malignancies, followed by acute leukemias and multiple myelomas. In females, lymphomas are more frequent than acute leukemias, chronic lymphocytic leukemias and multiple myelomas. Compared to population based registries in other countries, incidence rates are among the highest reported, and are particularly high for chronic lymphocytic leukemia.

[Biological changes induced by the removal of the tourniquet in orthopedic surgery]. / Les modifications biologiques induites par la levée du garrot en chirurgie orthopédique.

In 49 patients undergoing surgery on the lower limb with the use of a tourniquet, arterial PO2 and PCO2 were determined. In 16 cases they were also determined in the ischemic limb. In 40 of these patients coagulation studies were done and in 8 cases on blood from both the general circulation and from the ischemic limb. Some minutes after recirculation, PCO2 of the general circulation increased by 6.7 +/- 9 mm Hg. This increase is closely correlated with ischemia time and patient's weight. In the ischemic limb, PCO2 is about 72 +/- 10 mm Hg. In the general circulation, PO2 decreased by 21,6 +/- 42 mm Hg after recirculation. The most important changes were found in aged patients. PO2 in the ischemic limb decreased by 19 mm Hg. The changes in systemic PO2 and PCO2 are probably due to the return of blood from the ischemic limb in the general circulation. No changes of the coagulation factors were found with the exception of a small increase of anti-plasmine. Fibrinolysis occurred in 5 of the 8 patients studied.

In vitro culture of spleen hairy cells.

In vitro culture of hairy cells were successful in 5 patients with hairy cell leukemia: splenic cells were cultured in methyl cellulose plus PHA-leucocyte conditioned stimulation medium for 7 days. Plating efficiency was 0.5% with colony cells having morphological and cytochemical characteristics of hairy cells. A linear correlation between the number of cells plated and the number of colonies produced was found for cell concentrations varying from 5.10(4) to 5.10(5).

[Antifactor VIII antibody in Rosai-Dorfman disease]. / Anticorps antifacteur VIII au cours d'une maladie de Rosai-Dorfman.

The case of a 72 year-old man with a sinus histiocytosis is reported. The clinical course was characterised by an hemorrhagic disorder linked to a factor VIII inhibitor. The characteristics of the antibody titer, affinity and saturability have been studied. In an attempt to stop the bleeding treatment included human and porcine factor VIII concentrates, plasmapheresis and immunosuppressive drugs. Repeated plasma exchanges did not modify the inhibitor titer for more than 24 h.

[Establishing a registry of malignant hemopathies in Côte-d'Or. Preliminary results]. / Mise en place d'un registre des hémopathies malignes en Côte-d'Or. Premiers résultats.

A registry of malignant haematological diseases was established on January 1st 1980 in order to assess the incidence and epidemiologic features of these diseases in the department of Côte-d'Or (population 470 000). Information was collected from various sources (public or private laboratories, public or private hospitals and death certificates). During the course of two years (1980 and 1981) 194 news cases were recorded. This registry collects acute leukaemia, lymphoid and myeloid proliferative diseases, lymphomas in leukaemic phase and myelodysplastic syndromes. The crude incidence rates were 25.0/100,000 in men and 16.1/100,000 in women. Sex ratio : 1.5. Compared to other French population based registries the incidence is higher in Côte-d'Or, showing the value of specialised registries. The rates are similar to those reported in Geneva and Connecticut.

[Sinus histiocytosis with massive lymphadenopathy: a new case in an adult with anti factor VIII antibody (author's transl)]. / Maladie de Rosaï: forme grave chez un adulte avec présence d'un anticorps antifacteur VIII.

The case history of a 71 year old french man with a lethal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman's Syndrome) is reported. The histological features were typical but the clinical course was remarkable because of the predominant expression was a life threatening hemorrhagic disorder closely linked to a circulating anticoagulant (anti-factor VIII). The bringing to light in a picture of hyperimmunity of a self antibody and a high anti-EBV titer suggest on lymphocyte T deficit could be important in the development of the sinus histiocytosis.