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Biomedical subjects

E Ascari

Publications and source records attributed to E Ascari.

At least 145 records · Page 8Linked to original sources
PubMed1983

Proliferative activity of bone marrow cells in primary dysmyelopoietic (preleukemic) syndromes.

The proliferative activity of bone marrow cells was studied in 24 patients with primary dysmyelopoiesis by means of flow cytometry and 3H-TdR autoradiography. Abnormal DNA content was found in two cases with aneuploid karyotypes. DNA content typical of a diploid population was observed in all patients with normal karyotype and in three patients with chromosomal aberrations. The fraction of bone marrow cells in S- and G2-phase was higher in primary acquired sideroblastic anemia and refractory anemia (without excess of blasts) than in refractory anemia with excess of blasts and chronic myelomonocytic leukemia. Regardless to the diagnosis, the patients with low fraction of cells in S- and G2-phase had short survival time and showed high rate of evolution into acute nonlymphoblastic leukemia. The labeling (LI) and mitotic (MI) indexes of both erythroblasts and granulocytic cells were decreased in nearly all patients. The lowest values of LI and MI of the granulocytic compartment were found in the patients who subsequently developed acute leukemia. These data suggest that cytokinetic analysis allows investigators to achieve useful information on the stage of disease in the dysmyelopoietic syndromes.

Adult↗
PubMed1983

Severe pruritus should be a B-symptom in Hodgkin's disease.

Pruritus was evaluated with respect to frequency, intensity, and prognostic significance in 360 patients with Hodgkin's disease. In order to discriminate severe from mild pruritus, the following criteria was used: (1) multiple excoriations; (2) ineffectiveness of local and systemic antipruritics; and (3) improvement with either radiotherapy or chemotherapy. Ninety of 360 patients had mild itching on admission and showed the same survival as the 249 nonitching cases; 21 patients presented with severe pruritus, which was also generalized, and showed a statistically shorter survival than that of mild and non-itching cases. This comparison was carried out between patients homologous with respect to sex, age, stage, A or B category, and histotype. Analysis of the data pointed out that the third requirement, i.e., improvement with antineoplastic therapy, is not substantial; moreover, for staging purposes, it is preferably replaced by the requisite of generalized pruritus. The intrinsically poor prognosis related to severe pruritus may be important for treatment, when this symptom occurs in early stages or without other systemic symptoms. The inclusion of severe pruritus among the Ann Arbor criteria for definition of the B-clinical category is proposed.

Adolescent↗
PubMed1983

Juvenile idiopathic haemochromatosis: a life-threatening disorder presenting as hypogonadotropic hypogonadism.

It is generally believed that idiopathic haemochromatosis is exclusively a disease of middle age, affecting primarily men. We describe here four cases of idiopathic haemochromatosis having onset of symptoms before or around the age of 20 years. Other similar cases have previously been reported. In this juvenile form, males and females appear to be equally affected. These subjects may have a history of unexplained abdominal pain, present with hypogonadotropic hypogonadism, and, unless proper treatment is started, die early because of cardiac dysfunction. In this regard, their clinical course is very similar to that of well-transfused thalassemia major. Thus, early diagnosis is even more important in the juvenile form than in the adult form of idiopathic haemochromatosis. We suggest that evaluation of body iron stores should be performed as a screening procedure in young subjects with hypogonadotropic hypogonadism and/or cardiac dysfunction.

Adult↗
PubMed1983

Basic and acidic isoferritins in the serum of patients with Hodgkin's disease.

Ferritin concentration has been measured in the serum of patients with Hodgkin's disease (HD) by radioimmunoassays with monospecific antibodies to liver (basic) and HeLa (acidic) ferritin. Elevated levels of serum ferritin with the liver ferritin assay were found only in patients with systemic disease, and were associated with low serum iron. Basic ferritin levels returned promptly to normal when complete remission was achieved. High levels of serum ferritin with the HeLa ferritin assay were found in 94% of all untreated patients. Acidic ferritin concentration was not related to systemic symptoms or alterations of iron metabolism, and returned to within the normal range only 1-2 yr after complete remission. These findings suggest that basic and acidic isoferritins can be distinguished in terms of biological and clinical significance. Basic ferritin is synthesized by the reticuloendothelial cells and the high values found in patients with systemic symptoms are compatible with the non-specific changes known to occur in the reticuloendothelial system during inflammation. In patients with untreated HD an elevated serum concentration of basic ferritin can be considered a marker of systemic symptoms and, therefore, an unfavourable prognostic factor. Acidic ferritin may be derived from abnormal lymphocytes and/or monocytes, including malignant cells, and its serum concentration may be of value in following the course of remission.

