Haptoglobin phenotype and gene frequencies in the population of Rome.
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Biomedical subjects
Publications and source records attributed to E Fagiolo.
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The results of an investigation on platelet antibodies by thromboagglutination and antiglobulin consumption test (AGCT) in 51 patients with different types of hemoblastoses (acute and chronic leukemias, Hodgkin disease, lymphosarcoma) are reported. Complete and/or incomplete antibodies were found in 50,9% of the cases, with lymphoid forms showing the highest frequency. Although a constant relation between thrombocytopenia and platelet antibodies is by no means demonstrable, four fully descirbed cases clearly show and the onset of autoimmune thrombocytopenia in the course of hemoblastoses. Two chronic myeloid leukemia cases treated with Myleran and one chronic lymphoid leukemia case presented thrombocytopenic purpura and normal number of megakaryocytes. One case of lymphosarcoma presented thrombocytopenia and autoimmune hemolytic anemia with bone marrow aplasia. Steroid therapy had been partially effective only in two cases. The pathogenetic relationship between platelet immunization and hemoblastoses and cytostatic therapy is discussed.
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Amyloid substance was looked for in bone marrow aspirates of 28 patients presenting an M-component, in association with different illnesses (plasmocytoma, chronic lymphatic leukemia, lymphocytic lymphoma, "benign monoclonal gammapathy" and liver cirrhosis). Amyloid was detected in 12 out of 20 patients with plasmocytoma (60%) and in 3 out of 8 patients with other illnesses (37.5%); it was found in 9 out of 13 cases (69.2%) presenting exclusive light chain production and in 6 out of 15 cases (40%) with a monoclonal complete Ig. Lambda type light chain, either free or Ig-bound, was slightly more often (6 cases out of 10, 60%) associated with amyloidosis than was K (9 cases out of 17, 52.9%). Bone marrow appeared to be more frequently involved than other sites considered elective such as rectal mucosa. Amyloid appeared as an intensively fluorescent substance present intercellularly in areas of variable number and size.
Our previous studies on autoimmune haemolytic anaemia (AIHA) have shown a hyperactivation concerning cytokine production in T and B lymphocytes obtained from AIHA patients. In this study the production of interleukin (IL)-10, basal and stimulated by lipopolysaccharide (LPS), was determined in cultured monocytes obtained from patients affected by AIHA, either idiopathic or associated with other autoimmune diseases, e.g. idiopathic thrombocytopenic purpura (ITP), systemic lupus erythematosus (SLE), myastenia gravis (MG). In cultured AIHA monocytes the IL-10 basal production (mean+/-SD) was increased in all but one patient, compared to the controls, 125.96+/-76.10 pg/mL and 19.18+/-15.80 pg/mL respectively. Specifically, LPS stimulation was able to induce a higher IL-10 production by monocytes in two AIHA cases, whereas in five patients there was no difference and in two cases the LPS stimulated IL-10 production was even decreased compared to the levels observed in the controls. Interestingly in the latter two cases AIHA was associated with other autoimmune diseases (ITP, SLE). These results indicate a constitutively higher basal IL-10 production in monocytes from AIHA patients. The increased level of IL-10 could play an important role in the modified pathways of the immune response in AIHA.
IgM paraproteinaemia is described in a patient with diffuse plasmacytoma involving the pleura, lymph nodes, and kidneys. Sixteen cases of pure plasmacellular tumor (diffuse or solitary plasmacytoma and plasmacellular leukaemia) associated with IgM paraproteinaemia have only been reported. These cases and experimental data indicate that neoplastic plasma cells can synthetize IgM as well as other immunoglobulins and that this particular M component is not associated solely with Waldenström's macroglobulinaemia.
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