Overall study of the in vitro plasma clotting system in an invertebrate, Liocarcinus puber (Crustacea Decapoda): considerations on the structure of the Crustacea plasma fibrinogen in relation to evolution.

An overall study of the in vitro plasma coagulation system in the crab Liocarcinus puber has been carried out using various analytical methods, namely thromboelastography, spectrophotometrical examination, and a new one based on changes of the mechanical impedance of the developing clot. From the results reported here the clotting pattern in this species appears surprisingly complex for an invertebrate and unexpectedly closer to that of the vertebrates. Indirect evidences suggest that the fibrinogen polypeptide chains in this species and very likely in the other crustacean, are very different from those of the vertebrates. This would imply that crustacean and vertebrate fibrinogen would have diverged from one another in a far remote past, far beyond the individualization of the vertebrate alpha chain, that is, over 1.5 million years ago.

[Antithrombin III deficiency induced by heparin and constitutional protein C deficiency. Apropos of a case]. / Déficit en antithrombine III induit par l'héparine et déficit constitutionnel en protéine C. A propos d'un cas.

Major mesenteric venous thrombosis in a young man who had already had spontaneous thromboembolic accidents prompted the diagnosis in 1982 of 'Antithromin III' deficiency. The initial reduction of this inhibitor proved to be secondary to a consumption raised abnormally by Heparin. Research into the family antecedents made it possible in 1983 to discover a constitutional 'Protein C' deficiency without stopping the antivitamins K for this patient.

[A case of dysfibrinogenemia complicated by hemorrhage and thrombosis]. / Observation d'une dysfibrinogénémie se compliquant d'hémorragie et de thrombose.

There are, as well as the more common coagulopathies such as deficiency in antithrombin III or Protein C, certain rare disorders complicated simultaneously by haemorrhage and thrombosis--such as dysfibrinogenemia--which can present very special therapeutic problems.

Immunoglobulin G antibodies to lymphadenopathy-associated virus in differently treated French and Belgian hemophiliacs.

Immunoglobulin G antibodies to lymphadenopathy-associated virus have been detected in two groups of French hemophiliacs and in one group of Belgian hemophiliacs, whose mode of treatment differed. Seropositivity was more frequent (58.9%) in patients heavily transfused with blood products of French and foreign origin than in less frequently transfused persons (10.3%). The Belgian group, treated only with local products, showed the lowest frequency of seropositivity (3.4%). In healthy French controls, 1 of 330 had antibody to the virus. The results indicate transmission of lymphadenopathy-associated virus via blood-derived products.

[Clinical problems posed by the consumption of antithrombin III induced by heparin]. / Problèmes cliniques posés par la consommation de l'antithrombine III par l'héparine.

Two cases of resistance to the heparin treatment, induced by large consumption of antithrombin III, are reported. Normally small, this consumption does not automatically call for the systematic dosage of Antithrombin III for each treatment using heparin. On the other hand, any clinical or biological resistance to higher doses of heparin, any personal or family history of thrombosis necessitate the administration of Antithrombin III. Where there is an acquired or congenital deficit, an injection of Antithrombin III corrects the disorder and reduces the risk of thrombosis. In the case of a heparin-antivitamin K relay, it is essential to achieve complete biological effectiveness of the antivitamins K to interrupt the administration of the heparin.

[Physiopathology of hemostasis. Which advances are useful for the clinician?]. / Physiopathologie de l'hémostase. Quels progrès utiles aux cliniciens?

Progress made in the field of haemostasis 30 years ago was essentially to do with the identification of the different activators of coagulation, the deficits of which provoke hemorrhage. Over the last ten years, progress has been made which is of benefit to research into thrombosis. This wave of progress, still in its infancy, aids both the understanding of the mechanisms involved, and the treatment of thrombosis. The role of the deficits reducing coagulation in the occurrence of thromboses is the most distinctive if not unique, fact. The molecular study of the factors of coagulation constitutes another aspect of recent progress; this gives a better understanding of the pathological phenomena and anticoagulant behaviour. Examples are given of this progress in molecular study and in the aetiological diagnosis of thrombosis.

