Thymopentin in the treatment of myelodysplastic syndromes.
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Aged↗
Biomedical subjects
M C Tirindelli
Publications and source records attributed to M C Tirindelli.
Disseminated intravascular coagulation and myocardial infarction in a haemophilia B patient during therapy with prothrombin complex concentrates.
A case of disseminated intravascular coagulation (DIC) and fatal myocardial infarction in a haemophilia B patient is described. DIC occurred after 4 days of therapy with unactivated prothrombin complex concentrates during the post-operative period. Therapy with fresh frozen plasma, heparin and antithrombin III concentrates was started without efficacy; after autopsy myocardial infarction was evident.
Blood Coagulation Factors↗
[Acute myeloid leukemia: prognostic factors].
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Adult↗
Long-term immunogenicity of a plasma-derived hepatitis B vaccine in HIV seropositive and HIV seronegative hemophiliacs.
Short-term studies indicate that hepatitis B vaccines are safe and satisfactorily immunogenic in hemophiliacs. The duration of immunity in these immunocompromised patients, however, is not known. To determine this, we studied 78 hemophiliacs prospectively 2, 3, and 4 years after the initial vaccination with a plasma-derived vaccine given as three monthly injections followed by a fourth booster injection at month 14. The duration of immunity clearly depended on whether the patients were infected with the human immunodeficiency virus (HIV). In HIV seronegative hemophiliacs (n = 67), there was a progressive decline in titers of antibody to the hepatitis B surface antigen (anti-HBs), but antibody was still detectable 4 years later in all of them. From the curves of decline of antibody titers, it appears that there is no need to revaccinate patients for at least 5 to 6 years. The HIV seropositive hemophiliacs (n = 11) not only started from much lower anti-HBs titers, but 5 of 11 lost anti-HBs. None of the 45 patients treated with concentrates during the postvaccination period developed serologic signs of hepatitis B, even though 6 of them had come into contact with live or inactivated hepatitis B virus as shown by the occurrence of spontaneous anamnestic antibody responses. This vaccine and schedule of vaccination afford a prolonged duration of immunity in HIV seronegative hemophiliacs, but HIV seropositive hemophiliacs have a risk of losing immunity early.
Adolescent↗
Evaluation of postoperative blood coagulation changes in elderly patients undergoing major surgery.
18 elderly patients submitted to major surgery for malignancies or other disease were studied to assess the relationship between changes of blood coagulation factors and inhibitors in the early post-operative period and the appearance of lower limb deep vein thrombosis. A decrease in serum antithrombin III (AT III) Protein C antigen (PC: Ag) and Plasminogen activity (PLG) levels from the second to the fourth postoperative day, together with a simultaneous increase in serum fibrinogen (FG) and von Willebrand Factor (vWF:Ag) antigen levels was observed. In 8 patients, PC:Ag levels dropped below the limit considered at risk to develop DVT (less than 60 U/dl). A patient with the lowest PC:Ag levels had deep vein thrombosis From the analysis of data it was concluded that in the postoperative period, blood coagulation changes occur in elderly patients, predisposing to the risk of deep vein thrombosis.
Aged↗
Clinical evaluation of subcutaneously administered DDAVP.
1-deamino-8D-arginine vasopressin was given subcutaneously at the dosage of 0.3 micrograms/Kg. b.w. to 24 mild factor VIII deficient patients (16 mild, 2 moderate hemophiliacs and 6 patients with von Willebrand's Disease), to treat bleedings (10 episodes) or to prevent bleeding during and after dental extractions (6 extractions) and surgery (11 interventions). None of the patients who underwent surgery bled. The vasopressin analogue was effective in the early treatment of muscle hematomas and promptly stopped all mucosal hemorrhages. Most of the patients treated for "spontaneous" bleedings performed self-injections at home. The drug was administered in two pharmaceutical forms (4 and 40 micrograms/ml): no differences in the clinical outcome were found. No significant side effects were recorded. The subcutaneous route of DDAVP administration thus seems to be particularly useful (mainly in the concentrated pharmaceutical form) in treating mild factor VIII deficiencies even on self- and home-treatment basis.
Deamino Arginine Vasopressin↗
Immunogenicity of a recombinant hepatitis B vaccine in hemophiliacs.
Yeast-recombinant vaccines against hepatitis B virus (HBV) are now available, but there is no information about whether or not they are immunogenic in patients with hemophilia and other congenital bleeding disorders. Twenty micrograms of a recombinant vaccine expressing the adw serotype of the hepatitis B surface antigen (HBsAg) were given to 41 patients negative for HBV markers and again after 1 and 6 months. Ten percent of the vaccinees had anti-hepatitis B surface antibody (anti-HBs) responses, with titers of 10 mIU/ml or more, 1 month after the first dose of vaccine. The percentage of anti-HBs-positive patients increased to 54% after the second dose and to 98% after the third dose, with only one non-responder. Hence, the recombinant vaccine was immunogenic, with percentages of seroconversion and anti-HBs titers similar to those achieved with plasma-derived vaccines.
