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Surface phenotype and immunoglobulin levels in B-cell chronic lymphocytic leukaemia.

The aim of the present study is to analyze the relationship between serum immunoglobulin (Ig) levels and the immunological phenotype and the clinicohaematological features of B-cell chronic lymphocytic leukaemia (B-CLL) in a series of 126 patients. Eighty-eight of the cases (70%) had a decreased concentration of at least one Ig. IgM and IgA were the most frequently decreased (60% and 49% respectively). A serum monoclonal gammapathy was found in 4 patients, Ig M/k in two cases and IgM/l and IgG/k in one case. Patients with hypogammaglobulinaemia had a similar surface phenotype as patients with normal Ig levels (MRFC+, sIg+, CD20+, HLA/DR+, FMC7-, CD5+, CD9+). On the other hand the cases with hypogammaglobulinaemia displayed the features of a more advanced disease, a higher incidence of organomegalies (p less than 0.05), anaemia and/or thrombopenia (p less than 0.05), a diffuse bone marrow pattern (p less than 0.05), advanced clinical stages (p less than 0.05) as well as higher levels of both peripherial blood (p less than 0.02) and bone marrow lymphocytosis (p less than 0.02). These findings suggest that the presence of hypogammaglobulinaemia in B-cell patients is probably more related to a higher tumor burden than to either certain stages of B-cell differentiation or a particular cellular phenotype.

Agammaglobulinemia↗

[Combined chemotherapy with cisplatin in esophageal cancer].

Combined chemotherapy with cisplatin was performed in patients with advanced esophageal cancer. Two types of administration schedule were used: method I (three-drug combination of cisplatin, bleomycin and methotrexate) and method II (combination of cisplatin, peplomycin and methotrexate). Of 16 cases, 6 (37.5%) showed partial remission. With regard to the method of administration, the response rate for method I was 33%, and that for method II was 43%. Nausea (84%), vomiting (56%), loss of appetite (94%), malaise (75%) and alopecia (25%) were observed as side effects. Nausea and vomiting were ameliorated by use of metoclopramide. In bloodchemistry, anemia (87%), leukopenia (56%), thrombopenia (31%) and increase of BUN (63%) were observed. However, these changes were ameliorated by hydration or blood transfusion. Combined chemotherapy with CDDP should be a more useful future treatment for esophageal cancer.

Aged↗

[Antiphospholipid antibodies in systemic lupus erythematosus: incidence, significance and relation to lupus nephritis].

The prevalence of three different types of antiphospholipid antibody in 88 consecutive patients with systemic lupus were 27.2% for lupus anticoagulant (LAC), 31.8% for anticardiolipin antibody (aCL), and 13.6% for falsely positive serologic tests for syphilis (FPSTS). The three tests were correlated, thus confirming the overlapping specificities of this family of antibodies. Although FPSTS was not associated with any particular manifestation of systemic lupus, aCL correlated with thrombosis (p = 0.0001), thrombopenia (p = 0.009), neuropsychiatric features (p = 0.02) and membranous nephropathy (p = 0.001), while LAC correlated with thrombosis (p = 0.001) and hemolytic anemia (p = 0.04). The previously unreported association between membranous nephropathy and aCL might explain some features of the former, particularly the higher incidence of thromboembolic complications and the poorly known relation with renal vein thrombosis.

Adolescent↗

[Renal cortex ischemia, right atrial thrombosis and coronary occlusion in anti-phospholipid antibody syndrome].

The presence of a lupus anticoagulant (LA) is paradoxically associated with a high incidence of arterial and venous thrombosis. In a patient with a lupus-like systemic disease, having received phenindione for 11 years, LA was discovered in association with recurrent deep venous thrombosis, a right atrial thrombus, coronary occlusion, arterial hypertension, thrombopenia, and anticardiolipin antibodies without anti-DNA antibodies. Renal cortical ischemia was detected by a tomographic scan. Renal biopsy showed glomerular ischemia and diffuse interstitial fibrosis. After a one-year anticoagulant and steroid therapy, LA has disappeared despite a high level of anticardiolipin antibodies, and renal function remains normal.

Autoantibodies↗

[Outbreak of hepatitis A in day nursery: diagnosis and follow-up in a pediatric clinic].

We describe one of four epidemic known outbreaks of type "A" hepatitis during the year 1987 in Jaén, and whose focal point was a day nursery placed in our sanitary area. We identify 75 cases; we followed 48 from a pediatric surgery (PS), 33 of them were from a day nursery (attack rate, 31.73%). To the 48 children from PS, antibodies IgM and IgG against type "A" hepatitis virus were detected. No case was treated with drugs and in every case it was suggested that hygienic rules be observed and a knowledge of the disease be given to the parents. It had a good evolution in all cases, and only two of them were hospitalized, one of the parents' request and the other with thrombopenia. The remaining cases till 75, were diagnosed in other surgeries (OS) and in Hospitals (H).

