Forty-five individuals with hepatosplenic schistosomiasis mansoni were studied with the aim of measuring levels of von Willebrand factor antigen (vWF:Ag), detecting abnormalities in platelet morphology and aggregation, and identifying changes to surface antigens. Haemograms, platelet aggregation tests, flow cytometry investigations of CD41/CD42b antibody and vWF:Ag assays were performed. Mean platelet counts were low (77,522/mm3) and 82.2% of patients presented thrombocytopenia. An inverse relationship between spleen size and platelet count was seen. Macroplatelets were found in 57.1% of patients, indicating good bone-marrow response, but were insufficient to compensate for the decrease in platelets due to splenomegaly. Decreased or absent platelet aggregation was seen in 50% of patients, probably due to low platelet counts. Markers for GPIIb/IIIa were normal in more than 90% of patients, not supporting the increased capture and destruction of platelets in the spleen that is hypothesized to occur with cirrhosis. Similar to cirrhosis, vWF:Ag levels were high or very high in 70.5% of patients. High levels of vWF:Ag were associated with platelet counts <100,000/mm3, larger spleen diameter and oesophageal varices. In conclusion, hepatosplenic schistosomiasis leads to a lower platelet count due to pooling in the spleen and, consequently, impaired aggregation, but not to increased capture and destruction of platelets in the spleen. High vWF:Ag levels probably promote stabilization of platelet microaggregates and prevent minor manifestations of thrombocytopenia such as petechiae, ecchymosis and gingival bleeding.

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Kabuki syndrome seems to have a relatively benign course. However, it has many aspects that remain to be cleared and, therefore, the possibility of its association with other condition that might be important for the anesthesiologist exists. The objective of this report was to alert for the risk of its association with latex allergy. For such, during the pre-anesthetic evaluation, a good anamnesis with analysis of predisposing factors other than the syndrome itself should be stimulated.

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Summary: Teixeira VC, Neves MA, Castro RA -Latex Allergy in a Patient with Kabuki Syndrome. Case Report. Background and objectives:The knowledge of anesthesiologists of specific aspects of patients with rare syndromes is a growing need since those patients are increasingly taken to the operating room. The objective of this report was to describe a case of latex allergy in a patient with Kabuki Syndrome, whose aspects have not been completely explained, alerting anesthesiologists for the possibility of this association.

Background: B-cell chronic lymphocytic leukemia (B-CLL) is characterized by the progressive accumulation of a neoplastic clone consisting of CD5/CD19/CD23/surface immunoglobulin cells. Despite this homogeneity in phenotype, B-CLL can follow either an indolent or a progressive course. CD38 is a transmembrane glycoprotein expressed on the surface of leukemic cells in a significant percentage of patients with B-CLL. Recent studies suggest that CD38 expression in CLL is a reliable prognostic marker, leading to an unfavorable clinical course with a more advanced stage of disease, poor responsiveness to chemotherapy and a shorter survival rate, along with others markers like ZAP-70 that reflect more accurately the mutagenic status of VH gene region, unfortunately not yet available in clinical practice routinely. Objectives: To assess the association of CD38+ expression with clinical and laboratory parameters at diagnosis in patients with B-CLL. Patients and Methods: CD38 expression was analyzed in 64 unselected newly diagnosed B-CLL patients from January 2003 to May 2005 seen at the Hematology Center of Pernambuco-Brazil (HEMOPE). According to Damle et al. patients with 30% or more B cells expressing CD38 were considered positive(CD38+), and those with less than 30% were considered negative(CD38−). Various patient characteristics were studied including age, sex, Binet stage, hemoglobin, ß2 microglobulin and lactate dehydrogenase levels in the serum. Statistical differences between each group (CD38+ vs CD38−) were analyzed using χ2 tests for categorical variables and Student’s t-tests for continuous variables. Results: Thirty-six patients (56.25%) were CD38+ and 28 (43.75%) were CD38-. Their median age at diagnosis was 64 years (range, 39–83) and the male to female ratio was 1.46:1. There were no differences between age, sex, hemoglobin and ß2 microglobulin levels in the CD38+ and CD38- groups. ß2 microglobulin level, however, was not available for assessment in all patients. Lactate dehydrogenase level was significantly higher in the CD38+ group (p=0.007). It was observed a trend toward a higher Binet stage (B or C) in the CD38+ compared with CD38- group (63.4 vs. 36.6% respectively), although not statistically significant (p=0.09) possibly because of the small number of patients studied. Conclusion: CD38 expression was associated with a higher lactate dehydrogenase level and a tendency for more aggressive clinical stage. CD38 evaluation is a measurable biological parameter that is not as subjective as the evaluation of some clinical parameters and should be considered a stantard clinical test for newly diagnosed B-CLL patients.