Biomedical subjects
Publications and source records attributed to M Ruggeri.
A celiac disease update review and a case report are presented, especially concerning a Clinical Pathology Laboratory approach. Implemented basic research, case finding strategy and information technologies are the key tools for a better understanding of the multi-etiological features of this disease. By these tools it will be achieved appropriateness in diagnosing and monitoring of the related clinical pictures.
Long-lasting remission after high-dose intravenous immunoglobulins in a case of relapsing thrombotic thrombocytopenic purpura (TTP) is reported. The patient had two early relapses after intensive plasma-exchange therapy. Serum platelet aggregating factor was absent. High-dose intravenous immunoglobulins (i.v. Ig) (0.4 g/Kg/day for 5 days) were infused. Platelet count promptly rose and 7 days after the completion of immunoglobulins complete response was obtained. The patient is still in complete remission, without further Ig infusion, 19 months later. This case adds to the anecdotal reports on the effectiveness of Ig, even when not associated with other treatments, in TTP. High-dose i.v. Ig could be successfully used in relapsing or unresponsive patients with TTP.
BACKGROUND AND METHODS: We report the results of extensive "in vitro" fibrinolytic studies in 18 homozygous and 14 obligatory heterozygous subjects belonging to 13 unrelated families with factor XII deficiency. All homozygotes had unmeasurable factor XII activity (XII:C) and antigen (XII:Ag). None had bleeding symptoms, whereas a myocardial infarction occurred in one of them at age 51. In heterozygotes XII:C and XII:Ag were 55.9 +/- 14.1% and 52.1 +/- 16.4% (corresponding figures in 40 normals 100.6 +/- 18.3% and 101.5 +/- 29.7%). Total intrinsic fibrinolytic activity was assayed on fibrin plates in the dextran sulfate euglobulin fraction of plasma from resting subjects, to which flufenamate was added to inhibit blood plasminogen activator inhibitors. RESULTS: Fibrinolytic activity was reduced in all homozygotes (40 +/- 12 BAU/ml) in comparison to heterozygotes (103 +/- 12 BAU/ml) and normals (98 +/- 20 BAU/ml). The addition of purified activated beta-XII led to a complete restoration of fibrinolysis in homozygotes. The addition of anti-urokinase antibodies completely suppressed the reduced intrinsic fibrinolytic activity in homozygotes (4 +/- 7 BAU/ml), whereas a reduction to about 50% was evident in heterozygotes and normals. CONCLUSIONS: Our data confirm that reduced "in vitro" intrinsic fibrinolytic activity is a common finding in homozygous factor XII deficiency and that two independent mechanisms, one factor XII-dependent and urokinase-independent and the other factor XII-independent and urokinase-dependent, are responsible for the generation of intrinsic fibrinolysis in human plasma.
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Authors describe two new cases of mucocutaneus lymphonode syndrome (MCLS). These are the tenth and eleventh in Italy since 1977, confirming that this syndrome, known also as Kawasaki disease, is world-wide spread. More attention is therefore required to diagnosis particularly for possible coronary artery involvement. Echocardiography may be useful in doing that. A survey of recent literature indicates that salicylate therapy may be useful not only for symptoms relief but also in preventing life threatening of coronary artery vasculitis.
BACKGROUND AND METHODS: Polycythemia vera (PV) and essential thrombocythemia (ET) in young patients are rarely reported. Their natural histories seem to differ from those of older patients and the best treatment is still uncertain. In this follow-up study we have evaluated a cohort of 64 consecutive patients younger than 40 to determine the incidence of thrombohemorrhagic events and the long-term outcome. RESULTS: Twenty-eight patients (20 M; 8 F) had PV, and 36 ET (21 F, 15 M). Mean follow-up was 8.2 years (range 4 months-16.7 years) in PV and 6.5 years (range 5 months-15 years) in ET. Thrombohemorrhagic symptoms were present at diagnosis in 10/28 patients (35%) with PV and in 12/36 patients (33%) with ET; during follow-up in 15/28 PV patients (53%) and in 13/36 ET patients (36%). Thrombotic events were the most frequent symptoms, both at diagnosis (52% in PV, 65% in ET) and during follow-up (43% in PV, 52% in ET). A total of 19/28 PV patients (67%) and 17/36 ET patients (47%) had thrombotic complications. Hemorrhagic complications at diagnosis were 4% and during follow-up 13% in PV, and 15% and 13% in ET. A total of 5/28 (18%) PV and 6/36 (17%) ET patients had hemorrhagic events. No laboratory parameter, including platelet count, was predictive of these events. Five PV patients had major thrombotic complications (18%). Four PV patients died (14%), 2 because of ANLL (7%), 2 because of thrombotic events (7%). Four ET patients experienced major complications, in three cases thrombotic (8.3%), in one hemorrhagic. No leukemic transformation occurred in ET and no ET patient died. CONCLUSIONS: In our experience, severe thrombohemorrhagic complications are present in young patients with PV and ET, which excludes young age as a favorable prognostic factor. Treatment also seems advisable for young patients and myelosuppressive treatment might be required. Prospective studies are urgently needed to assess the best treatment for this particular subset of patients.
