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Biomedical subjects

U Magrini

Publications and source records attributed to U Magrini.

At least 55 records · Page 3Linked to original sources

Myelofibrosis in chronic granulocytic leukaemia: clinicopathologic correlations and prognostic significance.

We performed 221 marrow trephine biopsies in 139 patients with Ph1-positive (Ph1+) chronic granulocytic leukaemia (CGL) in order to assess the incidence, degree and prognostic significance of marrow fibrosis (MF) at various stages of the disease. We also attempted to elucidate the relationship between development of MF and the various clinical and haematological features of CGL. A significant correlation was found between the amount of fibrosis (graded from 0 to 3) and the stage of CGL, indicating that major fibrotic changes are associated with accelerated or blastic disease. Survival studies performed to assess the prognostic significance of the various degrees of MF, showed a progressively worse life-expectancy from grade 0 to grade 3 fibrosis. Multivariate regression analysis indicated Hb level, age, number of marrow megakaryocytes (MKs), time from diagnosis as the features most significantly correlated with the degree of MF. This study demonstrates that the natural history of CGL involves a progressive increase in reticulin deposition towards severe MF, although the rate of this progression varies widely. Monitoring changes of fibrosis with sequential biopsies could give a measure of the rate of progression of the disease and help in prognostic assessment of CGL patients. Our findings also confirm that among marrow features the number of MKs is the cytological variable most significantly correlated with MF.

Adolescent↗

Chronic myocarditis leading to a right ventricular cardiomyopathy. Case report.

A case of dilated cardiomyopathy arose after a spontaneous abortion at the second month of pregnancy is reported. Whereas clinical, hemodynamic and gross features pointed to a diagnosis of right ventricular cardiomyopathy, histologic findings of inflammatory infiltration, myocyte degeneration and interstitial fibrosis of the atrial and ventricular walls allowed to a correct diagnosis of chronic myocarditis.

Abortion, Spontaneous↗

Immunoblastic lymphoma: a clinicopathologic study with emphasis on the cases intervening during or following other disorders.

Fifty-eight patients with immunoblastic lymphoma (IBL) were the object of this study. Fifteen of them (26%) developed IBL during or after other diseases, either immunologic or neoplastic, including angio-immunoblastic lymphadenopathy, autoimmune disorders, chronic lymphocytic leukemia, Waldenström's disease, lymphoplasmacytoid lymphoma and Hodgkin's disease (subsequent IBL). The comparison between de novo and subsequent IBL revealed a significantly higher incidence of bone marrow involvement and bulky abdominal disease in the latter group, with a lower response rate to chemotherapy. The favorable primary extranodal disease of Waldeyer's ring exclusively belonged to de novo IBL, whereas the frequency of immunoglobulin abnormalities was higher in the subsequent IBL group (67%). The stage of disease, systemic symptoms at diagnosis and response to therapy were predictive of survival. The overall complete remission (CR) rate in the whole series was 37% and the median overall survival 14 months. Complete remitters have a median survival in excess of 60 months; all relapses occurred within the first 12 months of CR. No CNS relapse terminated the CR, and CNS prophylaxis seems unnecessary in IBL. The analysis of subsequent IBL may provide information on the pathogenesis of non-Hodgkin's lymphomas; the still poor prognosis of IBL deserves new therapeutic attempts to improve on the standard regimens.

Adult↗

Experimental systemic toxicology of 4'-epidoxorubicin, a new, less cardiotoxic anthracycline antitumor agent.

The acute and chronic iv toxicity of 4'-epidoxorubicin, a new antitumor anthracycline antibiotic, was compared with doxorubicin. The LD50 of 4'-epidoxorubicin was 16.07 mg/kg in mice, 14.27 mg/kg in rats, and about 2 mg/kg in dogs; the LD50 of doxorubicin was 11.98 mg/kg in mice, 10.51 mg/kg in rats, and about 2.5 mg/kg in dogs. Rats and dogs were also dosed iv for 91 days (3 injections/week) with 4'-epidoxorubicin or doxorubicin at doses of 0.128, 0.32, and 0.8 mg/kg to rats and 0.064, 0.16, and 0.4 mg/kg to dogs. A comprehensive toxicological evaluation of the animals was carried out before, throughout, and at the end of the study. High-dose 4'-epidoxorubicin induced toxic clinical signs in dogs, and in both species caused loss of body weight, antiproliferative effects on blood-forming organs and testes, and degenerative lesions in kidneys and heart. The cardiac damage was moderate in rats and very mild in dogs; only three male rats died at this dose. The medium dose induced less pronounced changes and no heart lesions and the low dose was practically nontoxic. Doxorubicin showed similar antiproliferative activity, but more evident toxic effects, especially on the heart; many rats given the high dose died and some at the medium dose showed initial cardiac lesions. Thus 4'-epidoxorubicin appeared less toxic than doxorubicin; in particular cardiac damage was much less evident in animals chronically injected with the new drug.

