words count=192 Text words count=3125 Tables=3 Figure=4 ABSTRACT Autoimmune cytopenias (AIC) are ... more words count=192 Text words count=3125 Tables=3 Figure=4 ABSTRACT Autoimmune cytopenias (AIC) are frequent in 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 males, median age 63 years, 77.9% Binet stage A, 17.6% B and 4.5% C. AIC occurred in 46 patients (16%): 31 autoimmune haemolytic anaemias, 10 autoimmune thrombocytopenias, 4 Evans syndromes and 1 pure red cell aplasia. Of the 46 cases of AIC, 37 (80%) occurred in ZAP-70positive patients and 9 (20%) in ZAP-70negatives. ZAP-70 expression (HR=7.42; 95%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 for ZAP-70 expression and IGHV status the occurrence of AIC was higher in ZAP-70positive/IGHVunmutated cases (35%) than in patients ZAP-70negative/IGHVmutated (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).
Two sequential lymph node biopsies taken from a non-Hodgkin lymphoma patient revealed two karyoty... more Two sequential lymph node biopsies taken from a non-Hodgkin lymphoma patient revealed two karyotype abnormalities peculiar to B cell neoplasias: trisomy 12 and t(2;8)(p12;q24) translocation. The first was documented in all cells analyzed, while the second was present in 20% of the metaphases from the first biopsy and in 100% from the second. This suggests that the t(2;8) translocation arose as a secondary karyotypic change. In addition, although immunological characterization of the neoplastic cells disclosed a monoclonal B cell population that expressed immunoglobulin kappa light chains, as usually found in Burkitt's lymphoma with t(2;8) translocation, Southern blot analysis provided evidence of rearrangement in only one kappa chain allele.
words count=192 Text words count=3125 Tables=3 Figure=4 ABSTRACT Autoimmune cytopenias (AIC) are ... more words count=192 Text words count=3125 Tables=3 Figure=4 ABSTRACT Autoimmune cytopenias (AIC) are frequent in 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 males, median age 63 years, 77.9% Binet stage A, 17.6% B and 4.5% C. AIC occurred in 46 patients (16%): 31 autoimmune haemolytic anaemias, 10 autoimmune thrombocytopenias, 4 Evans syndromes and 1 pure red cell aplasia. Of the 46 cases of AIC, 37 (80%) occurred in ZAP-70positive patients and 9 (20%) in ZAP-70negatives. ZAP-70 expression (HR=7.42; 95%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 for ZAP-70 expression and IGHV status the occurrence of AIC was higher in ZAP-70positive/IGHVunmutated cases (35%) than in patients ZAP-70negative/IGHVmutated (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).
Two sequential lymph node biopsies taken from a non-Hodgkin lymphoma patient revealed two karyoty... more Two sequential lymph node biopsies taken from a non-Hodgkin lymphoma patient revealed two karyotype abnormalities peculiar to B cell neoplasias: trisomy 12 and t(2;8)(p12;q24) translocation. The first was documented in all cells analyzed, while the second was present in 20% of the metaphases from the first biopsy and in 100% from the second. This suggests that the t(2;8) translocation arose as a secondary karyotypic change. In addition, although immunological characterization of the neoplastic cells disclosed a monoclonal B cell population that expressed immunoglobulin kappa light chains, as usually found in Burkitt's lymphoma with t(2;8) translocation, Southern blot analysis provided evidence of rearrangement in only one kappa chain allele.
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