Adolescent↗
Concentrated DDAVP: further improvement in the management of mild factor VIII deficiencies.
This study was carried out to evaluate the pharmacological efficacy of a new concentrated 1 Deamino - (8-D-arginine)-vasopressin (DDAVP) preparation. Concentrated DDAVP (C-DDAVP), (40 micrograms/mL) was given subcutaneously (s.c.) in hemophilia and von Willebrand Disease (vWD), and the response was evaluated in terms of factor VIII/vWF (VIII/von Willebrand Factor) complex response. This response was also compared to that obtained using the currently available commercial preparation (4 micrograms/mL) given either s.c. or intravenously (i.v.). The maximal f. VIII response after s.c. C-DDAVP was reached one hour after the injection (means:3.5 times the resting values) with an average decline of 15% at two hours. The response to s.c. C-DDAVP in patients with hemophilia was slightly better than that obtained with the diluted brand, but the difference did not reach any statistical significance even when the schedules were compared in the same patients. In type I (platelet normal subtype) vWD, a higher response in terms of factor VIII:C increase in comparison with hemophiliacs was obtained. Both Ristocetin co-factor activity (RiCof) and bleeding time responded to this vasopressin analogue, when administered subcutaneously.
Deamino Arginine Vasopressin↗
Evaluation of factor VII antigen in factor VII congenital deficiencies with a new ELISA assay.
An evaluation of a new Enzyme Linked Immunosorbent Assay (ELISA) factor VII:Ag assay in factor VII congenital deficiencies was carried out. This assay was compared to factor VII:C assay and the Inhibitor Neutralization Assay (INA) for factor VII:Ag, both in normals and F VII-deficient patients. The correlations between ELISA F VII:Ag and VII:C, as well as the one between INA F VII:Ag and VII:C, were good (r = .86 and .81, respectively). The correlation between the two immunologic methods of assay in normals was fairly good (r = 0.73, P less than 0.001); whereas in mild F VII deficiencies (heterozygotes for F VII deficiencies), the two methods correlated very well (r = .96). In the severe deficiencies, ELISA F VII:Ag assay allowed the evaluation of F VII protein levels below 1 u/dl. The sensitivities of the INA and ELISA assays were evaluated by creating artificially prepared plasmas containing serial amounts of purified F VII: INA was unable to pick up F VII protein levels lower than 75 ng/ml, whereas the ELISA assay could detect up to 5 ng of F VII.
Adult↗
Danazol therapy in refractory chronic immune thrombocytopenic purpura.
We report our experience with danazol in the treatment of patients with refractory immune thrombocytopenic purpura (ITP). The effects of this drug were investigated in 10 patients, 6 males and 4 females, aged from 40 to 85 years, (median 58 years), with a platelet count below 50 X 10(9)/l. The patients had previously been treated with steroids; one of them had also been unsuccessfully splenectomized. Danazol was administered at a dosage of 600 mg/day for 3 months. Before and after treatment, detection of antiplatelet antibodies was performed. Seven patients were treated for 3 months. One of them showed a transient increase of platelet count, in the others, no significant rise was noted. Six patients experienced side effects during treatment. We think that danazol does not appear to be an alternative therapeutical approach in refractory ITP.
Adult↗
Inhibitor to factor VIII in a non-haemophilic patient: evaluation of the response to DDAVP and the in vitro kinetics of factor VIII. A case report.
We report a case of inhibitor to factor VIII in a non-haemophilic patient. Immunosuppressive therapy with azathioprine was started, but without any advantage. Evaluation of the kinetics of exogenous factor VIII in vitro showed a rapid but incomplete neutralization of factor VIII. Following s.c. 1-deamino-8-D-arginine vasopressin (DDAVP) administration, a large and prolonged increase in factor VIII:C and von Willebrand factor antigen occurred together with complete inhibitor saturation. Therefore DDAVP may represent an important tool in the management of the bleeding episodes in these patients and evaluation of its suitability in the management of these patients should be carried out.
Blood Coagulation Disorders↗
Familial dysfunctional protein C.
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Factor X↗
Homozygous protein C deficiency with moderately severe clinical symptoms.
