We report a patient in whom hepatosiderosis was diagnosed at the age of 55 years and who has since been treated by regular bleeding. The H63D mutation was found in the heterozygous state in the HFE gene. No mutation was recorded in the SLC11A3 gene (ferroportin). Hepatosiderosis did not seem primary, nevertheless its cause long remained elusive. Only 2 years ago did we find the responsible condition, a very mildly expressed form of dehydrated hereditary stomatocytosis (DHS). This genetic disease is a strongly iron-loading condition. Haemolysis was fully compensated. Kalaemia was slightly elevated, suggesting a pseudohyperkalaemia that may be associated with DHS. Osmotic gradient ektacytometry allowed to assess the diagnosis of DHS. The red cell monovalent Na+ and K+ concentrations were moderately elevated and reduced respectively. The temperature dependence of the ouabain + bumetanide-resistant K+ influx produced a shallow slope, above and parallel to the control curve. These features were consistent with the diagnosis of DHS. The pronounced hepatosiderosis contrasted with the mildly expressed DHS, and with the ferritinaemia that was slightly elevated, if at all, prior to bleeding. Bleeding caused ferritinaemia to decrease and hepatosiderosis to recede. The whole picture accounts for a misleading presentation of DHS, in which the primary condition long remained hidden behind one of its remotest complications, hepatosiderosis.

erythrocyte recovery; 82% and 24.3% for platelets and leukocytes, respectively; 97.8% for heparin).Cell-derived microparticles are, on average, smaller in diameter than platelets and range in size from about 100 nm to 1.0 m. Thus, the largest microparticles may overlap in size with platelets, which on average range in from 1.0 m to 5.0 m. Both erythrocyte-derived microparticles and platelet-derived microparticles were removed efficiently (97% and 99%; P ϭ 0.002 and P Ͻ .001, respectively). Interestingly, the efficiency of the Cell Saver device to remove platelet-derived microparticles as well as erythrocyte-derived microparticles was significantly increased compared with the efficiency to remove thrombocytes (P ϭ .019 and P ϭ .002, respectively; Wilcoxon signed rank test). Thus, cell-derived vesicles, which on average are smaller in diameter than thrombocytes, are removed more efficiently from blood by a Cell Saver device than thrombocytes in a clinical setting.This study is the first to directly evaluate the efficiency of a Cell Saver device to remove cell-derived microparticles from patient blood. Our data show that a Cell Saver device efficiently reduces the numbers of coagulation-and inflammation-promoting microparticles. From these data we cannot exclude that microparticles may bind to cells present within the blood or that low numbers of microparticles are generated by cell activation during the passage of blood through the Cell Saver device.

No abstract

No abstract

No abstract