[Immunologic deficiency syndromes].
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Examination of 30 patients with immunodeficient diseases showed that the system of natural antibodies was considerably changed depending on the form and the extent of deficiency of the immune system: in agammaglobuinemia the antibodies under study were almost completely absent, and in immunological insufficiency with ataxia-teleangiectasis the production of antibacterial antibodies proved to be sharply decreased. The data obtained can be used both for the diagnosis of immunodeficiencies and for control of the restoration of the immunological competence after a number of therapeutic measures, particularly after the transplantation of the thymus-sternum complex.
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Although supported by a number of experimental models, the assumption assigning a crucial role to the immune system in the antineoplastic defense mechanisms has not been convincingly demonstrated so far for human tumors. Should the theory be correct, severe functional impairment of the immune system would obviously result in the occurrence of tumors with abnormally high frequency. Registry holdings systematically collecting pertinent information on the malignancies developed in patients with primary immunodeficiency diseases or in organ transplant recipients maintained on therapeutically-induced immune depression, as well as the observation of tumors occurring in patients treated with immunosuppressive agents and of second malignancies arising after radio- and/or chemotherapy of the primary tumor consistently indicate that depressed immunity is usually associated with an increased incidence of cancer as compared with that expected in the general control populations. However, not all types of tumors are increased to the same extent, in that lymphoreticular neoplasias (especially non-Hodgkin's lymphomas), acute leukemias as second tumors and, among solid neoplasms, squamous cell carcinomas are those most frequently reported. These observations suggest that even deeply impaired tumoricidal immune mechanisms may facilitate the growth of certain tumors only, especially of those arising from the cells of the immune system itself, in remarkable contrast with their frequency in the general population. Oncogenesis may be favoured in various states of depressed immunity by a number of ways. Their elucidation might have bearing on the comprehension of the more general phenomenon of the neoplastic transformation.
Three cases of multiple opportunistic infections in previously healthy homosexual males were observed in 3 different University Hospitals in Switzerland. Two of the patients died. Infections were multiple, with P. carinii pneumonia (3 cases), chronic mucocutaneous ulcers probably due to Herpes simplex virus (2 cases), mucosal candidiasis (2 cases) and disseminated infections due to cytomegalovirus (1 case) and Mycobacterium avium (1 case). All patients had depressed cellular immunity with marked lymphopenia. These cases are similar to those recently observed in the United States, but two of the patients had never been to the USA although both had vacationed in Haïti where the syndrome has been described. These patients illustrate that this new syndrome should also be suspected outside the USA when a patient (especially a male homosexual) presents with persistent fever of unknown origin and develops opportunistic infections without obvious underlying disease.
The morphology of lymphatic tissues in 43 autopsy cases of children with inherited immunodeficiency states were analysed. Among the more common diseases, such as Di-George-syndrome, CID-patients, congenital agammaglobulinemia Bruton, CVID, selective IG-A deficiency, Wiskott-Aldrich-syndrome, tissue sections of very rare conditions associated with immunodeficiency, e.g. fetopathia diabetica and leprechaunismus, were investigated by routine and immunohistochemical stainings. Clinical history and laboratory data, augmented by the characteristic pathomorphology of lymphatic tissue sections, will establish or at least suggest a definite diagnosis. Since true thymic dysplasia is very rare (or even non-existent) in the human, this term should be abandoned. Severe thymic tissue alterations in SCID-patients, occur secondary to enzyme defects in lymphatic cells. If patients are successfully treated by bone marrow transplantation, the thymus will subsequently develop into a functionally normal organ.