Monoclonal gammopathy in heart transplantation: clinical significance and risk factors.
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Biomedical subjects
Publications and source records attributed to G Amadori.
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A 60-year-old patient with intra-abdominal lymphangiomatosis is described. He presented with anaemia due to enteric haemorrhage, hypoproteinaemia with heavy hypogammaglobulinaemia and T-cell lymphopenia. Duodenal biopsy showed lymphangiectasia while a small bowel study revealed several filling defects in the terminal ileum. On exploratory laparotomy, numerous inoperable lymphangio-haemangiomata were found, involving the small and large intestine, appendix, mesenterium, gallbladder and main biliary tract. The importance of T-cell lymphopenia and hypogammaglobulinaemia in the diagnosis of intra-abdominal lymphangiomatosis with lymphangiectasia is stressed.
We report a retrospective study about the incidence of second neoplasms (SN) in patients affected by chronic lymphocytic leukemia (CLL) admitted to Padua Hospital between 1989 and 1991, comparing data with those of a similar population. We examined the records of 212 patients, finding in 19 of them 22 second neoplasms; the most common kind was lung cancer. There was an increased incidence of SN, without statistic significance if compared with all sites of cancers in the general population, especially during the first 2 years from the diagnosis of CLL. In accordance with the majority of authors, there is an unknown connection between the two diseases, but certainly independent of chemotherapy with alkylating agents.
The authors report the case of a young nun who came to their attention because of the simultaneous development of mammary and pelvic lymphoblastic lymphoma and acute leukemia showing aspects typical of nonendemic Burkitt's lymphoma. The rapid tumoral growth and the equally rapid spontaneous cell lysis led to severe renal insufficiency and metabolic acidosis which were ultimately the cause of death. Given the rareness of the clinical situation and the diagnostic problems involved, the authors examined the literature dealing with this subject.
During phenytoin therapy, a male patient showed hypertermia, myalgias and mediastinal and abdominal lymphadenopathy. Despite the impossibility of histological diagnosis, he was treated with antiblastic polychemotherapy with little benefit. Another female patient on the same therapy showed splenomegaly with pancytopenia. The spleen and some abdominal lymph nodes were removed; they revealed a pattern of tubercular-like gigantocellular-epithelioid chronic flogosis. As in the first patient, the suspension of therapy gave a disease-free period. A third female patient on diphenylhydantoin therapy undervent some hemolytic crysis and, finally, SLE with cardiac involvement. We point out the complexity of the pathogenesis of hydantoinic immunologic syndromes, the polymorphism of the clinical patterns and the difficulty to make a correct diagnosis, mostly on presence exclusively deep involvement.
In this study, 118 consecutive adult patients with supratentorial gliomas underwent preoperative immunological monitoring, with particular regard to B-lymphocyte and T-lymphocyte markers. Most patients were treated surgically and with radiotherapy. Three months later, they were readmitted for postoperative immunological investigation and follow-up control. A total of 76 cases could thus be completely investigated and were statistically eligible for evaluation. A pronounced failure of T-cell-mediated immunity was observed: "E-active" rosette-forming cells and mitogen-induced blastogenesis tests turned out to be markedly depressed, with a slight postoperative recovery. Spontaneous cell-mediated cytotoxicity was significantly (p less than 0.01) increased both in preoperative and postoperative findings. The main immunodiagnostic patterns (immunoglobulins assay, surface immunoglobulins, "mouse" rosettes) concerning the B-cell-dependent "pool" were found to be within normal limits.
High fever, spleen and lymph node enlargement, and joint pains that assumed the character of rheumatoid arthritis in the ensuing months were noted after a viral hepatitis episode in a 21-year-old woman. Serious anaemia and myocarditis also appeared when the picture was at its worst. A lymphoma was suspected, and the spleen and some abdominal lymph nodes were removed. These displayed signs of intense follicular reaction unaccompanied by atypia. The possibility that juvenile rheumatoid arthritis may be triggered by hepatitis is examined.
