Cytological evidence of plasmacytoma of the thyroid.
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Cytodiagnosis↗
Biomedical subjects
E Ascari
Publications and source records attributed to E Ascari.
Intravenous prostacyclin (as epoprostenol) infusion in thrombotic thrombocytopenic purpura. Four case reports and review of the literature. Italian Cooperative Group for Thrombotic Thrombocytopenic Purpura.
BACKGROUND: The enhanced platelet aggregation which is observed in TTP, was suggested to be due to an imbalance between unknown agents insulting endothelial wall and defense factors, such as prostacyclin (PGI2). Several reports suggested an aberration of PGI2 activity as a critical step in the pathogenesis of TTP. Therefore, PGI2 was proposed as an alternative treatment for TTP patients. METHODS: We report the results obtained with increasing doses (from 2 ng/Kg/min to 10 ng/Kg/min in 5 days) of PGI2-as epoprostenol-in 4 TTP patients from the retrospective series of the Italian Cooperative Group who were considered resistant to conventional plasma-exchange (PE)-based treatments. RESULTS: Despite PGI2 infusion, 2 patients died, while the extant 2 achieved stable complete remission. Notably, the only patient whose PE was administered with adequate frequency and for an adequate period of time, and thus the only unquestionably PE-resistant patient, was also resistant to PGI2 infusion. Major side-effects were few and observed at the highest doses. CONCLUSIONS: In our experience and from the analysis of the literature, which, as far as we know, includes only 23 patients treated with PGI2-like substances, the role of PGI2 in the treatment of TTP appears to be modest. Maybe the identification of subgroups of TTP patients exhibiting some defects in PGI2 metabolism, together with the use of more manageable PGI2 analogs, such as iloprost, could revive interest in these molecules in the future.
Adult↗
Estimate of expected survival at diagnosis in Hodgkin's disease: a means of weighting prognostic factors and a tool for treatment choice and clinical research. A report from the International Database on Hodgkin's Disease (IDHD).
PURPOSE: The aim was to identify a mathematical model that, when fitted with the survival time distribution of a Hodgkin's disease population, would provide a reliable estimate of expected survival at diagnosis for any new Hodgkin patient. This model would be based upon a multivariable selection of the best prognostic factors evaluable at diagnosis and its forecast could be of assistance in the choice of treatment. METHODS: The study sample consisted of the 5,023 patients whose basic clinical information was collected into the IDHD. These were people treated with standard protocols over the last two decades in 18 institutions. Several survival time distributions (exponential, Weibull, Gompertz, log-logistic and log-normal) were investigated to find the one that best fit the data and to relate its parameters to patient prognostic characteristics. RESULTS: The log-normal model provided the best fit for the data. The most statistically significant prognostic covariates were stage, age, histotype, B symptoms, serum albumin, sex and involved area distribution. Mediastinal, extranodal or bone marrow involvement, erythrocyte sedimentation rate, hemoglobin, serum alkaline phosphatase and lactate dehydrogenase did not add significant information. An equation containing these seven variables was derived to estimate median survival. Five distinct prognostic classes were identified by four cut-off values for this estimate. CONCLUSIONS: Direct use of estimated median survival or allocating each patient into one of the five prognostic classes allows better tailoring of clinical strategies according to prognostic characteristics, more accurate patient stratification and evaluation of results in clinical trials and metaanalyses. Instructions are given for using this tool for both clinical and investigational purposes.
Adolescent↗
Primary splenic lymphoma: does it exist?
