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Autoimmune Thrombocytopenia (autoimmune + thrombocytopenia)
Selected AbstractsAutoimmune thrombocytopenia: flow cytometric determination of platelet-associated autoantibodies against platelet-specific receptorsJOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 1 2005A. TOMER Summary., Immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by antibody-induced platelet destruction. Despite its clinical importance, the diagnosis of ITP is one of exclusion, thus, inevitably associated with potential difficulties. We here describe a feasible diagnostic method using the commonly available technique of flow cytometry. An antigen-specific assay for platelet-associated antibody was developed and tested in 62 adult patients with chronic ITP, 14 patients with thrombocytopenia of decreased production and 60 healthy controls. The method is based on flow cytometric (FCM) detection of autoantibodies reacting with specific platelet receptors immobilized on microbeads. The average fluorescence level in the ITP group calculated as a ratio to normal was 4.07 (range 0.8,31.0), in the non-ITP thrombocytopenic patients 0.9 (range 0.7,1.2), and in the healthy controls 1.0 (range 0.7,1.3). The average assay coefficient of variation was 0.218 [95% confidence interval (CI) 0.213, 0.221]. The difference between the ITP patients and both groups was highly significant (P < 0.001), using a stringent non-parametric analysis. A comparison of the FCM assay with the radioactive immunobead assay previously reported on the same cohort of patients showed significant correlation (R2 = 0.71, 95% CI 0.39, 0.53). The overall performance of the FCM assay in discriminating between ITP patients and normals was estimated by the receiver operating characteristic (ROC) plot, showing an area under the curve of 0.96 (maximal value 1.0), with standard error of 0.033. We conclude that the present FCM assay is clinically useful for routine diagnosis and follow-up of ITP. [source] Efficacy and safety of anti-D given by subcutaneous injection to patients with autoimmune thrombocytopeniaEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 1 2004Oliver Meyer No abstract is available for this article. [source] Replacement of intravenous administration of anti-D by subcutaneous administration in patients with autoimmune thrombocytopeniaPEDIATRIC BLOOD & CANCER, Issue S5 2006O. Meyer Abstract Intravenous (IV) administration of anti-D in patients with autoimmune thrombocytopenia (AITP) may result in severe hemolysis and even death. Over a 3-year period, we gave anti-D only subcutaneously (SC), and none of our patients have developed any acute adverse reaction. Most importantly, SC delivery of anti-D produces largely the same beneficial effect as obtained by IV anti-D. We recommend replacement of IV administration of anti-D by SC administration in AITP. Pediatr Blood Cancer 2006;47:721,722. © 2006 Wiley-Liss, Inc. [source] Multimodal management, including precisely targeted irradiation, in a severe refractory case of Evans syndromePEDIATRIC BLOOD & CANCER, Issue S5 2006Thomas D. Miale MD Abstract A challenging case of acute autoimmune thrombocytopenia (ITP) which evolved into a chronic refractory case of Evans syndrome over a period of more than 23 years is presented and may illustrate current therapeutic dilemmas now perplexing patients and clinicians. Newer modalities are being developed and their eventual role in the scheme of clinical management remains to be established. While this development unfolds, highly targeted radiotherapy was applied in this case to reduce platelet uptake by a refractory recurrent splenule with the goal of stabilizing the platelet count until promising investigational thrombopoietic agents or other newer, less toxic therapies might become available for wider application. Pediatr Blood Cancer 2006;47:726,728. © 2006 Wiley-Liss, Inc. [source] Polymicrogyria in a neonate with severe autoimmune thrombocytopenia: rare coincidence or related disorder?PRENATAL DIAGNOSIS, Issue 1 2007Enrico Lopriore No abstract is available for this article. [source] ZAP-70 expression is associated with increased risk of autoimmune cytopenias in CLL patients,AMERICAN JOURNAL OF HEMATOLOGY, Issue 7 2010Roberta Zanotti Autoimmune cytopenias (AIC) are frequent in chronic lymphocytic leukemia (CLL) patients, but risk factors and prognostic relevance of these events are controversial. Data about the influence on AIC of biological prognostic markers, as ZAP-70, are scanty. We retrospectively evaluated AIC in 290 CLL patients tested for ZAP-70 expression by immunohistochemistry on bone marrow biopsy at presentation. They were 185 men, median age 63 years, 77.9% Binet stage A, 17.6% B and 4.5% C. AIC occurred in 46 patients (16%): 31 autoimmune hemolytic anemias, 10 autoimmune thrombocytopenias, four Evans syndromes, and one pure red cell aplasia. Of the 46 cases of AIC, 37 (80%) occurred in ZAP-70 positive patients and nine (20%) in ZAP-70 negatives. ZAP-70 expression [Hazard Ratio (HR) = 7.42; 95% confidence interval (CI): 2.49,22.05] and age >65 years (HR = 5.41; 95% CI: 1.67,17.49) resulted independent risk factors for AIC. Among the 136 patients evaluated both for ZAP-70 expression and IGHV status, the occurrence of AIC was higher in ZAP-70 positive/IGHV unmutated cases (35%) than in patients ZAP-70 negative/IGHV mutated (6%) or discordant for the two parameters (4%; P < 0.0001). In ZAP-70 positive patients, occurrence of AIC negatively influenced survival (HR = 1.75; 95% CI: 1.06,2.86). The high risk of developing AIC in ZAP-70 positive CLL, particularly when IGHV unmutated, should be considered in the clinical management. Am. J. Hematol. 2010. © 2010 Wiley-Liss, Inc. 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