Animals↗

Lymphoblastic lymphoma in adults: a study on 30 patients treated with two different programs according to bone marrow findings.

Thirty adult patients with lymphoblastic lymphoma were treated with two different programs according to bone marrow findings. Bone marrow positive patients were given an ALL-like program: vincristine, daunorubicin, cyclophosphamide and prednisone for induction of remission, CNS prophylaxis, continuous maintenance for three years and monthly reinductions. Bone marrow negative patients were given a conventional lymphoma program with CHOP-Bleo and limited RT on bulky mediastinum without CNS prophylaxis. The CR rate of the whole group was 54% (62% for ALL-treated versus 47% for lymphoma-treated patients; not significantly different), with a median survival for remitters of 28.5 mos. Relapse-free survival of the whole group was 65% at 12 and 25% at 24 mos. Stage IV ALL-treated patients had a median survival of 16.5 versus 10 mos for stage IV lymphoma-treated ones (p = 0.05); the three-years survival was 24 and 10%, respectively. No patients undergoing CNS prophylaxis (ALL-therapy) had neurological complications or late meningeal relapse. The better prognosis of ALL-treated patients, in spite of bone marrow positivity, argues in favor of an ALL-like therapy in all adult lymphoblastic lymphomas, in terms of CR rate, overall survival, and absence of CNS relapse. This therapy must be adopted irrespective of bone marrow findings, and regardless of how localized the lymphoma appears to be.

Adolescent↗

The sicca syndrome in thalassaemia major.

A 20 year old man with beta thalassaemia developed symptoms of the sicca syndrome. His serum contained rheumatoid factor and antinuclear antibodies. A biopsy specimen of labial salivary gland showed large accumulations of haemosiderin within the parenchymal cells of the acini. Although in this case the sicca syndrome could not be definitely distinguished from Sjögren's syndrome, the patient's HLA type was not the one usually associated with Sjögren's syndrome. Histological appearances suggested that the causative factor of the sicca syndrome was iron overload owing to an intensive blood transfusion regimen.

Adult↗

An enzyme-histochemical approach to the study of the human bone-marrow stroma.

The different cells and structures of the bone-marrow stroma were studied with histochemical markers. Reticulum cells, endothelial cells and macrophages were identified by their morphologic characteristics as well as by their different positivity to alkaline-phosphatase, alpha-naphthyl-acetate-esterase and Prussian blue staining. Similarities, as well as distinguishing features of all these cells, possibly related to their common or different origin and functional properties, are described.

Alkaline Phosphatase↗

Red cell aplasia in myelofibrosis with myeloid metaplasia. A distinct functional and clinical entity.

Three patients with myelofibrosis with myeloid metaplasia are described. All three presented with grave anemia and red cell aplasia. Erythroid failure was the functional characteristic of erythropoiesis as resulted from erythrokinetic studies. Bone marrow was hypercellular or normocellular with hyperplasia of granuloblasts and megakaryocytes. Despite absent or mild splenomegaly at diagnosis, massive splenomegaly associated with a leukoerythroblastic blood film developed during the course of the disease. A relative young age (42-47 years), a subacute course of the disease and terminal blast crisis in two of them were the prominent clinical features of the patients. The differentiation from acute or malignant myelofibrosis is discussed. The patients appear to be a distinct subset within myelofibrosis with myeloid metaplasia.

Adult↗

Low-grade malignancy non-Hodgkin's lymphomas: prognostic relevance of their clinicopathologic heterogeneity.

We reviewed 182 consecutive adult patients with low-grade malignancy, non-Hodgkin's lymphomas classified according to the Kiel classification, followed at the Division of Hematology, Policlinico S. Matteo, Pavia, from January 1975 to December 1981, to recognize, in each histopathologic type, important subgroups from the prognostic standpoint. Median follow-up was 36 months. No significant differences were observed in the response rate to conventional therapy (radiotherapy for localized disease, CVP for advanced stages) between the 4 cytologic types. The centrocytic-centroblastic lymphoma with diffuse nodal architecture showed an intermediate-grade malignancy (median survival, 50 months) and underwent cytologic progression to the high-grade malignancy centroblastic type in 10% of the cases. Large-cell centrocytic and polymorphic lymphoplasmacytoid lymphomas had a poor prognosis (median survival less than 30 months) when treated with conventional therapy for favorable histologies, and 6% of the cases transformed into the high-malignancy immunoblastic type. Patients with lymphocytic lymphoma with bulky mediastinum had a median survival of 20 months. The identification of these subgroups with a worse prognosis may have therapeutic implications.