The number of primary splenic lymphomas being reported is increasing despite the rarity of this malignancy, but what really constitutes a lymphoma arising primarily in the spleen is still a matter of discussion. The authors choose the "restrictive" definition of a lymphoma involving the spleen and the splenic hilar lymph nodes only. In this way, the risk of epidemiologic or clinical overestimation is avoided. The clinical features of this condition are characterized by non specific symptoms and signs, while the prevailing histology is that of a low-grade or intermediate-type lymphoma. Disease spreading outside of the spleen and its hilar lymph nodes is the single most important factor associated with an unfavorable prognosis. From this usual clinical picture, two distinct nosologic entities can be outlined on the basis of histologic and immunologic peculiarities: splenic lymphoma with circulating villous lymphocytes and marginal-zone splenic lymphoma. The former arises from follicular center cells and is characterized by hypersplenism, variable percentages of circulating villous lymphocytes and, frequently, a monoclonal gammopathy. The latter originates from a peculiar splenic B-cell structure separated by the mantle zone. The proliferating cells are medium-sized KiB3-positive lymphocytes with round or cleaved nuclei and pale cytoplasm, which surround follicular centers and infiltrate the mantle zone. It is interesting that marginal-zone lymphoma cells share some of the characteristics of the lymphocytes involved in both lymphomas of mucosa-associated lymphoid tissue and the B-monocytoid lymphomas. Splenectomy is still the most effective therapy for all primary splenic lymphomas.
Humans↗
Silymarine during maintenance therapy of acute promyelocytic leukemia.
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Adult↗
Acute promyelocytic leukemia: morphological and clinical features.
BACKGROUND AND METHODS: Acute promyelocytic leukemia (APL) is not a morphologically homogeneous entity: to verify whether there is any relationship between this heterogeneity and other biological and clinical aspects, we studied 43 cases of APL with morphological, cytochemical, cytogenetic and immunological methods. RESULTS: Three morphological categories were present: a classic hypergranular type (30 cases), a microgranular type (6 cases) and a form with basophilic granules (M3b) that stained metachromatically with toluidine blue (7 cases). In all these groups there were cases with cytochemical features of both myeloid and monocytic type (alpha-naphthyl-acetate esterase positive). No immunological and cytogenetic differences were observed; the morphological variant with basophilic granules was more frequent in females; age distribution was not related to the morphological subtype; organomegaly was extremely rare in M3b. A low white blood cell count was constant in M3b, whereas no differences were observed in hemoglobin and platelet values. Severity of bleeding was worst in the group with toluidine blue metachromasia; this and the microgranular type had poor prognosis. CONCLUSIONS: Our study confirms the importance of identifying different cytologic categories in APL. In particular we focused our attention on a new variant with basophilic granules.
Adolescent↗
Iron-deficiency anemia resistant to iron therapy.
Iron-deficiency anemia resistant to iron administration is a common clinical problem. This paper analyzes the causes of primary failure of iron therapy. From a practical point of view, the main recommendation is that a physician should not be satisfied with simplistic explanations for "refractoriness". In fact, failure to respond to treatment is always the result of a diagnostic or therapeutic error which must be corrected in order to achieve the natural result of all substitution therapies: replenishment.
Absorption↗
Characterization of the biophysical properties of human erythroblasts as a preliminary step to the isolation of fetal erythroblasts from maternal peripheral blood for non invasive prenatal genetic investigation.
BACKGROUND AND OBJECTIVE: Fetal erythroblasts in maternal circulation represent a valuable source of fetal cell material which can be obtained with non-invasive procedures that do not endanger the fetus. Physical separation techniques have been invaluable in the isolation and characterization of different cells. There are basically two principles that have been used most successfully: separation according to density and separation according to size. In order to determine whether physical separation procedures are capable of purifying human erythroblasts, the biophysical characteristics of these cells were determined. METHODS: Bone marrow particles were obtained from formal adults and peripheral blood buffy coats from blood banks. A single cell suspension was initially fractionated by buoyant density gradient centrifugation. Fractions enriched in erythroblasts were pooled and further processed by velocity sedimentation in order to take advantage of the differences in size of erythroblasts and other cells. RESULTS: Density distribution curves were drawn after density gradient centrifugation for the different cell types present in the starting cell samples. Separation of the erythroblast-enriched density fractions by velocity sedimentation was successful and a highly purified population of erythroblasts was obtained. Cell size distribution of the different cell types was determined. INTERPRETATION AND CONCLUSIONS: This initial study defines the biophysical properties (size and density) of human erythroblasts in bone marrow and peripheral blood and is a necessary preliminary step in setting up the optimal procedure for the isolation of fetal erythroblasts from maternal peripheral blood in sufficient amounts and purity for prenatal non-invasive genetic investigation.