Adolescent↗
PubMed1983

Iron loading in congenital dyserythropoietic anaemias and congenital sideroblastic anaemias.

The relationship between body iron status, degree of anaemia, erythroid expansion, age and sex has been studied in eight patients with congenital dyserythropoietic anaemia (CDA) and two patients with congenital sideroblastic anaemia, who had received no or very few blood transfusions and no medicinal iron during the course of their illness. All patients had increased iron stores. Iron load was mild in three women in the reproductive age and severe in two men, in middle age, who had evidence of parenchymal organ dysfunction. Iron loading, as judged by the plasma ferritin concentration, was independent of the degree of anaemia while it was closely related to the patient's age and the degree of increase in the total erythropoietic activity. It is concluded that patients with CDA or congenital sideroblastic anaemia are at high risk of developing haemochromatosis in middle age. Prophylactic phlebotomy or iron chelation therapy should be considered for such patients.

Adolescent↗
PubMed1983

Ferritin in the red cells of normal subjects and patients with iron deficiency and iron overload.

Red cell ferritin was measured in normal subjects and patients with iron deficiency and iron overload by means of radioimmunoassays with antibodies to liver (basic) and heart (acidic) ferritins. In most of the subjects examined, red cells were found to contain greater amounts of heart-type than liver-type ferritin. The basic ferritin content reflected the abnormal body iron status both in iron deficiency and iron overload while the acidic ferritin content was less closely related to the iron status. The two immunologically different red-cell ferritins probably represent distinct ferritin molecules and may have different metabolic functions within haem-synthesizing erythroid cells.

Anemia, Aplastic↗
PubMed1983

Cold haemagglutinin disease with severe anaemia, reticulocytopenia and erythroid bone marrow.

A quantitative assessment of total, effective and ineffective erythropoiesis, and mean red cell life-span was performed in 2 patients with idiopathic cold-haemagglutinin disease (CHAD) who had severe anaemia, reticulocytopenia and erythroid bone marrow. In both cases, ineffective erythropoiesis represented the major factor in the pathogenesis of the anaemia. The most likely explanation of this was that cold antibodies had some effect on the maturing red-cell precursors. Ineffective erythropoiesis has already been shown in patients affected by auto-immune haemolytic anaemia due to warm antibodies with reticulocytopenia and erythroid bone marrow. Therefore, it is apparent that both warm and cold antibodies may cause not only peripheral destruction of mature red cells but also intramedullary death of red-cell precursors.

Aged↗
PubMed1983

Biologic and clinical significance of red cell ferritin.

Red cell ferritin was measured in normal subjects and patients with disorders of iron metabolism, inflammation, liver dysfunction, impaired hemoglobin synthesis, and increased red cell turnover by means of radioimmunoassays with antibodies to liver (basic) and heart (acidic) ferritins. The normal mean values for basic and acidic ferritin were 8.9 and 22.7 altogram (ag)/cell, respectively. The red cell ferritin content reflected changes occurring in tissues both in iron deficiency and iron overload. Basic ferritin was more closely related to the body iron status than acidic ferritin, and the acidic/basic ferritin ratio was increased in iron deficiency and decreased in iron overload. The major factor determining the red cell ferritin content appeared to be the transferrin saturation, that is, the distribution of iron between monoferric and diferric transferrin. This is in keeping with recent data indicating a competitive advantage of diferric transferrin in delivering iron to erythroid cells. In addition, the red cell ferritin content was increased in thalassemic patients with normal iron status, appearing to be inversely related to the rate of hemoglobin synthesis. The determination of red cell ferritin, based on a commercially available basic ferritin assay, can have clinical application. It can be used for evaluating the adequacy of the iron supply to the erythroid marrow, particularly in patients with increased red cell turnover. Moreover, it may be useful in evaluating the body iron status in patients with hemochromatosis and liver disease.

Adolescent↗
PubMed1982

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↗
PubMed1982

Sequential vincristine, arabinosylcytosine and adriamycin in acute leukemia: cytologic and cytokinetic studies.