Adolescent↗

[Parvovirus infections in children with hemolytic anemia].

Acute episodes of erythroblastopenia in children with chronic hemolytic anemias have been recognized for a considerable length of time. In 1981, a small virus with a single strand of DNA, parvovirus B 19, was identified as the causative agent in most such episodes. Other diseases have been ascribed to parvovirus B 19, including erythema infectiosum or fifth disease, polyarthralgia, fetal death. Schonlein-Henoch disease, and bone marrow aplasia. In acute attacks of erythroblastopenia, anemia is the most prominent manifestation, but coexistence of neutropenia and thrombopenia has been reported. Hematologic disorders last for approximately ten days. The diagnosis of recent parvovirus B 19 infection rests on detection of specific IgM antibodies, or direct visualisation of the virus by electron microscopy. More recently, detection of the viral genome using molecular hybridization techniques has been achieved. In vitro studies have confirmed the inhibiting effect of parvovirus B 19 on red cell line progenitors, particularly CFU-E, but the exact mechanism of the inhibition remains uncertain. Other diseases due to parvovirus B 19 or other parvoviruses probably remain to be discovered.

Anemia, Hemolytic↗

[Pilot combination phase II study of mitomycin C plus cisplatin for non-small cell lung cancer].

Fifteen patients (six patients with adenocarcinoma, seven patients with squamous cell carcinoma, and two patients with large cell carcinoma) with advanced non-small cell lung cancer (NSCLC) were evaluable for mitomycin C (MMC; 8 mg/m2 day 1, 8, every 3-4 weeks) plus cisplatin (CDDP; 80 mg/m2 day 1, every 3-4 weeks). Ten patients had had prior chemotherapy. Among 15 evaluable patients, no patient achieved complete response, and two patients showed partial response. The response rate of MMC plus CDDP against NSCLC was 13.3%. Toxic effects included anorexia (80%), nausea and vomiting (67%), leukopenia (53%), anemia (47%), nephrotoxicity (47%), thrombopenia (27%), liver injury (27%), and fever (7%). These toxic effects were reversible and manageable. The combination of MMC and CDDP appears to be valuable regimen against advanced NSCLC.

Aged↗

[AIDS in elderly patients. Apropos of 22 cases observed in the Paris region].

If geriatric AIDS is defined as the occurrence of this disease in individuals over 60 years old, it represents about 5% of the cases reported to the Direction Générale de la Santé by the end of 1988. We retrospectively analyzed 22 clinical cases of geriatric AIDS observed between 1985 and 1987, i.e. 21% of the cases reported at that time. In 55% of them, infection resulted from contaminated blood transfusions. Initially, hospitalization was indicated due to a significant deterioration of the patient's general condition, however, neuropsychiatric disorders and intellectual degeneration were present in 18% of the cases. Biological anomalies at the time of admission were classical with the exception of a high frequency of leukopenia often associated with anemia or thrombopenia. During the evolution of the disease, opportunistic infections were very common (90% of the cases). However, the major characteristic of this clinical form of AIDS is the high percentage (55%) of patients suffering from major neurological and psychiatric disorders, including subacute encephalitis in more than half of these patients. The prognosis is very bad, with an average survival time of 4.3 months from the time of diagnosis (median 2.5 months). The cumulative effect of the delay in diagnosing AIDS during the first hospitalization and the classical seriousness of encephalitis suffice to explain the very poor prognosis in patients whose age can play a role in altering the immune response.

AIDS Dementia Complex↗

[Imported falciparum malaria in Marseilles hospitals in 1987. Apropos of 104 cases].

Patients were composed of 85 adults and 19 children; 70 were European, 23 Comorian; malaria was contracted by 9 patients in French Guyana, 60 in Africa, 23 in Comoro Islands; prophylaxis was correct for 45 patients (nearly all of the cases with chloroquine) when the first symptoms occurred. Every case of malaria appeared during the month following their return from an endemic area. Fever was often moderate or intermittent, altered by prophylaxis and previous treatments. Some patients had a clinical profile of "visceral evolutive malaria" and 3 a cerebral malaria. The most frequent biologic alteration was thrombopenia (40 times under 100,000/microliters). No relation between parasites density and clinical profile has been identified. The sensitivity of the strains for antimalarial drugs has been studied 35 times: 28 strains were chloroquine resistant, 3 have a decreased sensitivity for quinine. Most of the resistant strains came from Central Africa. Two patients died.

Adolescent↗

[Postoperative pulmonary embolism].