Adult↗
Antiplatelet agents in thrombotic thrombocytopenic purpura (TTP). Results of a randomized multicenter trial by the Italian Cooperative Group for TTP.
BACKGROUND AND OBJECTIVE: Antiplatelet agents are often included in plasma exchange-based regimens for thrombotic thrombocytopenic purpura (TTP) patients; however, the opportuneness of their use in TTP is still controversial. The italian Cooperative Group for TTP carried out a randomized trial to investigate their actual effectiveness, both in acute TTP and as maintenance treatment. METHODS: Seventy-two TTP patients were randomized to receive plasma exchange and steroids with (group B) or without (group A) aspirin and dipyridamole. Treatment efficacy was evaluated after 15 days and salvage treatments were also considered for non-responders. Upon disease remission, the patients already treated with antiplatelet agents received ticlopidine for one year. RESULTS: Regarding the treatment of acute phase TTP, similar overall response rates were observed in the two groups (91.4% in group B vs. 75.6% in group A), but lower mortality rates were observed at 15 days in the patients treated with antiplatelet agents; as a matter of fact, 5 patients from arm A died in the first 15 days (13.5%) versus only one in arm B (2.8%). These figures, while not statistically significant, seem to suggest that antiplatelet agents might be useful in preventing deaths in acute TTP; moreover, bleeding did not worsen in antiplatelet agent-treated patients. As for the role of maintenance treatment, our results support the efficacy and safety of one-year ticlopidine therapy since the current relapse rate is significantly higher in non-treated patients; as a matter of fact, 6 patients (21.4%) in the non-ticlopidine group and only 2 (6.25%) in the ticlopidine group relapsed (P = .0182 in favor of maintenance treatment). INTERPRETATION AND CONCLUSIONS: Our results suggest the usefulness of antiplatelet agents in the treatment of acute phase TTP patients. Moreover, one-year ticlopidine maintenance therapy appears to be beneficial in preventing TTP relapses; however, only the completion of an adequate follow-up for all patients will definitively confirm this observation.
Adult↗
Idarubicin and cytosine arabinoside in the induction and maintenance therapy of high-risk myelodysplastic syndromes.
BACKGROUND AND OBJECTIVE: Recently, the results of a few pilot studies have shown the efficacy of the association of idarubicin (IDA) and cytosine arabinoside (Ara-C), already successfully employed in acute myeloid leukemia (AML), for remission induction in patients with myelodysplastic syndrome (MDS). We set out to evaluate in a multicenter study the efficacy and tolerability of an intensive therapy with IDA and Ara-C in patients with RAEB and RAEB-t, the rate and duration of CR and the overall survival in adults treated with full doses and in the elderly treated with lower doses; furthermore, we investigated the efficacy of low-dose maintenance chemotherapy. METHODS: Pretreated adult patients with de novo RAEB and RAEB-t, meeting at least one of the following criteria, were included: neutrophils < 0.5 x 10(9)/L or moderate neutropenia with infectious episodes, platelets < 30 x 10(9)/L or moderate thrombocytopenia but with bleeding symptoms, transfusion > 4 red cell units/months, rapid increase of bone marrow blasts. Induction treatment consisted of a cycle with IDA and Ara-C. Adult patients less than 65 years old were treated with the following doses: Ara-C 1 g/m2/day i.v. 6-hour infusion, on days 1-4, IDA 10 mg/m2/day i.v., on days 1-3. Elderly patients (> or = 65 yrs) were treated with lower doses: Ara-C 1 g/m2/day 6 hours infusion, on days 1 and 2, IDA 10 mg/m2/day i.v., on days 1 and 2. Responders followed a consolidation course identical to induction. RESULTS: From February 1994 to February 1997, 25 patients were enrolled, 20 males and 5 females aged between 22 and 76, 10 were > or = 65 years old, 7 had RAEB and 18 had RAEB-t. Twelve cases (48%) achieved complete remission (CR), 7 cases (28%) achieved partial remission, 4 patients were resistant and two patients (8%) died during the aplastic phase. A significantly higher CR rate was found in younger patients (p = 0.036), while gender, FAB subtype, presence of Auer rods, cytogenetic findings, and the interval from diagnosis to treatment did not significantly influence CR achievement. INTERPRETATION AND CONCLUSIONS: Our results show that in de novo RAEB and RAEB-t, the employed treatment with IDA and Ara-C is associated with satisfactory frequency of response with acceptable toxicity.
