1/5. Alterations of blood group antigens in angio-immunoblastic lymphadenopathy with dysproteinemia.Antigen analysis of the red cell membrane in a patient with angio-immunoblastic lymphadenopathy with dysproteinemia (AILD) with red cell autoantibody revealed that four blood group antigens had been acquired. These four antigens consisted of S of MNSs blood group, Lua of Lutheran blood group, and K and Kpa of Kell-Cellano blood group. These antigens disappeared and the Coombs' test became negative after complete remission induced by combination chemotherapy. Free amino acid analysis after Dispase treatment of red cell membrane suggested that the antigenic modifications were associated with abnormal composition of amino acids.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/5. antithrombin iii Padua: a "new" congenital antithrombin iii abnormality with normal or near normal activity, normal antigen, abnormal migration and no thrombotic disease.A "new" antithrombin iii abnormality is described in four members of a family. The proposita is a 38 years old female who showed no thrombotic disease and the following laboratory pattern: normal routine clotting tests, normal or near normal AT III activity (chromogenic substrates S-2238 and Chromozym Th) both in plasma and in serum and in the presence or absence of heparin, slightly decreased antifactor Xa activity (chromogenic substrate S-2222), normal progressive antithrombin, normal AT III antigen but abnormal migration in the agarose-heparin bidimensional system. In the latter test, one major abnormal peak, less anodal than the normal counterpart, and a smaller, apparently normal peak, were seen. In agarose without heparin the pattern was similar to normal both in plasma and in serum. heparin tolerance to heparin in vivo and in vitro was slightly increased but still within normal limits. The two sons and a paternal aunt showed the same pattern. The hereditary pattern seems therefore autosomal dominant. The abnormality described appears different from AT III Budapest. The toponym of antithrombin iii Padua is proposed to define this peculiar abnormality.- - - - - - - - - - ranking = 0.625keywords = antigen (Clic here for more details about this article) |
3/5. Multiple clonally-restricted immunoglobulins in human sera: disease associations.Diseases associated with multiple clonally-restricted serum immunoglobulin (Ig) abnormalities present at levels which, in most cases, preclude detection by conventional immunoelectrophoresis, were studied using methods for detection and characterization of homogeneous Ig that are approximately equal to 40 times more sensitive than either cellulose acetate zone electrophoresis or immunoelectrophoresis. patients with these Ig abnormalities had a high incidence of infectious disease (29% of total cases), malignancy (19%), connective tissue disease (14%) and liver disease (10%). The concentration of individual clonal products was found to wax and wane, but it could not be determined whether these clonally-restricted Ig species represent, wholly or in part, the products of dominant antibody-producing plasma cell clones involved in the patients' response to their disease. We conclude that multiple homogeneous serum Ig abnormalities occur in clinical situations where heightened antigenic stimulation and/or immune reactivity are thought to occur (e.g., infections, malignancies and autoimmunity). Laboratory evaluation of these Ig abnormalities could be useful for diagnostic and/or therapeutic monitoring purposes in situations where the specificity of the clonally-restricted Ig species can be established.- - - - - - - - - - ranking = 0.125keywords = antigen (Clic here for more details about this article) |
4/5. A case of slow variant bisalbuminmia: study of its composition in amino acids.The authors report an observation of slow variant bisalbuminemia. After electrophoretic separation of two varieties, the amino acid chromatographic analysis has permitted them to show that in the slow variant the molecule of albumin has undergone a substitution of several glutamic acid residues by lysine and of several alanine residues by proline. Such an alteration poses the problem of its effect on the antigenic specificity of the molecule and that of its mechanism of production.- - - - - - - - - - ranking = 0.125keywords = antigen (Clic here for more details about this article) |
5/5. Development of systemic lupus erythematosus in a patient with selective complete C1q deficiency.A 7-year-old male with recurrent erythematous and desquamated skin lesions and respiratory infections was diagnosed as selective complete C1q deficiency following detailed studies of the complement system. His asymptomatic sister also had selective complete C1q deficiency. During a follow up period of 3 years, his skin lesions persisted, he suffered from recurrent bronchopneumonias and glomerulonephritis developed. Renal function deteriorated with the appearance of anti-dna antibodies. Renal biopsy was consistent with systemic lupus erythematosus. The patient was treated with immunosuppressive drugs, but died of renal failure. It is postulated that in this patient defective clearance of antigen-antibody complexes by the reticulo-endothelial system resulted in progressive renal disease as observed in other complement deficiencies. A retrospective molecular study disclosed a point mutation in the ClqA chain gene in a heterozygous state in parents and two siblings; in a homozygous state in the asymptomatic sister. The reason why some individuals with this defect are asymptomatic is not known at present. diagnosis of heterozygotes by molecular studies is extremely important to give genetic counselling to the family. Conclusion: patients with recurrent infections, erythematous desquamative skin lesions, malar rash and oral mucosal involvement should be screened for complement c1q deficiency.- - - - - - - - - - ranking = 0.125keywords = antigen (Clic here for more details about this article) |