Antibody deficiency syndrome associated with beta-2 macroglobulinemia.

作者: Richard Hong , William K. Schubert , Eugene V. Perrin , Clark D. West

DOI: 10.1016/S0022-3476(62)80193-0

关键词:

摘要: A patient with an uncommon variation of the antibody deficiency syndrome was studied by means quantitation serum immunoglobin response to injected antigens, determination granulocyte kinetics, and skin homografting. The defect defined as a persistent absence beta-2A globulin, severe moderate hypogammaglobulinemia, extreme beta-2 macroglobulinemia (concentrations 400 per cent normal mean). antigens indicated that patient's mechanism for synthesis macroglobulin intact appeared compensate partially failure synthesize nonmacroglobulin antibody. homograft underwent delayed rejection. Survival studies granulocytes showed decreased survival time white cells infused into patient. At age 27 months died alveolar proteinosis Pneumocytis carinii pneumonia. thymus noted be atrophied, peculiar deformed type Hassall's corpuscle found.

参考文章(28)
H.F. Deutsch, R.A. Alberty, L.J. Gosting, BIOPHYSICAL STUDIES OF BLOOD PLASMA PROTEINS IV. SEPARATION AND PURIFICATION OF A NEW GLOBULIN FROM NORMAL HUMAN PLASMA Journal of Biological Chemistry. ,vol. 165, pp. 21- 35 ,(1946) , 10.1016/S0021-9258(17)41202-6
Fred S. Rosen, Sherwin V. Kevy, David Gitlin, Charles A. Janeway, Ezio Merler, RECURRENT BACTERIAL INFECTIONS AND DYSGAMMAGLOBULINEMIA: DEFICIENCY OF 7S GAMMA-GLOBULINS IN THE PRESENCE OF ELEVATED 19S GAMMA-GLOBULINS Report of Two Cases Pediatrics. ,vol. 28, pp. 182- 195 ,(1961)
Melvin Cohn, H. F. Deutsch, J. C. Nichol, Biophysical Studies of Blood Plasma Proteins XI. Immunological and Electrophoretic Studies of Immune Chicken Serum Journal of Immunology. ,vol. 63, pp. 195- 210 ,(1949)
H. G. Kunkel, E. C. Franklin, H. J. Muller-Eberhard, STUDIES ON THE ISOLATION AND CHARACTERIZATION OF THE “RHEUMATOID FACTOR” Journal of Clinical Investigation. ,vol. 38, pp. 424- 434 ,(1959) , 10.1172/JCI103817
J. Lospalluto, M. Ziff, CHROMATOGRAPHIC STUDIES OF THE RHEUMATOID FACTOR Journal of Experimental Medicine. ,vol. 110, pp. 169- 186 ,(1959) , 10.1084/JEM.110.2.169
Solomon J. Zak, Robert A. Good, IMMUNOCHEMICAL STUDIES OF HUMAN SERUM GAMMA GLOBULINS* Journal of Clinical Investigation. ,vol. 38, pp. 579- 586 ,(1959) , 10.1172/JCI103835
H.E. Schultze, The synthesis of antibodies and proteins Clinica Chimica Acta. ,vol. 4, pp. 610- 626 ,(1959) , 10.1016/0009-8981(59)90002-6
ARTHUR R. PAGE, Studies on Cyclic Neutropenia A.M.A. Journal of Diseases of Children. ,vol. 94, pp. 623- 661 ,(1957) , 10.1001/ARCHPEDI.1957.04030070035006
W. K. SCHUBERT, R. FOWLER, LESTER W. MARTIN, CLARK D. WEST, Homograft rejection in children with congenital immunological defects: agammaglobulinemia and Aldrich syndrome. Plastic and Reconstructive Surgery. ,vol. 26, pp. 125- 127 ,(1960) , 10.1097/00006534-196007000-00044