Adult↗
Idarubicin and cytosine arabinoside in the induction and maintenance therapy of high-risk myelodysplastic syndromes.
BACKGROUND AND OBJECTIVE: Recently, the results of some pilot studies have shown the efficacy of the association of idarubicin (IDA) and cytosine arabinoside (Ara-C), already successfully employed in acute myeloid leukemia (AML), for remission induction in patients with myelodysplastic syndrome (MDS). We set out to evaluate in a multicenter study the efficacy and tolerability of an intensive therapy with IDA and Ara-C in patients with RAEB and RAEB-t, the rate and duration of complete remission, and the overall survival in adults treated with full doses and in the elderly treated with lower doses; furthermore, we investigated the efficacy of low-dose maintenance chemotherapy. METHODS: Pretreated adult patients with de novo RAEB and RAEB-t, meeting at least one of the following criteria, were included: neutrophils < 0.5 x 10(9)/L or moderate neutropenia with infectious episodes, platelets < 30 x 10(9)/L or moderate thrombocytopenia with bleeding symptoms, transfusion > 4 red cell units/months, rapid increase of bone marrow blasts. Induction treatment consisted of a cycle with IDA and Ara-C. Adult patients less than 65 years old were treated with the following doses: Ara-C 1 g/m2/day i.v. 6-hour infusion, on days 1-4, IDA 10 mg/m2/day i.v., on days 1-3. Elderly patients (> or = 65 yrs) were treated with lower doses: Ara-C 1 g/m2/day 6 hours infusion, on days 1 and 2, IDA 10 mg/m2/day i.v., on days 1 and 2. Responders followed a consolidation course identical to induction. RESULTS: From February 1994 to February 1997, 25 patients were enrolled, 20 males and 5 females aged between 22 and 76, 10 were > or = 65 years old, 7 had RAEB and 18 had RAEB-t. Twelve cases (48%) achieved complete remission (CR), 7 cases (28%) achieved partial remission, 4 patients were resistant and two patients (8%) died during the aplastic phase. A significantly higher CR rate was found in younger patients (p = 0.036), while gender, FAB subtype, presence of Auer rods, cytogenetic findings, and the interval from diagnosis to treatment did not significantly influence CR achievement. INTERPRETATION AND CONCLUSIONS: Our results show that in de novo RAEB and RAEB-t, treatment with IDA and Ara-C is associated with satisfactory frequency of response with acceptable toxicity.
Adult↗
[Gastrointestinal stromal tumor as the cause of intestinal hemorrhage: description of a clinical case].
The term gastrointestinal stromal tumor describes a heterogeneous group of tumors of mesenchymal origin with particular histologic features. Their classification has recently been made possible thanks to numerous immunohistochemical and ultrastructural studies. We report the case of a patient who came to our attention because of serious anemia due to a gastrointestinal stromal tumor located between the second and third portion of the duodenum. This pathology, although not frequent, should be considered in the differential diagnosis of gastrointestinal bleeding. In these cases it is important to perform careful endoscopic inspection to the third portion of th duodenum, even when previous tracts evidence lesions that could be responsible for the patient's symptoms.
Duodenal Neoplasms↗