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[Surgical infections in patients suffering from haematological diseases].

Due to their systemic character, haematological diseases may interfere with erythro-, granulo- and thrombopoiesis, thus causing the onset of infections especially in cases of neutropenia and thrombopenia. Not only the defence mechanism of the individual is impaired by the basic disease but by the different therapeutic measures as well. Close cooperation between the haematologist and surgeon is of fundamental importance here.

Agranulocytosis↗

[Clinical staging and course of chronic lymphatic leukemia].

77 patients with chronic lymphocytic leukemia were clinically staged according to a staging system recently proposed by Binet and limited to 3 stages. The 49 patients with stage A disease (not more than 2 areas of palpable nodes or organs) had a median survival time of 141 months; the 17 patients with stage B disease (three or more involved areas) had a median survival time of 71 months and the 11 patients with stage C disease (anemia and/or thrombopenia) had a median survival of 37 months. With this simple three-stage system statistically significant differences of survival between the three groups of patients have been observed. This staging system is useful for the prognostic evaluation of patients with chronic lymphocytic leukemia.

Adult↗

[Thrombocytopenic thrombotic purpura in adults. 10 cases (author's transl)].

Thrombocytopenic thrombotic purpura is a rare condition of controverted pathophysiology. The authors have observed and treated 10 cases between 1974 and 1979. The clinical features were uniform: haemolytic anaemia with schistocytosis (10/10), thrombopenia (10/10), fever (9/10), varying neurological disorders (9/10) which required assisted ventilation in 7 cases. Renal insufficiency was found in 7 patients, but associated with renal lesions only one and never interfered with the course of the disease. All patients received corticosteroids in high dosage and anti-platelet aggregation drugs. That treatment alone cured 3 patients. In addition, 5 patients underwent plasmapheresis, which seemed to be very effective in 2 cases. Five patients died, 3 of whom despite corticosteroids, anti-aggregants and plasmapheresis. Thus, none of these treatments seems to be effective in all cases, and prospective multicentre therapeutic trials are probably required.

Adult↗

[Thrombocytopenic purpura in infectious mononucleosis (author's transl)].

A patient with infectious mononucleosis (IM) and thrombocytopenic purpura is reported. Clinical symptoms were not suggestive and diagnosis was based on the presence of lymphomonocytic cells in peripheral blood and serologic study. Bone marrow aspirate showed characteristic features of an immunologic thrombopenia, though antiplatelet antibodies were not found. Literature on this subject is revised and pathogenic and therapeutic problems are discussed.

Autoantibodies↗

[Asiatic dengue in France: 9 cases, of which one of the thrombopenic type (author's transl)].

Asiatic dengue was detected in nine tourists. In eight patients it was of the classical benign type, the ninth case being associated with severe thrombopenia (4 000/mm3), but without signs of shock. A brief summary is given of the physiopathology of the hemorrhagic forms of the disease: acquired sensitivity from a primary attack and reinfection with a heterologous virus; selection of virulent strains by an atypical vector; genetic recombination producing a strongly pathogenic virus.

Adult↗

[Leukoerythroblastosis as a manifestation of disseminated neuroblastoma in childhood].

A check is made of the hematologic disorders in 20 children with neuroblastoma and its relation with the stage of the tumour. The 75% of the patients had metastasis when they are examined, 70% anemia, 15% leukopenia, 20% thrombopenia and 50% leukoerythroblastosis. The patients with leukoerythroblastosis all had remote metastasis and made up 66% of the children with IV or IV-S stage. In 50% of the patients there was metastasis in the bone marrow and 80% of these had leukoerythroblastosis.

Anemia, Myelophthisic↗

[Chromosome 5q-- in the medullar cells of a patient with anaemia which later developed into acute non-differentiated leukaemia (author's transl)].

The authors report the observation of a 76-year-old man who since 1974 had a persistent anaemia considered as a pre-leukaemic state. The patient was hospitalized in May 1977 with fever and severe asthenia. The laboratory results indicated a probable diagnosis of acute non-differentiated leukaemia of stem cells. In spite of treatment, the anaemia grew worse, the leukocytosis accompanied by blast cells became more pronounced, a massive thrombopenia occurred and the patient died in irreversible shock. Cytogenetic examination done on a medullar culture revealed the presence in all the cells of a chromosome No. 5 with the long arms deleted : 46,XY,5q--. This rare medullar anomaly was reported for the first time in 1974-1975 by the Louvain school (Van den Berghe, Sokal, et al.) in a group of refractory anaemias. It has also been described in association with other chromosomal aberrations, in anaemias or other hemopathies which all developed into acute myeloblastic leukaemia. The clinical evolution and the cytogenetic data of the patient presented here are compared with those of other cases of 5q-- published in the literature, and the significance of this 5q-- chromosome aberration in hemopathies is discussed.

Aged↗

[Fluorouracil, mitomycin-C and adriamycin in the treatment of metastasizing gastrointestinal adenocarcinomas].

