Familial myeloma. A new observation in two distinct pairs of siblings.
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Biomedical subjects
Publications and source records attributed to E Ascari.
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BACKGROUND: The experimental observation that plasma from TTP patients sometimes exhibits a protein which can cause platelet agglutination, and that such agglutination can be inhibited in vitro by the use of IgG led some authors to treat plasma exchange-resistant TTP patients with high-dose IgG (HDIgG). METHODS: We report the results obtained with HDIgG treatment in 17 patients retrospectively examined by the Italian Cooperative Group for the study of TTP: 6 males and 11 females, mean age was 31.7 years for the women (range: 20-65) and 44.6 for the men (range: 26-66). In all cases HDIgG administration was combined with other treatment modalities. RESULTS: Of the 17 patients, 7 died from disease progression (41.1%), 2 achieved partial remission (11.7%) and the remaining 8 achieved complete remission (47%). Of the 10 cases (58.8%) with a positive response, only in 4 did the addition of HDIgG seem to produce significant improvement. All efforts made to characterize the subgroup of patients who responded to HDIgG and compare them with the non responders failed. CONCLUSIONS: Although our results do not unquestionably demonstrate the role of HDIgG in the treatment of TTP, they suggest a possible role for HDIgG in the treatment of those rare plasma exchange-resistant TTP cases.
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A 23-year-old male patient developed aplastic anemia and was treated with cyclosporin A and rhG-CSF. Bone marrow biopsy showed an improvement in cellularity with a recovery of all hematopoietic precursors after nine weeks of therapy. WBC increased after two weeks of treatment, mostly due to an increase in the absolute granulocyte cell count. Hb, RBC, Plts and reticulocytes showed an increase six weeks after the beginning of therapy. Of the serum cytokines, EPO levels progressively decreased, while sTfR increased in peripheral blood. A reverse correlation between blood neutrophil count and serum levels of G-CSF was observed, indicating an increased clearance of G-CSF. Finally, sIL-2R showed a rapid increase in the first week of treatment and prior to the increase in PMN cells. Thus, the use of cyclosporin A and rhG-CSF in our patient induced a complete recovery of hemopoiesis, and this may be explained by a synergic effect induced by the capacity of cyclosporin A to remove inhibitory factors and the stimulatory activity of rhG-CSF.
Seventeen consecutive patients with previously untreated poor prognosis Hodgkin's disease (clinical stage II and III with systemic symptoms, and stage IV) received 6 courses of aggressive chemotherapy, with (9 patients) and without (8 patients) the addition of recombinant human granulocyte-macrophage colony stimulating factor (rhGM-CSF). Chemotherapy (MOPP/ABV/CAD regimen) included full doses of nitrogen mustard, lomustine (CCNU), vindesine, melphalan, prednisone, epidoxorubicin, vincristine, procarbazine, vinblastine and bleomycin, and was administered between days 1 and 15 of each course. Course were planned for 28-day intervals. rhGM-CSF was given at a dose of 5 micrograms/kg/day subcutaneously from day 16 to 26 of each course. With cytopenia (i.e. white blood cell, WBC, count < 3.0 x 10(9)/L and/or platelet count < 100 x 10(9)/L) delaying courses was preferred to administering reduced drug dosages. Substantial delays (ranging from 7 to 28 days) in delivering cytostatics were necessary between 70% of courses. The cumulative mean number of days for which the courses had to be delayed before completing the 6 MOPP/ABV/CAD courses was 57. The percentage of planned doses of cytotoxic drugs (nitrogen mustard, melphalan, epidoxorubicin, procarbazine) actually administered was 92%. Causes of treatment delay were presented by leucopenia in 82% and by leuco-thrombocytopenia in 23% of the courses. The WBC nadir was constantly encountered at day 20-21 following completion of courses, and slightly worsened with subsequent courses. The decrease in platelet values was milder than that in WBC counts. There were no differences in any of the above parameters between patients treated with MOPP/ABV/CAD alone or followed by rhGM-CSF.(ABSTRACT TRUNCATED AT 250 WORDS)