BACKGROUND: Bleeding and thrombosis are frequent complications in patients with chronic myeloproliferative disorders (cMPD). Antiplatelet therapy is extensively used by many physicians for primary prophylaxis of thrombotic events, even though there have been no prospective trials that demonstrate clinical benefit. The use of aspirin has been associated with a heavy incidence of serious hemorrhages, particularly of gastrointestinal origin. This evidence is mainly based on data from patients treated with dosages far higher than those presently recommended. Moreover, patients with bleeding symptoms or prolonged bleeding time (BT) had not usually been excluded from treatment. METHODS: In this study 58 patients with cMPD and thrombocytosis were treated with aspirin (325-500 mg/day, 31 patients) or with ticlopidine (500 mg/day 27 patients). Only patients with negative bleeding histories and normal BT were considered. Ticlopidine, a drug not extensively investigated in cMPD, was reserved only for patients with histories of gastritis, gastric discomfort, peptic ulcer and/or intolerance to aspirin. All other patients were given aspirin combined with antacids in a buffered preparation. RESULTS AND CONCLUSIONS: Average follow-up was 2 years. Aspirin was associated with a high incidence of gastrointestinal hemorrhages (5/31). Ticlopidine was tolerated better and no bleeding complications were recorded. Both drugs were similarly effective in relieving erythromelalgia and painful paresthesia in almost all cases with these symptoms within 24-48 hours.
BACKGROUND: High dose intravenous immunoglobulin (h.d. Ig) is increasingly used in several hematological diseases. There are few data on the safety and no review of side effects after this treatment is available. We review here the literature on reported adverse reactions after h.d. Ig and report our own experience in the management of 83 patients with idiopathic thrombocytopenic purpura (ITP). PATIENTS AND METHODS: Computer assisted (Medline) and manual searches of the cumulative Index Medicus were undertaken to retrieve articles reporting side effects after infusion of h.d. Ig, published between 1981 and August 1993. Our own experience is based on clinical records of 83 patients, 56 females, 27 male, with a median age of 61 years (range 8-84), treated with h.d. Ig for acute or chronic ITP between 1985 and June 1993. RESULTS AND DISCUSSION: Major adverse reactions included aseptic meningitis (14 cases), hemolytic anemia (8 cases) and renal dysfunction (12 cases). The most frequently reported mild side effect was headache. We have had 2 cases of severe skin reactions, 1 case of acute renal failure, 3 cases of severe headache and 1 case of chills and fever. For other 3 cases, an anaphylactic/oid reaction, caused by a too rapid infusion rate, occurred. Thus, 10/83 (12%) patients, 7 female and 3 male, developed side effects, but they were unpredictable and severe in only 3 cases (4%). Side effects developed only in 21/480 (4%) infusions.
