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N Vianelli

Publications and source records attributed to N Vianelli.

52 records · Page 3Linked to original sources
PubMedDate not supplied

Successful treatment of essential thrombocythemia in a pregnancy with recombinant interferon-alpha 2a.

Essential thrombocythemia (ET) in pregnancy is associated with adverse perinatal outcome, which is likely due to thrombosis of the uteroplacental circulation. Obstetrical management is still controversial. Different therapeutic protocols have been adopted, including the use of aspirin, plateletpheresis, and interferon. We herein report a case of ET treated with interferon-2 alpha from 13 weeks gestation until term. Therapy was well tolerated, leading to a linear reduction of platelet and white blood cell count that rapidly returned within normal limits. A healthy, 3,020-g male infant was delivered at 40 weeks gestation. Albeit further experience is required, recombinant interferon-alpha 2a may play an important role in preventing complications in pregnant patients with ET.

Adult↗
PubMedDate not supplied

Ascorbic acid for the treatment of chronic refractory idiopathic thrombocytopenic purpura (ITP).

We describe our experience with ascorbic acid in the treatment of chronic refractory ITP. Nine patients, 5 males and 4 females aged 27-74 years, 4 of whom were previously splenectomized, received the drug at a daily dose of 2 grams. After 2-12 months (median 4) of treatment, a partial response was observed in only one patient, while no response was registered in the other eight. Our data, analyzed together with those of the literature, allow us to conclude that ascorbic acid may not be considered a drug of interest in the treatment of chronic refractory ITP.

Adult↗
PubMedDate not supplied

In vitro inhibition of interferon alpha-2a antiproliferative activity by antibodies developed during treatment for essential thrombocythaemia.

BACKGROUND: Various authors have reported the development of anti-interferon (IFN) antibodies following IFN-alpha treatment for haematological malignancies. So far the methods for detecting these antibodies have not considered the antiproliferative activity of this IFN, which is its most important property in anticancer therapy. METHODS: In this in vitro study we evaluated the ability of anti-IFN alpha-2a neutralising antibodies to inhibit the antiproliferative activity of IFN alpha-2a and lymphoblastoid IFN alpha using megakaryocyte colony growth as the revelatory system. These antibodies were detected in two patients affected by essential thrombocythaemia (ET) who lost their haematological response to IFN alpha-2a, but responded to a subsequent treatment with lymphoblastoid IFN alpha. RESULTS AND CONCLUSION: The results show that the inhibition of megakaryocyte colony growth induced by IFN alpha-2a was totally suppressed in the presence of the two ET patients' sera, whereas the inhibition induced by lymphoblastoid IFN alpha was not significantly affected. These in vitro data demonstrate the high specificity and activity of these antibodies on the antiproliferative effect of IFN alpha-2a.

Adult↗
PubMedDate not supplied

A regimen for antithrombin III substitution in patients with acute lymphoblastic leukemia under treatment with L-asparaginase.

BACKGROUND AND METHODS: Seventeen adult patients with acute lymphoblastic leukemia (ALL) treated with L-asparaginase (20,000 IU/m2 on six alternate days) were infused with antithrombin III (AT III) concentrates (Kybernin P, Behring). Substitution therapy was aimed at increasing the reduced AT III concentration usually found in these patients, since AT III deficiency is thought to be associated with an increased risk of thrombosis. Two schedules of AT III administration, different in dosage, timing and duration were evaluated. The first 7 patients (group A) received a fixed dose of 2,000 U every day for 6 times, starting with the second L-asparaginase (L-ase) infusion, independently of their plasma AT III levels. In the following 10 patients (group B), 20-25 U/Kg b.w. were administered daily for 7 times only when the plasma AT III level was lower than 60% with plasma fibrinogen higher than 100 mg/dl and platelet count higher than 50 x 10(9)/l, or when AT III was below 40%. Thirteen patients who received L-ase without AT III substitution served as controls. RESULTS AND CONCLUSIONS: Both substitution regimens resulted in mean plasma AT III nadir values significantly (p less than 00.1) higher than in the controls. Our data suggest that, in ALL patients receiving L-ase according to the L20 protocol, satisfactory plasma AT III levels may be assured with infusions of 20-25 U/Kg b.w./day for 7-10 days, starting by day 2 of L-ase treatment.

Adult↗
PubMedDate not supplied

Prompt plasma-exchange treatment and coma reversibility in two patients with thrombotic thrombocytopenic purpura.

We report on the cases of two women with acute thrombotic thrombocytopenic purpura (TTP) whose clinical courses were characterized by the onset of a coma state. Prompt commencement of plasma-exchange (PE) treatment led to complete hematological and neurological remission, which can still be observed without any maintenance therapy. No CNS abnormalities were observed in either patient using brain CT and NMR scans.