[Improper therapy in phlebology. Anticoagulants]. / La thérapeutique abusive en phlébologie. Les anticoagulants.

In phlebology, an anticoagulant treatment is usually called for in the fight against thrombosis. The favourable short-term development of most phlebites means that an anticoagulant treatment should not be used unless there are serious risks involved in the various complications observed. This treatment may therefore be used, on condition that diagnosis is certain, that the risks involved are serious enough; the least restrictive, least expensive form of treatment is preferable, provided that it is as effective, of course; the conditions of applications should be scrupulously upheld: no contraindications, the cooperation of the patient, quality of the biological controls etc. If these conditions are respected, the anticoagulant treatment combines efficiency and safety to a degree which places it foremost among anti-thrombotic methods, after physiotherapy.

[Asplenia: a study of 21 subjects splenectomized during childhood for spleen injury (author's transl)]. / L'asplénie. Etude de 21 sujets splénectomisés dans l'enfance pour traumatisme.

Clinical and biological studies were carried out in 21 subjects one to twenty-three years after the injured spleen had been removed. Asplenia did not give rise to clinical symptoms, did not facilitate infections and had no effect on growth. Laboratory investigations revealed a decrease in IgM in 6 subjects and minor quantitative and qualitative platelet abnormalities. The absence of Jolly's bodies in 7 subjects was suggestive of spleen regeneration, which was confirmed by scintigraphy in 2 cases. The significance of these findings in discussed.

[Pseudo-tumoral haematoma of the iliac fossa in haemophiliacs (author's transl)]. / Hématomes d'allure tumorale, de localisation iliaque, chez les hémophiles.

Pseudo-tumoral blood collections in haemophiliacs are uncommon but must be borne in mind because of their severity and of the need for timely and appropriate treatment. Surgical excision appears to be required as soon as the tendency towards enlargement is recognized and before the volume and complications of the tumour increase the difficulties, particularly in the iliac fossa. Other treatments, especially puncture-aspiration, seem to be ineffective and/or dangerous. A better knowledge of the pathogenic processes involved might lead to new therapeutic approaches.

[Familial recurrent phlebitis due to deficiency of antithrombin III. Apropos of a case]. / Phlébites récidivantes familiales par déficit en antithrombine III. A propos d'un cas.

The authors report the case of a young woman aged 29, mother of 2 children, presenting a post-phlebitic syndrome with hyperalgic capillaritic ulcer, a 10 year history of serious thrombophlebitic episodes with repeated pulmonary embolism despite heparin therapy, and reports of similar episodes in the family, some of which proved fatal. Haematological study showed a clear diminution in progressive antithrombin activity. A discussion follows on the clinical and haematological aspects of this constitutional and familial deficiency, which has been known since 1965 (Egelberg). The relative rarity of this condition should not prevent the routine search (by combined functional, immunological and chromogenic estimation) in the presence of repeated thrombosis, and when the thrombosis is biologically resistant to heparin : in such cases, the indication for long term treatment with vitamin K antagonists is undeniable.

Kasabach-kasabach-. / Problémes pratiques posés par deux cas d'hémangiomes géants avec syndrome de Kasabach Meritt

Giant haemangiomas combined with a haemostasis disorder of the Kasabach-Meritt type are rare and even more rare are the problems posed during labour when vulvo-vagino-uterine sites are involved. Two cases are reported. After summary of the clinical and biochemical studies, the authors report attempts to improve the haemostasis disorder, first with heparin and then with antifibrinolytic preparations, and to improve the angiomas with antifibrinolytic preparations in the hope of stimulating intrasacular thrombosis. In particular, the obstetric and surgical problems are discussed in the context of labour. In one case the outcome of labour was successful only moderate transfusion being required, and in the other the outcome was fatal despite the use of about 100 bottles of blood in the first 24 hours. In both cases the infants were normal.