Adult↗

Angiosarcoma of the heart: report of a case in a 9-year-old boy.

A 9-year-old boy with a myocardiac angiosarcoma is described; the tumor was diagnosed by echocardiography and the boy underwent surgery followed by radio and chemotherapy. Death occurred 5 months after surgery, probably due to abdominal hemorrhage from metastases. The young age of the patient, the acute and reversible hepatic damage, the use of echocardiography for diagnosis and the possible role of the extracorporeal circulation in determining the metastases are emphasized.

Child↗

Pulmonary arteriovenous fistulas: an unusual complication of congenital hepatic fibrosis.

A 16-year-old boy with congenital hepatic fibrosis, on whom a portocaval anastomosis was performed because of bleeding esophageal varices, developed lip cyanosis, exertional dyspnea, and clubbing of the fingers 7 years postoperatively. This progressive syndrome was caused by a right-to-left shunt due to the presence of pulmonary arteriovenous fistulas. Increased erythrocyte sedimentation rate, hypergammaglobulinemia, and histological features of cholangitis were also present. It is proposed that this unusual pulmonary complication of congenital hepatic fibrosis could be due either to congenital pulmonary abnormalities or to a chronic unrecognized cholangitis causing a progressive deterioration of liver histology and function, with subsequent opening of pulmonary arteriovenous shunts.

Arteriovenous Fistula↗

Kaposi's sarcoma complicating immunosuppressive therapy for angioimmunoblastic lymphadenopathy with dysproteinemia.

Cutaneous and visceral dissemination of Kaposi's sarcoma (KS) occurred in a patient with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) who had been treated with combination chemotherapy. Three other cases of KS complicating immunosuppressive therapy of AILD have been reported in the literature, and there is evidence to indicate that AILD displays features which are known to predispose to KS. Like in other subjects with profound immunodeficiency (e.g. in young homosexual men), in our patient KS pursued an unusually aggressive course, with involvement of lymph nodes and internal organs as well as the skin. It is concluded that the risk of developing severe KS is a further reason to avoid aggressive combination chemotherapy in patients with AILD, particularly in those of Jewish or Mediterranean ancestry. Even the use of corticosteroids should be reduced to a minimum to avoid immunosuppression, and a conservative approach to treatment seems advisable.

Aged↗

Anomalous erythrocytes produced by rabbits with liver damage.

Rabbit "stress macroreticulocytes" complete their membrane sialic acid content after a short "homing" in the liver. Since also normal rabbit reticulocytes are characterized by a low membrane sialic acid, the role of the liver in the maturation of these cells was investigated. For this purpose red cell modifications consequent to a severe liver steatosis, induced in rabbits by a diet lacking choline, were investigated. Animals with liver damage showed severe anemia and red cell membranes were demonstrated to have a low sialic acid content. Moreover rabbits with severe fatty liver, unlike normal animals, were not able to restore the viability of desialylated normal young erythrocytes. On the basis of these and others evidences we suggest that rabbit liver plays an important role in physiological maturation of reticulocytes.

Animals↗

Cardiomyopathy of doxorubicin in experimental animals, Factors affecting the severity, distribution and evolution of myocardial lesions.

Heart lesions induced in mice, rats, rabbits and dogs by Doxorubicin administered i.v. according to various schedules were studied by light and electron microscopy Vacuolization of myocardial cytoplasm due to distention of the sarcoplasmic reticulum, the T-tubule system and the Golgi vesicles was one of the most common findings. Myocytolysis, clumping and loss of fibrils, fragmentation of sarcomeres, swelling of mitochondria and an increase in lysosomes and residual bodies were also observed. The severity of the cardiomyopathy, quantitatively evaluated by a score system, proved to be dose-dependent. Cardiomyopathy was more severe when the treatment was given in a short period by administration of high doses than when the same cumulative dose was administered as low doses repeated for a long period. The left atrium was more severely affected than the ventricles when high doses were given, whereas it was less affected in animals given low doses. The cardiomyopathy was less severe in animals receiving the same dose in a high volume of solvent and during a long perfusion time. Threshold doses were needed both to induce the cardiomyopathy and to establish it as a progressive disease.

Animals↗