Adult↗
PubMedDate not supplied

Ticlopidine in the treatment of thrombotic thrombocytopenic purpura: report of two cases.

The observation of two clinical cases make possible an evaluation of the potential therapeutic activity of platelet function inhibitors in thrombotic thrombocytopenic purpura (TTP). In particular, the clinical and hematological effects of ticlopidine (TC), employed alone in two TTP patients, are reported. The mechanism of action of this peculiar antiplatelet drug is mainly represented by the inhibition of fibrinogen binding on the platelet surface. In the first patient, a 45-year-old female in whom plasma-exchange (PE) and corticosteroids (C) led to a partial remission (platelets 80 x 10(9)/l), treatment with TC at a dose of 750 mg/day was carried out, and after 6 weeks a normal platelet count was observed. A complete remission was maintained for 31+ months, even after reduction of the TC dose to 250 mg/day. In the second patient, an 18-year-old female affected by relapsing TTP, a complete remission obtained with PE and C was maintained for 19 months in concomitance with TC treatment, started at a dose of 750 mg/day and lowered to 250 mg/day. After 11 months of treatment at this low dosage there was a relapse (platelets 20 x 10(9)/l), but the increase of the TC dose to 750 mg/day in a few weeks induced a complete remission again. These data, in accord with a few other recent preliminary reports, suggest that TC, even alone, may play an interesting role in the management of TTP patients.

Adolescent↗
PubMedDate not supplied

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↗
PubMedDate not supplied

Negligible hemostatic toxicity of intermediate-dose Erwinase in adult patients with acute lymphoblastic leukemia: preliminary data.

The hemostatic toxicity of low dose L-asparaginase from Erwinia carotovora (Erwinase) has been reported to be negligible in adult patients with acute lymphoblastic leukemia (ALL); conversely, no consistent data have been obtained when Erwinase is administered at intermediate doses. We report preliminary clinical and laboratory hemostatic data from 10 adult patients with ALL treated during induction phase with intermediate doses of Erwinase (20,000 IU/m2s.c. every other day, for a total of six administrations). No thrombotic or hemorrhagic events were registered and the mean values of PT, aPTT, fibrinogen, antithrombin and D-dimer did not change during treatment. Only one patient showed a decrease of antithrombin (48% on day 8) requiring temporary suspension of Erwinase therapy. These data suggest that intermediate doses of Erwinase also have negligible hemostatic toxicity in adult patients with ALL.

Adolescent↗
PubMedDate not supplied

Increased P-selectin plasma levels in patients with thrombotic thrombocytopenic purpura.

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a rare vascular disorder of unknown etiology. There is evidence to support the hypothesis that platelets and endothelium play a pivotal pathogenetic role. Immunological assays for plasma thrombomodulin and P-selectin levels have recently been made available and they allow simple evaluation of endothelial damage and endothelial/platelet activation, respectively. In this study, we measured the plasma levels of thrombomodulin, P-selectin and von Willebrand factor in 9 TTP patients during active disease and at the time of complete remission (CR). METHODS: Thrombomodulin, P-selectin and von Willebrand factor were measured by enzyme immunoassay. RESULTS: Mean thrombomodulin and von Willebrand factor plasma values were always within the normal range. P-selectin plasma levels, both in the active phase of the disease and in CR (median 312 and 185 ng/mL, respectively), were significantly higher than in normal controls (mean 96 +/- 35 ng/mL, mean 88 ng/mL; p < 0.05). However, the mean value of P-selectin in CR (median 185 ng/mL) was significantly lower than that observed at diagnosis (p < 0.05). In addition, an inverse relationship between P-selectin plasma levels and platelet count (r = -0.526; p = 0.03) was observed. CONCLUSIONS: These findings suggest that activation of platelets and/or endothelium may play a relevant role in the pathogenesis of TTP.

Adult↗
PubMedDate not supplied

Autoimmune mediated thrombocytopenia associated with the use of interferon-alpha in chronic myeloid leukemia.

We report on two patients with Ph+ chronic myeloid leukemia (CML) in chronic phase who developed severe thrombocytopenia during treatment with interferon-alpha 2A (IFN-alpha 2A). In both cases, we detected the presence of platelet-associated antibodies (PAIg) by autoimmune flow cytometry. We postulated an immune mediated platelet destruction mechanism; corticosteroid therapy was employed and interferon therapy was withdrawn, resulting in an increase in platelet count and a reduction of PAIg. Our observation reports the detection of PAIg associated with IFN-alpha 2A therapy in CML and suggests that this immunomodulant drug could induce thrombocytopenia through a mechanism other than antiproliferation.

Adult↗
PubMedDate not supplied

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↗