In 29 bronchogenic, 22 esophageal carcinomas and 17 glioblastoma peripheral mononuclear cells cytotoxicity toward chicken red blood cells was studied under three different sets of conditions: spontaneously induced, PHA induced, or mediated by antibodies toward target cells lysis. Cultures were 48 hours. While the cytotoxicity induced by mitogen and that medicated by antibody are diminished when compared to the control groups, spontaneous cytotoxicity shows values significantly higher in all the tumoral forms examined. Kinetically this type of lysis shows an already increased release of isotope after 24 hours and a steady rise in the successive hours. After depletion of adherent cells the mononuclear elements lose most of their lytic power. This datum, which contrasts with the diminished spontaneous cytotoxicity toward tumoral cells frequently described in the course of neoplasm, underlines the heterogenicity of the element responsible for SCMC and the diverse effects that the disease provokes on cell function.
Certain properties of the bond between lymphocytes and mouse red blood cells were studied. The turnover of the receptor on the lymphocytes of twelve healthy subjects was evaluated. After two hours of incubation in a medium devoid of additives the receptor is released and its resynthesis is completed in twenty-four hours. In experiments employing proteolytic enzymes inhibition of rosette formation is observed, whereas neuraminidase, under the same experimental conditions, significantly increases the number of rosette forming cells. The percentage of mouse rosette forming cells in nineteen patients affected with chronic lymphatic leukaemia was then evaluated (17 with type B and 2 with type T). On the basis of these cases and other recently published data, an ontogenetic explanation for the appearance of this receptor is proposed.
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The authors report their experience on the immunological monitoring of children with posterior fossa medulloblastomas. The most important findings concerning humoral and cell-mediated immunity in this kind of patient are discussed. Among the main immunobiological pictures, the authors stress the remarkable failure of the T-cell dependent immune response, generally correlated to the degree of malignancy of the tumor, and the characteristic appearance of cells with natural cytotoxic activity, whose precise outlining is, at present, in progress. The B-cell dependent pool, mostly investigated through immunofluorescence studies, turned out to be very close to normal.
T cell immune function in 20 newborn infants was investigated. Previous studies showing increased spontaneous transformation and higher 3H-thymidine incorporation at lower PHA concentration in newborn infants were confirmed. A net increase in the number of active E rosette-forming lymphocytes and a slight decrease in the percentage of total E rosette-forming cells was also found. Our results suggest the presence of a subpopulation of activated T lymphocytes in the peripheral blood of newborns during the first days of life. Possible mechanisms of this activation are discussed.
A wide pre- and postoperative immunological monitoring of B- and T-cell markers in children with medulloblastomas is presented. Preoperative investigations showed a noticeable failure of T-cell-dependent immunity, currently identified by "active" E-rosette forming cells (Ea-RFC) and blastogenesis tests, with a significant increase of spontaneous cell-mediated cytotoxicity (SCMC). In postoperative long-term controls, a slight recovery of Ea-RFC and blastogenesis tests was observed. The main findings concerning cytotoxicity kinetics are discussed.
The effect of alpha-mercaptopropinyl-glycine on the in vitro proliferative response of healthy subjects' lymphocytes was studied. Low doses of the drug enhanced spontaneous lymphocyte blastigenesis but had no effect on the PHA-induced blastic response. With increasing concentrations of alpha-mercaptopropionyl-glycine inhibition of 3H-thymidine incorporation was found in unstimulated as well as in PHA stimulated lymphocytes.
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Preliminary findings in the evaluation of the immune response of children with primary neoplasms of the CNS, mainly medulloblastomas, are reported and discussed. A broad scheme for the monitoring of B- and T-cell-dependent immunity and of delayed hypersensitivity reactions in this type of patient is presented. The most important immunobiological findings are discussed. Special attention is given to the striking failure of the T-cell-dependent pool (currently identified by "active" RFC and blastigenesis tests) and to the remarkable decrease of hypersensitivity reactions (depressed skin-test response), both of which seem to be related to the degree of malignancy of the tumour. A very peculiar feature, i.e., the appearance of cells with natural cytotoxic activity, is dealt with in some detail. Our present knowledge concerning the immunobiology of primary CNS neoplasms is still very incomplete, but seems to suggest a possible role for immunotherapy in paediatric neurosurgery.
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