Biomedical subjects
Publications and source records attributed to A Guarini.
The ultrastructural analysis of platelets from patients with Primary Thrombocythemia (PT) showed some anomalous features. Platelets having structural modifications of their organelles were observed and a decrease in the number of dense bodies was seen. Freeze-fracture analysis of the plasma membrane of platelets from PT patients showed a significant increase in the number of intramembranous particles (IMP).
A significant reduction in the proportion and absolute number of circulating T gamma (suppressor) lymphocytes was observed in 15 adult patients with idiopathic thrombocytopenic purpura (ITP). The proportion of T gamma cells was also reduced in the spleen of four patients so investigated. This abnormality was not seen in 5 patients cured by splenectomy whilst it persisted in 3 who remained thrombocytopenic after splenectomy. The proportion of Tmu (helper) lymphocytes in ITP was normal. These findings, similar to those reported in systemic lupus erythematosus, suggest that an inbalance of the immunoregulatory T-cell subsets may be important in the pathogenesis of ITP.
51Cr-platelet kinetics study was performed in 197 patients with chronic ITP after corticosteroid therapy had failed to induce a long lasting remission. The incidence of splenic, spleno-hepatic, hepatic and diffuse platelet sequestration site was 58%, 17%, 6% and 19%, respectively. Splenic and spleno-hepatic sequestration sites were more frequent in patients less than 30 years old and in patients with a platelet count lower than 50 x 10(9)/1. 111 patients were splenectomized shortly after the study. Normalization of the platelet count was obtained more frequently in patients with splenic and spleno-hepatic sequestration than in the others. Labelled platelet sequestration site was the best predictor of the outcome of splenectomy. Platelet kinetics is a non-invasive investigation that should be performed early after the diagnosis of chronic ITP in all patients eligible for splenectomy.
Defibrination is a rare event in acute lymphoblastic leukaemia (ALL). This paper describes four cases of acute defibrination in a series of 52 adult patients with ALL. All four patients had blood clotting tests consistent with or suggesting defibrination prior to therapy, but haemorrhages and unequivocal laboratory evidence of defibrination developed only after leukaemic blast cells had been destroyed early during therapy for remission induction. All patients received supportive therapy, and three of them were also given a continuous IV heparin infusion. Haemorrhages were controlled and blood clotting tests were improved within 12--48 h.
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Acute promyelocytic leukemia (APL) was diagnosed in 13 of 84 adult patients (15.4%) with acute myeloid leukemia (AML) first admitted between 1972 and 1976. All patients had clinical and/or laboratory evidence of defibrination syndrome. Four patients died of cerebral hemorrhage within 2 days of admission. Two patients died of generalized infection on days 7, and 16, respectively, after admission. The remaining 7 patients (54%) underwent complete remission (CR) with daunomycin, arabinosyl cytosine, and adriamycin. All patients received massive platelet transfusion, no heparin, and no granulocyte transfusion. CR was more frequent in patients with a very low blast cell count and a fibrinogen level higher than 100 mg/100 ml. Median survival of these seven CR patients with APL is similar (15 months) to that of CR patients with other types of AML treated at the same institution during the same period.
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The last fifteen years have witnessed impressive changes in our approach to patients with different haematological malignancies. These have largely stemmed from the continuous development of biotechnologies and from their progressive implementation in the clinical setting. In this brief review, we will highlight some of the areas (diagnosis, follow-up and treatment) in which biotechnologies have had an objective impact and will also underline how a close biologico-clinical integration, unparalleled in all other fields of oncology, is today mandatory for an up-to-date management of haematological malignancies.
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