28 patients with advanced adenocarcinomas were treated with combinations of 5-fluorouracil and mitomycin-C (FM, 21 patients) or of 5-fluorouracil, adriamycin and mitomycin-C (FAM, 7 patients). 5 patients responded, 3 showing an objective response of more than 50% lasting 3-5 months. Toxicity was primarily hematologic, chiefly comprising severe thrombopenias. The results indicate that with these combinations a limited palliative effect can be achieved in only a few patients.

Adenocarcinoma↗

[Treatment of severe systemic lupus erythematosus. Long-term results in 55 patients (author's transl)].

Out of 55 patients with severe systemic lupus erythematosus (SLE) retrospectively studied from 1962 to 1979, 32 had diffuse proliderative glomerulonephritis and 23 had one or several extrarenal and/or haematological manifestations of the disease. All received corticosteroids in high dosage (0,8 to 1,5 up period was 48 months from the beginning of treatment. The actuarial survival rates for the whole groupe after 1,5 and 10 years were 92,4%, 83,4% and 77,2% respectively. Eight patients died: 5 of SLE and 3 of iatrogenic complications. Four are kept alive by maintenance haemodialysis. At the end of the study period, SLE was quiescent in 37 patients, including 14 who had discontinued corticosteroids for 5 to 72 months. The five-year survival rates were very similar in patients with renal and extrarenal involvement (86,4% and 81,6% respectively). In the latter group myocardial insufficiency, thrombopenia and thromboembolic complications were the main factors of morbidity and mortality. Corticosteroids were administered alone to 25 patients and in combination with cyclophosphamide to the remaining 30. Cyclophosphamide was given initially in 13 cases or subsequently on account of intolerance (7 cases) or resistance (10 cases) to steroids. Similar results were obtained with these two therapeutic regimens, but it must be noted that cyclophosphamide was mainly used in the more severe forms of SLE.

Adrenal Cortex Hormones↗

[Acquired coagulation inhibitors. 10 cases (author's transl)].

A circulating anticoagulant with antiprothrombinase activity was detected in 10 patients, 4 of whom had systemic lupus erythematosus. Clinically, haemorrhages occurred only in patients with associated thrombopenia; some developed thrombosis. Recalcification time and activated partial thromboplastin time were prolonged in the patients' plasma and in mixed patients' and control plasma. In 5 cases the anticoagulant was isolated by chromatography as IgG or IgM.

Adolescent↗

[Ticlopidine in heart surgery with cardiopulmonary by-pass (author's transl)].

An interesting potential use of platelet aggregation inhibitors lies in the prevention of the thrombotic process originating in the oxygenator of the cardiopulmonary by-pass apparatus used in cardiac surgery. A 5-day course of ticlopidine HCl immediately before surgery results in a significant reduction in the thrombopenia usually observed and attributed to platelet aggregation and adhesion to the apparatus. Biological investigations have failed to show interference of the drug with coagulation factors, and no unwanted effect on per and post-operative bleeding was observed. Cardiopulmonary by-pass appeared in this study as an excellent model to test the clinical effectiveness of anti-aggregation compounds.

Adolescent↗

[Temporal evolution of fibrinogen and platelets in reaction to various stressors in coronary and non-coronary subjects (author's transl)].

Twenty-one postmyocardial infarction patients are compared with twenty-one matching non-coronary subjects. Fibrinogen level as well as whole blood platelet count were measured six times for each subject in a timespan of 2 hours, during which two stress situations are induced. Throughout the experiment, non-coronary subjects show significantly lower levels of fibrinogen and higher blood platelet count. These two variables follow a specific temporal evolution. Stressfull situations are directly related with a significant decrease in the whole blood platelet count. Films inducing a directional anxiety match a lower blood platelet count, as compared to films inducing a diffuse anxiety. A link may be hypothesized between the stress-induced decrease in blood platelet count and the relative thrombopenia of coronary patients.

Anxiety↗

[Prenatal diagnosis of upper limb malformations].

Three children with thrombopenia and radial aplasia were examined at the Centre de Génétique Médicale of Marsielles from 1974 to 1976. In each case the genetic basis of the anomaly was discussed with the family at genetic counseling underlining the high risk of recurrence related to the autosomal recessive transmission. Each proband was the only child of the couple. In the first case the father and mother, each having one child from a previous marriage, decided to have no more children. In the second case, the use of fetoscopy and its limitations were discussed as another pregnancy was desired. However, the parents finally refused the procedure during pregnancy which yielded a second child with phocomelia who did not survive. In the third and most recent case the proband died. Since the young couple strongly desired another child, ultrasonic monitoring of pregnancy was cautiously suggested and its limitations clearly exposed. Echography was regularly repeated from the third month of pregnancy. The forearms were never detected on echography, nor on fetal radiography. Pregnancy was thus interrupted with prostaglandins at 21 weeks gestational age. On examination the fetus presented bilateral aplasia of the forearm. Based on these observations, the authors discuss the problems raised by prenatal diagnosis of upper limb malformations.

Ectromelia↗

[Psychiatric drugs as risk factor in fatal heat stroke].