We report a large family with two members homozygotes for protein C deficiency, with activity levels of 5% and 9%. Thirteen additional members were heterozygotes, with protein C activity ranging from 36-66% and equally low levels of protein C antigen. The homozygotes presented with recurrent deep-vein thromboses and pulmonary emboli, but have reached the ages of 26 and 37 years. Hence, protein C levels of 5% appear sufficient to avoid life-threatening clinical symptoms in the neonatal period.
Adolescent↗
Hepatitis B vaccination of 113 hemophiliacs: lower antibody response in anti-LAV/HTLV-III-positive patients.
One-hundred thirteen adults and children with hemophilia or other congenital bleeding disorders were vaccinated against the hepatitis B virus. Each patient was given three subcutaneous injections of the vaccine at monthly intervals and then a fourth booster dose 14 months after the first. The vaccine was highly immunogenic, since 111 of 113 patients (98%) produced anti-HBs (10 mIU/ml or more). After the first three vaccine doses and after the booster dose, ten anti-LAV/HTLV-III-positive hemophiliacs produced anti-HBs but had a lower average titer than anti-LAV/HTLV-III-negative hemophiliacs. Of the 23 patients treated with concentrates in the 15 month postvaccination period only, none acquired HBV infection. Of the 50 patients treated with concentrates also in the 6 month prevaccination period, one developed hepatitis B. In summary, the vaccine was highly immunogenic in both children and adults with hemophilia; anti-LAV/HTLV-III-positive patients responded to the vaccine, but the average anti-HBs response was lower; no case of hepatitis B occurred in patients treated with concentrates only in the postvaccination period.
Adolescent↗
Pipobroman therapy of essential thrombocythemia.
We report our results with pipobroman (PB) therapy in patients with essential thrombocythemia (ET). 21 consecutive untreated patients were treated with PB from 1975 to 1984. PB was given at a dose of 1 mg/kg/d until platelet count dropped below 600 X 10(9)/l. In 18 patients (86%) a hematological remission was obtained. Median duration of induction phase was 49 d. In all cases a maintenance regimen was required at a dose ranging from 0.2 mg/kg/d to 0.5 mg/kg/d, according to platelet number. Follow-up of responder patients ranged from 6 to 108 months (median 17 months). Treatment was well tolerated and we observed only a very moderate and transient hematological toxicity. No patient had relapsed or developed secondary neoplasms at the time of writing. Median survival time of all patients was 24 months (range 10-115).
Adult↗
Comparison between subcutaneous and intravenous DDAVP in mild and moderate hemophilia A.
Sixteen patients with mild and moderate hemophilia were given Desmopressin (DDAVP) subcutaneously in the absence of any actual bleeding. The response to the drug - in terms of factor VIII coagulant activity rise - became apparent 15 min after the injection, reaching the maximal response after one hour (means 3.2 times the baseline levels; SD 1.21). This response was not different from that elicited using the intravenous route in 18 hemophiliacs of comparable severity after the same time interval. No local or general side-effects were recorded after the subcutaneous administration of DDAVP. We therefore conclude that the subcutaneous route adds further evidence to the reliability of this alternative treatment in mild factor VIII deficiencies, thus making home treatment with this vasopressin analogue possible.
Deamino Arginine Vasopressin↗
Evaluation of the clotting defect in a factor XII-deficient kindred.
A family with factor XII severe congenital deficiency is described. Factor XII activity and factor XII antigen were both undetectable in the propositus plasma; levels of FXII:C and FXII:Ag were intermediate in heterozygotes. Plasma prekallikrein activity was low in the propositus, whereas normal levels of antigen could be found, suggesting a defect of kallikrein activation due to factor XII deficiency.
Blood Coagulation↗
Treatment of recurrent promyelocytic leukemia with a combination regimen utilizing amsacrine, cytosine arabinoside and 6-thioguanine (AAT).
Nine patients with recurrent acute promyelocytic leukemia have been treated between July, 1984 and November, 1987 with a combination therapeutic regimen consisting of amsacrine, cytosine arabinoside and thioguanine (AAT). Complete remission was achieved in 5/9 patients, one person died in aplasia of hepatic failure, and the remaining 3 died from heart failure with resistant disease. The 5 patients who achieved CR were successively treated with allogeneic (n = 2) or autologous bone marrow transplantation (n = 3). As of December, 1988 there were only 2 patients still alive and in CR, both underwent autologous bone marrow transplantation. The duration of the second complete remission in these two patients is 48+ and 29+ months, respectively. Moreover the overall survival duration for these two patients is 88+ and 41+ months, respectively. These data confirm that the AAT regimen is useful in treating recurrent acute promyelocytic leukemia, a disease otherwise characterized by a catastrophic clinical course during the recurrent phase.
Adult↗