[Association of thrombosis and thrombopenia induced by heparin. Treatment with a low molecular weight heparin].
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The first case of haemolytic anaemia with thrombocytopenia and acute renal failure induced by ingestion of aspirin in a 22-year old woman is reported. An IgM anti-aspirin antibody which agglutinated erythrocytes of the patient and of ABO compatible donors in the presence of aspirin was isolated in the serum. In addition, the allergic nature of the patient's hypersensitivity to aspirin was confirmed by positive lymphocyte transformation and basophil degranulation tests in the presence of the drug.
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The HLA typing of mothers alloimmunised against the PLA1 antigen of their thrombocytopenic neonate shows that the A1, B8, DR3 haplotype is involved. The very strong association found with DR3 (20/21) suggests that an immune response gene located in the D region of the major histocompatibility complex might be responsible for the ability to develop an anti PLA1 antibody.
Haemorrhage or thromboembolism during heparin therapy are usually attributed to a prescribing error. However, these clinical manifestations--especially thromboembolism--may occur with heparin therapy during severe thrombocytopenia. The authors describe the clinical, biological and physiopathological features characterising this thrombocytopenia with reference to 7 personal cases and a review of the literature. The incidence of heparin-induced thrombocytopenia varies between 0.5 and 1%. It seems to be more common (4%) during heparin therapy for thromboembolic disease. The thrombocytopenia appears 8 days after the onset of heparin therapy. It is characterised by the high incidence of thromboembolism (70% of cases) compared to haemorrhagic phenomena (10% of cases). Thrombocytopenia is asymptomatic in 20% of cases. The thrombocytopenia is peripheral, i.e. the bone marrow is normal, and isolated, i.e. there are no deficiencies in the factors of coagulation. One of our cases was of special interest because it was complicated by disseminated intravascular coagulation. Eight cases of disseminated intravascular coagulation have previously been reported. Analysis of platelet aggregation demonstrates the relationship between heparin and thrombocytopenia. Mixing the plasma of patients with thrombocytopenia and plasma rich in platelets in the presence of heparin lead to thrombo-agglutination. In contrast, in control and non-thrombocytopenic heparinised subjects, no reaction was found. These observations prove the existence of a platelet aggregant factor in the plasma during thrombocytopenia. This disappears 6 weeks to 2 months after stopping heparin. This platelet aggregant factor initiates platelet aggregation which is responsible for thrombocytopenia and for the initiation of phenomena of coagulation, so explaining the thromboembolic phenomena.(ABSTRACT TRUNCATED AT 250 WORDS)
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The authors report three cases of heparin-induced thrombocytopenia. Two cases were associated with deep venous thrombosis and pulmonary embolism and the other case was associated with arterial embolism. The clinical course was serious, with one death and one amputation of a limb. The thrombocytopenia developed suddenly, 10 to 15 days after the start of treatment; the platelet count was always below 100 000/mm3. The platelet aggregation test is an important diagnostic element, together with the rapid return to normal of the platelet count once the heparin is stopped. An anti-platelet antibody was detected in two patients, suggesting an immuno-allergic mechanism for this new complication of heparin treatment. The treatment consist of immediately stopping the heparin and replacing it with an anti-vitamin K drug or low-molecular weight heparin. The platelet count should be monitored systematically in all patients receiving heparin treatment for more than 8 days.
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Many pregnant women with AITP bear children with transient thrombocytopenia which is a potentially life threatening complication. Previous reports have demonstrated that monitoring of IgG circulating platelet antibodies may help with the management of these women. We report the case of a pregnant woman with AITP whose sera contained an IgG auto antiplatelet demonstrated by the fluorescein labelled antiglobulin techniques. Steroids had a beneficial effect with a return to a normal of the mother's platelet count but with no disappearance of the circulating antibody. Surprisingly the neonatal platelet count was normal and studies in the baby showed that the antibody had not crossed the placenta in detectable amounts. This case demonstrates our current inability to predict fetal thrombocytopenia and plan the mode of delivery.
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Possible relationships between Moschcowitz's disease and acute systemic lupus erythematosus are analyzed based on findings in one case. Initial exclusively buccal symptoms suggested a diagnosis of lupus, and then of Moschcowitz's disease leading to a fatal outcome. The main points discussed relate to diagnostic difficulties to enable distinction between the two diseases. A thrombotic thrombopenic purpura during the second phase of the disease showed a characteristic microangiopathy associated with a non-autoimmune hemolytic anemia and a schizocytosis: two forms of the affection are recognized, one with a chronic and one with an acute course. The latter, which has a fulminating progression leading to death, was that described in the case reported.
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Twenty cases of moderate thrombocytopenia in young women were examined in an assessment of the nosological status of this form. Parameters enjoying pathogenetic significance were distinguished in some instances: 4 cases displayed dysendocrinopathy, 2 the signs of consumption coagulopathy, 3 an antibody situation attributable to compensated autoimmune thrombocytopenia, and 3 a probable central inhibition of thrombocytopoiesis due to drugs. Since no significant cause could be determined in the remaining 8 cases, it can be said that this syndrome occupies a special place among the forms of chronic thrombocytopenia.