Abstract
The case is reported of a 4-year-old girl affected with recurrent infections; anaemia, thrombocytopenia, haemorrhages and hepatosplenomegaly. Immunological investigations revealed a defect in cellular immunity related to the thymus-dependent system, hypergammaglobulinaemia (especially of class IgE), and very high titres of antibodies against Epstein-Barr virus (EBV). After foetal thymus transplantation, correction of the immunological defect and significant clinical improvement were noted, as well as a decrease of IgE and EBV antibody titres.
Full text
PDF






Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Aiuti F., Wigzell H. Function and distribution pattern of human T lymphocytes. I. Production of anti-T lymphocyte specific sera as estimated by cytotoxicity and elimination of function of lymphocytes. Clin Exp Immunol. 1973 Feb;13(2):171–181. [PMC free article] [PubMed] [Google Scholar]
- Ammann A. J., Wara D. W., Salmon S., Perkins H. Thymus transplantation. Permanent reconstitution of cellular immunity in a patient with sex-linked combined immunodeficiency. N Engl J Med. 1973 Jul 5;289(1):5–9. doi: 10.1056/NEJM197307052890102. [DOI] [PubMed] [Google Scholar]
- August C. S., Rosen F. S., Filler R. M., Janeway C. A., Markowski B., Kay H. E. Implantation of a foetal thymus, restoring immunological competence in a patient with thymic aplasia (Digeorge's syndrome). Lancet. 1968 Dec 7;2(7580):1210–1211. doi: 10.1016/s0140-6736(68)91693-0. [DOI] [PubMed] [Google Scholar]
- BRETON A., WALBAUM R., BONIFACE L., GOUDEMAND M., DUPONT A. [Lymphocytophthisis with dysgammaglobulinemia in an infant]. Arch Fr Pediatr. 1963 Feb;20:131–146. [PubMed] [Google Scholar]
- Bach J. F. Target cell of thymic hormone. Lancet. 1973 Jun 9;1(7815):1320–1320. doi: 10.1016/s0140-6736(73)91336-6. [DOI] [PubMed] [Google Scholar]
- Cleveland W. W., Fogel B. J., Brown W. T., Kay H. E. Foetal thymic transplant in a case of Digeorge's syndrome. Lancet. 1968 Dec 7;2(7580):1211–1214. doi: 10.1016/s0140-6736(68)91694-2. [DOI] [PubMed] [Google Scholar]
- DE VRIES M. J., VAN PUTTENL, BALNER H., VAN BEKKUMD L'ESIONS SUGG'ERANT UNE R'EACTIVIT'E AUTO-IMMUNE CHEZ DES SOURIS ATTEINTES DE LA "RUNT DISEASE" APR'ES THYMECTOMIE N'EONATALE. Rev Fr Etud Clin Biol. 1964 Apr;9:381–397. [PubMed] [Google Scholar]
- Gatti R. A., Gershanik J. J., Levkoff A. H., Wertelecki W., Good R. A. DiGeorge syndrome associated with combined immunodeficiency. Dissociation of phytohemagglutinin and mixed leukocyte culture responses. J Pediatr. 1972 Nov;81(5):920–926. doi: 10.1016/s0022-3476(72)80544-4. [DOI] [PubMed] [Google Scholar]
- Goldman A. S., Haggard M. E., McFadden J., Ritzmann S. E., Houston E. W., Bratcher R. L., Weiss K. G., Box E. M., Szekrenyes J. W. Thymic alymphoplasia, lymphoma, and dys-gamma-globulinemia, hyper-gamma A-, normo-gamma M-, hypo-gamma G, a-gamma D-, and gamma E-globulinemia, plasmacytosis, normal delayed hypersensitivity, severe allergic reactions, and Coombs' positive anemia. Pediatrics. 1967 Mar;39(3):348–362. [PubMed] [Google Scholar]
- Henle G., Henle W. Immunofluorescence in cells derived from Burkitt's lymphoma. J Bacteriol. 1966 Mar;91(3):1248–1256. doi: 10.1128/jb.91.3.1248-1256.1966. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Jondal M., Holm G., Wigzell H. Surface markers on human T and B lymphocytes. I. A large population of lymphocytes forming nonimmune rosettes with sheep red blood cells. J Exp Med. 1972 Aug 1;136(2):207–215. doi: 10.1084/jem.136.2.207. [DOI] [PMC free article] [PubMed] [Google Scholar]