Cytokinetic and cytocidal effects exerted on peripheral blood blasts by sequential administration of vincristine (VCR) 2 mg on day 1, arabinosylcytosine (Ara-c) 50-60 mg/m2/12 hr from day 2-4), and Adriamycin (ADM, Farmitalia, Milan, Italy, 40-60 mg/m2 on day 5) have been examined in 22 courses of treatment performed on 12 patients with nonlymphoblastic and in 4 with lymphoblastic acute leukemia. In 4 patients, the bone marrow blasts wee examined before and also after VCR-Ara-c administration. In vitro tritiated thymidine autoradiography and propidium iodide-DNA flow cytometry were employed for kinetic studies. Blasts disappeared from blood with a median half time of 35 hr. After VCR-Ara-c administration, a significant increase in labeling index (LI) and in the aliquot of cells with DNA content between the diploid (2n) and the tetraploid (4n) values was observed in 80% of the courses in peripheral blood blasts and in all courses in bone marrow blasts. The median grain count over the labeled nuclei was decreased, and the 4n cell percentage and the bone marrow blast mitotic index did not increase. These findings suggest that the increase in the S phase fraction of blast population is due to cell synchronization. Increase in the S phase appears to heighten the cytocidal effect of ADM. The aliquot of the blasts cleared from blood after ADM were in fact related directly to the degree of labeling index increase observed during the previous administration of VCR and Ara-c.

Acute Disease↗
PubMed1982

New clinical criteria for the assessment of liver involvement in Hodgkin's disease.

The hepatic involvement in Hodgkin's disease, histologically verified in 133 patients who underwent laparotomy or laparoscopy, proved to be singly related to the following clinical findings: result of the liver isotopic scan, liver and/or spleen enlargement, serum albumin less than or equal to 3.5 g/dl, GOT and/or GPT greater than or equal to 20 mU/ml, serum alkaline phosphatase (SAP) greater than or equal to 210 mU/ml, BSP retention at 45 min greater than or equal to 6.5% and ESR greater than or equal to 51 mm at 1 hr. Such clinical findings were jointly evaluated and further selected by means of a logistic discriminant analysis, and the simplest function with the best discriminant ability between involved and non-involved liver was made by liver scan, spleen enlargement, BSP retention and GOT (89.5% of correct diagnoses). Since the Ann Arbor clinical criteria for liver involvement showed correct diagnoses in 69-80% of the cases, more reliable criteria can be proposed. So, liver involvement is highly probably (a) when three or more of the five variables indicated above are abnormal, or (b) when a markedly abnormal liver scan is associated with alteration of at least one of the other four parameters: otherwise liver will be non-involved.

Adolescent↗
PubMed1982

Quantitative evaluation of erythropoietic activity in dysmyelopoietic syndromes.

Based on the morphological appearances of the bone marrow and peripheral blood, 43 patients with dysmyelopoietic syndromes were categorized into four types: refractory anaemia with excess of blasts, chronic myelomonocytic leukaemia, primary acquired sideroblastic anaemia and refractory anaemia with cellular marrow, without excess of blasts and/or ring sideroblasts. Ferrokinetics allowed three distinct groups of patients to be defined. All cases of refractory anaemia with excess of blasts and chronic myelomonocytic leukaemia were classified in the same group. They were characterized by relative marrow failure and had a high likelihood of developing acute leukaemia. At the other end of the spectrum, individuals with primary acquired sideroblastic anaemia had high erythropoietic activity which was largely ineffective. They had a benign clinical course without evidence of leukaemic transformation. In the middle group, in terms of erythropoietic activity, lay patients with refractory anaemia with cellular marrow and a few individuals with primary acquired sideroblastic anaemia. Their clinical course and risk of developing acute leukaemia were intermediate between the other two groups. These findings indicate that separate entities may exist within the spectrum of dysmyelopoietic syndromes. In clinical practice, they may be recognized by morphological studies and other simple laboratory means.

Adolescent↗
PubMed1982

Serum alpha-1-acid-glycoprotein, haptoglobin and C3 in Hodgkin's disease. A comparison with other acute-phase indicators.

107 determinations of alpha 1-acid-glycoprotein (alpha 1S), haptoglobin (Hp) and complement fraction 3 (C3) were made in 85 consecutive patients with Hodgkin's disease, both in acute phase (77 measurements) and in complete remission (30 measurements). Only alpha 1S and Hp showed much higher levels in untreated disease than in remission, and elevation of alpha 1S in activity of the disease is correlated to advanced stages and - less significantly - to severe histology. The ability of alpha 1S, Hp and C3 to discriminate between activity and remission was compared with that of erythrocyte sedimentation rate (ESR), alpha 2-globinaemia (alpha 2), fibrinogenaemia (Fb), plasma copper (Cu) and iron (Fe), all data being collected at the same point in time in each patient. The well-known discrimination ability of combined Cu and Fe (75%) can be further improved by alpha 1S (81%) much more than by Hp, C3, ESR, alpha 2 and Fb singly computed and nearly up to the maximum allowed by the eight indexes together (82%).

Acute Disease↗
PubMed1982

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↗