Post-operative thrombo-embolic disease remains a frequent occurrence in spite of advances in their prophylaxis. Evaluation of 60 case-reports of this disease which often includes peripheral manifestations and always pulmonary manifestations, enables to specify the role of the procedure itself (mostly orthopaedic surgery 60%), pelvic surgery 20%, the chronology of events (possibility of early embolism between D1 and D3 and usual occurrence of manifestations between D8 and D18, and the importance of the background, whether investigated or not: deficiencies in anti-thrombin III, protein C and S: 4 cases. The diagnosis is based on clinical signs (non-specific) and the laboratory tests, especially scintigraphy (screening) and angiography, absolutely necessary for the diagnosis and evaluation of the amputation coefficient (Miller index). With a diagnosis of pulmonary embolism, it is always necessary to look for a proximal venous thrombosis. The treatment, calls for heparin (quite seldom), thrombolytics (Urokinase, Plasminogen in our experience), the indication of which must take into consideration the delays and the nature of the previous procedure, and finally surgery (massive forms where thrombolytics are contraindicated). The thrombo-embolic manifestations with thrombogenic thrombopenia secondary to heparin are quite frequent, in a surgical environment (10 cases) and difficult to treat.

Humans↗

[Human monocytic colony-stimulating factor].

Human monocytic colony-stimulating factor (hM-CSF) is a glycoprotein which stimulates monocyte production in the bone marrow. It enhances CSF (such as G- and GM-CSF) production of monocytes and megakaryocyte-potentiating activity (Meg-POT). It also enhances tumor-killing activity of monocytes against several leukemic cell lines such as K562, U937, HL60 and Daudi. In the clinical studies, it was shown that hM-CSF infusions accelerated the recovery from neutropenia as well as thrombopenia after anticancer chemotherapy against hematological, gynecologic and urogenital malignancies. Human M-CSF infusions were tolerable without any serious side effects. It is reported that infusions of G-CSF and GM-CSF cause the increment of leukemic cell counts in some cases, but hM-CSF infusions did not increase leukemic cell counts. These results indicate that hM-CSF may be potentially useful for the treatment of myelosuppression induced by cancer chemotherapy in cancer patients.

Adult↗

[Hepatitis caused by clometacin (Dupéran). Retrospective study of 30 cases. A model of autoimmune drug-induced hepatitis?].

Thirty cases of clometacin-induced hepatitis were retrospectively collected over a nine-year period in hepatogastroenterological units of non university, public hospitals. There was a strong female predominance (90 percent). Clometacin (Dupéran) was taken because of arthritis in 8 out of 10 cases. Administration was continuous in 85 percent of cases and median duration was 445 days. median dose was 450 mg per day. Jaundice, fatigue, and weight loss were the most frequent symptoms, but edema, ascites and palmar erythema were not uncommon. Thrombopenia (38 percent) was the most frequent hematologic abnormality. Renal failure, always with benign course, was present in 1/4 of cases. Biochemical disorders indicated hepatocellular and cholestatic hepatitis in 3/4 and 1/4 of cases respectively. Hypoprothrombinemia below 50 percent was noted in 1 out of 6 cases, and was associated with death in half cases. Gamma-globulins were increased in 80 percent of cases, with a predominant increase of IgG. Antinuclear or anti-smooth muscle antibodies were present in 60 percent of cases, whereas antimitochondrial and antimicrosomes were absent. Histopathological examination of the liver biopsy specimens obtained in 25 patients showed acute hepatitis in 8 and chronic active hepatitis with fibrosis in 17--including 6 patients with cirrhosis; there were no epidemiological, clinical (except ascites), or biochemical differences between these two groups. Four of the 7 patients tested had HLA B8 antigens; they all had chronic active hepatitis, with autoantibodies in 3 cases. Median duration of hospitalization was 21 days. Hepatitis was directly responsible for death in 3 patients; biochemical sequelae (hypergammaglobulinemia or anicteric cholestasis) were present in 8 patients, 2 of whom most likely had cirrhosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

[Necrosis of the bone marrow and cancer].

Bone marrow necrosis (BMN) is a rare complication and is characterized by the presence of an eosinophilic amorphous material in the bone marrow. The clinical, analytical and histological characteristics of 4 patients with BMN associated to a neoplastic process are described. One of them was a gastric cancer but the neoplastic origin could not de determined in the other three cases. Three patients presented bone pain, but in all four patients thrombopenia, anemia, leuko-erythroblastic reaction and elevated LDH was found. A literature review is carried out and the possible physiopathological mechanisms are discussed.

Adult↗

[Zidovudine. The current status of its evaluation].