- KELLUM M. J., SUTHERLAND D. E., ECKERT E., PETERSON R. D., GOOD R. A. WASTING DISEASE, COOMBS-POSITIVITY, AND AMYLOIDOSIS IN RABBITS SUBJECTED TO CENTRAL LYMPHOID TISSUE EXTIRPATION AND IRRADIATION. Int Arch Allergy Appl Immunol. 1965;27:6–26. doi: 10.1159/000229594. [DOI] [PubMed] [Google Scholar]
- Komuro K., Boyse E. A. In-vitro demonstration of thymic hormone in the mouse by conversion of precursor cells into lymphocytes. Lancet. 1973 Apr 7;1(7806):740–743. doi: 10.1016/s0140-6736(73)92127-2. [DOI] [PubMed] [Google Scholar]
- Kravis L. P., Donsky G. J., Lecks H. I., Bhagwat S. Detection of tuberculin sensitivity in children by leukocyte culture. Am J Dis Child. 1968 Feb;115(2):247–252. doi: 10.1001/archpedi.1968.02100010249014. [DOI] [PubMed] [Google Scholar]
- Levine P. H., Ablashi D. V., Berard C. W., Carbone P. P., Waggoner D. E., Malan L. Elevated antibody titers to Epstein-Barr virus in Hodgkin's disease. Cancer. 1971 Feb;27(2):416–421. doi: 10.1002/1097-0142(197102)27:2<416::aid-cncr2820270227>3.0.co;2-w. [DOI] [PubMed] [Google Scholar]
- Ling N. R., Holt P. J. The activation and reactivation of peripheral lymphocytes in culture. J Cell Sci. 1967 Mar;2(1):57–70. doi: 10.1242/jcs.2.1.57. [DOI] [PubMed] [Google Scholar]
- Lischner H. W. DiGeorge syndrome(s). J Pediatr. 1972 Nov;81(5):1042–1044. doi: 10.1016/s0022-3476(72)80575-4. [DOI] [PubMed] [Google Scholar]
- Mancini G., Carbonara A. O., Heremans J. F. Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry. 1965 Sep;2(3):235–254. doi: 10.1016/0019-2791(65)90004-2. [DOI] [PubMed] [Google Scholar]
- Marchi A. G., Massimo L. Su di un caso di neutropenia cronica e anemia emolitica autoimmune in un bambino di 2 anni e 7 mesi. Minerva Pediatr. 1969 Jun 19;21(23):1045–1050. [PubMed] [Google Scholar]
- Miller J. F., Osoba D. Current concepts of the immunological function of the thymus. Physiol Rev. 1967 Jul;47(3):437–520. doi: 10.1152/physrev.1967.47.3.437. [DOI] [PubMed] [Google Scholar]
- Miller J. F. Thymus tissue as a therapeutic measure. N Engl J Med. 1972 Oct 19;287(16):818–819. doi: 10.1056/NEJM197210192871608. [DOI] [PubMed] [Google Scholar]
- Pernis B., Forni L., Amante L. Immunoglobulin spots on the surface of rabbit lymphocytes. J Exp Med. 1970 Nov;132(5):1001–1018. doi: 10.1084/jem.132.5.1001. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Rezza E., Aiuti F., Businco L., Castello M. A. Familial lymphopenia with T lymphocyte defect. J Pediatr. 1974 Feb;84(2):178–182. doi: 10.1016/s0022-3476(74)80598-6. [DOI] [PubMed] [Google Scholar]
- Rubinstein A., Speck B., Jeannet M. Successful bone-marrow transplantation in a lymphopenic immunologic deficiency syndrome. N Engl J Med. 1971 Dec 16;285(25):1399–1402. doi: 10.1056/NEJM197112162852503. [DOI] [PubMed] [Google Scholar]
- Schaller J., Davis S. D., Ching Y. C., Lagunoff D., Williams C. P., Wedgwood R. J. Hypergammaglobulinaemia, antibody deficiency, autoimmune haemolytic anaemia, and nephritis in an infant with a familial lymphopenic immune defect. Lancet. 1966 Oct 15;2(7468):825–829. doi: 10.1016/s0140-6736(66)92255-0. [DOI] [PubMed] [Google Scholar]
- Stiehm E. R., Fudenberg H. H. Serum levels of immune globulins in health and disease: a survey. Pediatrics. 1966 May;37(5):715–727. [PubMed] [Google Scholar]
- Yunis E. J., Stutman O., Good R. A. Thymus, immunity and autoimmunity. Ann N Y Acad Sci. 1971 Sep 15;183:205–220. doi: 10.1111/j.1749-6632.1971.tb30752.x. [DOI] [PubMed] [Google Scholar]
