T-gamma lymphocytes in a case of congenital hypoplastic anemia (Diamond-Blackfan syndrome).
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Biomedical subjects
Publications and source records attributed to L Massimo.
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In 22 patients with ALL in first complete remission, who discontinued therapy after 3 years, longitudinal follow-up of immunological reconstitution was investigated by assessing serum immunoglobulin levels, number and mitogen responsiveness of B and T lymphocytes. Major modifications have been demonstrated for T cells both in terms of number and functionality, while B cells and serum immunoglobulins did not change significantly. Examining the behaviour of the patients one by one, it has been observed that recovery is often characterized by a remarkable rebound occurring from the 15th to the 18th month. Immunosuppression induced by antileukemic treatment appeared to be a transitory phenomenon, mainly limited to the first three months. Reconstitution was achieved, in almost all cases, within one year after treatment was stopped.
A case has been described of Trisomy 8 mosaicism Syndrome. At onset the child presented with hyporegenerative anemia; the study of colony forming capacity in vitro (CFU) by Bone Marrow (B) and Peripheral Blood (PB) showed an abnormal colony formation by myeloid and erythroid progenitor cells. No immunological defects were discovered. The in vitro colony formation appears to have a definite role in the identification of patients who may be at higher risk of developing leukemia. The importance of 8 chromosome for hematopoiesis control is discussed.
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Peripheral blood lymphoid cells of 29 patients with acute lymphocytic leukaemia (ALL) at onset were studied for characterization of B and T membrane markers and phytohaemagglutinin responsiveness. 24 cases (83%) were classified as "null" cell ALL and 5 (17%) as T-cell ALL. No relationship could be found between cytological presentation and immunological classification. Moreover, no correlation has been demonstrated between clinical-immunological parameters and prognosis, indicating that in our series of patients, assessment of cell size and surface markers were not a reliable predictor of prognosis.
Peptichemio (PTC) is a mixture of six synthetic peptides of m-L-phenylalanine mustard. It acts with both alkylating and antimetabolic effects, interfering with the synthesis of DNA, RNA, and proteins. PTC was administered iv to 18 previously untreated children with advanced neuroblastoma at a dose of 1-1.5 mg/kg/day for one to three cycles of 5-6 consecutive days each. Eleven of 12 patients (92%) experienced both objective and subjective improvement; complete remission was achieved in two of them. In spite of the high remission rate, the median duration of remission has been short (4 months) and the overall survival (median, 6 months) did not seem to be influenced by the use of PTC. The primary toxic effects were, in order of importance, bone marrow depression, phlebosclerosis, nausea and vomiting, and alopecia. Chronic use of PTC seems limited by two major factors: profound long-lasting thrombocytopenia and severe phlebosclerosis.
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In the present study, 15 children with non-Hodgkin lymphoma have been immunologically evaluated by the following parameters on peripheral blood (PB) lymphocytes: phytohemagglutinin responsiveness (PHA-r); non-immune rosette formation with sheep red blood cells (RF-L) as T-cell marker; presence of surface immunoglobulins (sIg-L) as B-cell marker; serum immunoglobulins levels (IgA, IgM, IgG). Our patients (pts) have been divided in two groups: the first one includes 10 children without PB involvement; the second one includes 5 pts with bone-marrow and PB invasion. From our data it appears that: 1) the majority of pts of the first group presented normal values of membrane markers; PHA-r was impaired in 4/8 pts; 2) in pts with PB invasion absolute number of B and "null" cells was always abnormal and PHA-r altered; 3) in the second group of pts, a "null" cell origin can be suggested by the high percentage and absolute number of cells without surface markers. In our opinion, the high incidence of "null" cells represents the most relevant question: whether they are cells deprived of specific markers, or endowed with markers not identifiable by our current techniques, remains to be established.
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32 children with different types of tumors have bben treated with peptichemio. The efficacy was excellent in rhabdomyosarcoma and embrional sarcoma; encouraging in neuroblastoma, Wilms tumor and histiocytosis X. Side and toxic effects were minimal. In conclusion we can say that peptichemio is effective in oncologic diseases, but a larger number of patients is required in order to have a better knowledge about the antitumor activity of this drug.
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