3'-azido-3'-deoxythymidine is a thymidine analogue with an in vitro as well as in vivo efficacy towards HIV-mediated infection. Zidovudine exerts its action, following an intracellular three-step phosphorylation, through viral reverse transcriptase inhibition. Its half-life is approximately one hour. Oral biodisponibility is 65%, and passage through blood-brain barrier results in therapeutic levels is CSF. Clinical evaluation has enabled demonstration of a beneficial effect on survival of stage IV AIDS patients, when treated after a PCP episode. In this setting, aggregate survival ratios reach 73% after one year of follow-up, and 41% after 2 years. In addition, zidovudine activity has been demonstrated in treatment of HIV-induced thrombopenias as well as HIV-related central nervous system disorders. Presently, zidovudine therapeutic evaluation proceeds through the following main axes: dosage tuning (either by lowering of standard dose, and/or dose interval modification); combination with other antiviral therapies; lastly, patient treatment et an early stage of disease.

Acquired Immunodeficiency Syndrome↗

[Splenectomy in Gaucher's disease. Apropos of 2 cases, one of which was preceded by embolization].

Two cases of Gaucher's disease type I are reported Splenectomy was indicated because of hypersplenism and massive splenomegaly. In one case hypersplenism was treated with pre-operative selective embolization because of the volume of the spleen (20 kg). The embolization corrected the thrombopenia but not the size of the spleen. Four years after operation for case 1 and eight months for case two, there is an improvement in the clinical status.

Child↗

[Complications in Mediterranean boutonneuse fever: prospective study of 78 patients].

A prospective study of the clinical evolution of 78 patients diagnosed of mediterranean botonous fever has been performed. Hospital admissions because of complications were necessary in 5 (6,4%) patients. 13 patients (16,6%) had a devious evolution with signs of severe involvement of organs and systems. The evolution was favorable in the remaining 60 cases (79,9%) on home treatment with doxycycline. The unfavorable evolution was associated with advanced age, underlaying chronic disease, and the delay in the initiation of the appropriate treatment. Previous thrombopenia was the major predictive biological factor of unfavourable evolution in our patients.

Adolescent↗

[Chemical characterization and biological activity of the immunologically active substances in Juzen-taiho-to (Japanese kampo prescription)].

Juzen-taiho-to (TJ-48) is prepared by extracting a mixture of ten kinds of medicinal plants. This prescription has long been used traditionally against anemia, anorexia, extreme exhaustion and fatigue. TJ-48 may now provide new advantages with little toxicity in combination with chemotherapy or radiation therapy, and promising results have actually been obtained in terms of preventing leukemia in cancer patients who have taken antitumor agents. The combination of TJ-48 and mitomycin C (MMC) produced significantly longer survival in p-388 tumor-bearing mice than MMC alone, and TJ-48 decreased the diverse effects of MMC such as leukopenia, thrombopenia and weight loss. However, mechanisms of the pharmacological action are still unclear. One of the possible mechanisms of the action of TJ-48 may be some effects on immune responses. Therefore we studied the effects of TJ-48 on immune response in mice and characterization of immunologically active substances. TJ-48 augmented antibody production and activated macrophage by oral administration of TJ-48, but reduced the MMC-induced immunosuppression in mice. TJ-48 showed a mitogenic activity in splenocytes but not in thymocytes, and an anti-complementary activity was also observed. Anti-complementary activity and mitogenic activity were both observed in high-molecular polysaccharide fraction but not in low-molecular weight fraction. Of several polysaccharide fractions in TJ-48, only pectic polysaccharide fraction (F-5-2) showed potent mitogenic activity. F-5-2 was also shown to have the highest anti-complementary activity. However, the polygalacturonan region is essential for the expression of the mitogenic activity, but that the contribution of poly-galacturonan region to the anti-complementary activity is less. F-5-2 activates complement via alternative complement pathway and induces the proliferation of B cells but does not differentiate those cells from antibody producing cells.

Adjuvants, Immunologic↗

[Infection caused by gram-positive and gram-negative bacteria. A comparative study].

The clinical symptomatology of bacterial septicemias was analysed in 417 patients of a University Hospital in West Berlin. Sepsis was caused by Gram-negative organisms in 229 cases, and by Gram-positive bacteria in 177 cases; 11 cases presented with a mixed type sepsis involving both Gram-positive and Gram-negative pathogens. With the exception of a drop in blood pressure, observed appreciably more often in Gram-negative infections (42.4% of the cases as compared with only 25.4% in the case of septicemia due to Gram-positive organisms, (p less than 0.01), no significant clinical differences were seen between Gram-negative and Gram-positive sepsis. Pathophysiological changes (thrombopenia, leukopenia, coagulopathies) that are considered classical reactions to endotoxin, were also observed in Gram-positive infections. The overall prognosis of septicemia was determined largely by the severity of the underlying pathological condition.

Bacterial Infections↗