Two men aged 33 and 31 years suffered a fatal heat stroke on a warm summer day. One of them used pimozide and clomipramine, the other zuclopenthixol, dexetimide, droperidol, promethazine and propranolol as psychiatric medication. Both of them had a body temperature > 42.3 degrees C, without perspiring. At first only a comatose situation with practically normal laboratory values existed; this was rapidly followed by massive liver damage, disseminated intravascular coagulation, anaemia, thrombopenia and acute renal failure. In spite of adequate and rapid treatment these complications were fatal. Both patients used medication with an antidopaminergic and anticholinergic (side) effect. The set point of the temperature regulation centre can be elevated by the antidopaminergic activity of antipsychotics. Use of anticholinergic medication can disturb the thermoregulation via inhibition of the parasympathicomimetically mediated sweat secretion. It is recommended to point out the danger of unusually high outdoor temperatures to patients using this medication.

Adult↗

[Gold salt alveolitis in 3 patients with rheumatoid arthritis].

BACKGROUND: When the characteristic symptoms for an interstitial pulmonary disease arise in patients with rheumatoid arthritis, a drug-induced alveolitis should be considered in the differential diagnosis. In such cases, the administration of the drug and gold salts should be stopped. PATIENTS AND METHODS: The cases of three patients with rheumatoid arthritis (RA) who had been treated with gold salts for 2 months (A), 23 months (B), and 36 months (C) are presented. The total dose of sodium aureothiomalate amounted to 280 mg for patient A, 1150 mg for patient B, and 2190 mg for patient C. Clinical signs, X-rays of the lungs, pulmonary function tests, and laboratory tests were evaluated for the three patients while, for patient A BAL as well as provocation tests were additionally performed before and after therapy. In this case, the histological picture of the lungs is presented; biopsies were taken during the first BAL. RESULTS: The clinical complaints of all 3 patients were similar, with the alveolitis being observed as diffuse in one case and above all in the upper regions in two cases on radiology. This led to differing degrees of diffusion disorders in the lungs. In patient A, the diagnosis was made in the stage of progressive fibrotic alveolitis and was treated with D-penicillamine. All 3 patients received steroids over 3-6 months and the gold salts were stopped. Because of the long duration and doubtful differential diagnosis for patient A with either rheumatoid lung or gold salt alveolitis, a provocation test with sodium aureothiomalate was performed. All 3 patients had blood eosinophilia while, in case A, a thrombopenia was also found. CONCLUSIONS: A gold salt alveolitis can occur as a side effect of gold salts in addition to skin vasculitis and hematological disorders. When the gold salt administration is not stopped a fibrotic alveolitis can develop. The provocation test can be diagnostically useful to distinguish between a rheumatoid lung and gold salt alveolitis.

Aged↗

[HELLP syndrome. Two cases observed at the University Hospital Center of Brazzaville (Congo)].

The HELLP syndrome is almost always encountered in patients with gravid hypertension. It is defined as the association between intravascular haemolysis, raised liver transaminase levels and thrombopenia. We report 2 cases of HELLP syndrome observed in the Gynecology-Obstetrics B ward in the Brazzaville (Congo) University Hospital in 1992. Frequency in the ward was estimated at 2.9% (2 cases among 67 patients hospitalized for pre-eclampsia). The two cases occurred at 29 weeks gestation in young, black, primipartants in a context of severe pre-eclampsia. The clinical course was rapidly favorable for the mother after delivery. One in utero fetal death occurred. Due to the number of maternal deaths recorded in the literature, we suggest that, in Africa, pregnancies should be interrupted in case of HELLP syndrome whatever the gestational age since proper medical facilities are need to care for these severe cases.

Abortion, Therapeutic↗

[Calcium nadroparin in the prevention of thromboembolic disease in elderly subjects. Study of tolerance].

OBJECTIVE: To evaluate the safety of a low molecular weight heparin for the prevention of thromboembolic disease in elderly patients. METHODS: A multicentre, randomized, open study was conducted in 295 patients: 146 patients (mean age 82.8 +/- 0.5 years) received calcium nadroparin and 149 patients (mean age 83.8 +/- 0.6 years) received calcium heparin. All medical patients were hospitalized with transient locomotor disability, requiring prophylaxis for thromboembolism. Patients were randomized to 28-days treatments with either subcutaneous calcium nadroparin 0.3 ml (3075 anti-Xa IU) daily or subcutaneous calcium heparin twice a day (0.2 ml-5000 UI--if bodyweight was less than 70 kg or 0.3 ml-7500 IU--if bodyweight was over or equal to 70 kg). RESULTS: The overall objective was to assess tolerability in terms of premature discontinuation of treatment for any reason: Discontinuation was more frequent in the calcium heparin group (16.1%) than in the calcium nadroparin group (11%), but without statistical significance. Premature withdrawal of therapy definitively attributable to drug administration occurred in 10 patients from the calcium heparin group (1 deep vein thrombus, 1 pulmonary embolus, 4 major bleeding events, 1 thrombopenia, 3 allergic events) and in only 1 from the calcium nadroparin group (1 allergic event). The difference was statistically significant (p = 0.01). CONCLUSIONS: The results of this study offer new evidence to confirm the safety and simplicity of administration of calcium nadroparin in the prevention of thromboembolism in elderly patients.

Aged↗