BACKGROUND: The usefulness of measuring antiplatelet antibodies by ELISA or cytofluorimetric techniques for the diagnosis of idiopathic thrombocytopenic purpura is still uncertain. METHODS: We evaluated the clinical significance of two widely applicable antiplatelet antibody tests for a series of 265 patients evaluated consecutively in our Department for the diagnosis or follow-up of thrombocytopenia. Flow cytofluorimetry was used to measure platelet-associated immunoglobulins (PAIg) and the enzyme-linked immunosorbent assay (ELISA) was used to detect antiplatelet antibodies in patients sera (S-PBIg). The significance of antiplatelet antibody testing was addressed by studying the factors that influence test positivity, the diagnostic value of a positive test and the prognostic implication of a positive test. RESULTS: The platelet count was found to be strongly associated with positive PAIg or S-PBIg (P < 0.001), while neither splenectomy nor corticosteroid treatment affected PAIg or S-PBIg positivity. Both PAIg and S-PBIg were limited diagnostic value for the differential diagnosis of idiopathic thrombocytopenic purpura (ITP) from secondary thrombocytopenia for patients with platelet counts between 25,000 and 100,000 platelets/microliters, but the percentages of misclassified patients based on only the PAIg or S-PBIg test were 32 and 54%. We found no relationship between PAIg and/or S-PBIg at time of diagnosis and the patient's clinical response to corticosteroid therapy. CONCLUSIONS: We conclude that antiplatelet antibodies are strongly correlated with platelet counts, discriminate poorly between ITP and secondary thrombocytopenia and have negligible prognostic value. Therefore, we do not recommend performing antiplatelet antibody tests as a routine laboratory test in the diagnostic workup of thrombocytopenia.
BACKGROUND: The reported incidence of thromboembolic episodes in people with nephrotic syndrome (NS) ranges from 6% to 44% and it has been ascribed to the presence of a hypercoagulable state, as suggested by abnormalities of several hemostatic parameters in these patients. However, the results of the studies are often contradictory and fragmented and are rarely based on prospective studies. To assess the incidence of venous thrombosis and the pattern of abnormalities of the hemostatic parameters in NS, we planned a prospective study of a group of patients with NS. PATIENTS AND METHODS: Thirty-six consecutive patients with NS were enrolled during a 9-month period. Every 4-6 months, clinical history was collected and physical examination was carried out. Blood samples for laboratory investigation were taken at entry into the study and 24 months later. A critical review of the literature was also carried out (Medline database and Current Contents). RESULTS: During the follow-up (mean 45.2 months), no thrombotic symptoms were recorded, PTT, PT, TT and mean AT-III levels were within the normal range, whereas fibrinogen, plasminogen, protein C and S, heparin cofactor II levels were significantly higher than normal both at entry into the study and 24 months later. No patient was positive for antiphospholipid antibodies. Slightly decreased levels of AT-III and heparin cofactor II were found in only two cases. CONCLUSIONS: Our study confirms neither the high incidence of thrombotic complications in NS nor the presence of abnormalities of hemostatic parameters commonly associated with venous thromboembolism.
High-dose intravenous immunoglobulin (IVIG) is considered a safe and efficacious treatment for patients with a variety of hematological and non hematological diseases, including patients with idiopathic thrombocytopenic purpura (ITP) not responsive or with contraindications to corticosteroids and/or splenectomy. Side-effects are usually minor, but severe reactions have been rarely reported. We describe the case of a patient with ITP, without pre-existing renal disease, who developed a severe transitory acute renal failure following administration of IVIG, promptly reversed after hemodialysis.
Gestational thrombocytopenia (GT) is commonly observed in pregnancies with otherwise limited obstetric and hematologic complications. However, few data are available on the natural history of the disease, and on the recurrence of thrombocytopenia in subsequent pregnancies. From June 1987 to December 1993, 37 consecutive patients with GT were enrolled in a prospective study, with a total of 41 pregnancies observed. Vaginal delivery was carried out in 33/41 (80%); two patients were transfused fused with packed red cells for obstetric hemorrhage (post-partum uterine atony). Neonatal bleeding did not occur. In all newborns platelet count was performed within 24 hours after delivery: 2 newborns had mild (80 and 75 x 10(9)/L) and 1 severe thrombocytopenia (12 x 10(9)/L) at birth; all of them recovered to a normal platelet count within 10 days without treatment. Twenty-eight out of 3% patients were followed for 12 months after delivery; in 23 a normalization of platelet count occurred within 1-5 months from delivery; in 5 mild thrombocytopenia (100-120 x 10(9)/L) persisted during follow-up. Four patients had a second pregnancy and recurrence of thrombocytopenia was observed in all of them. GT is rarely associated with bleeding episodes during pregnancy and partum, and recovers spontaneously within few months after delivery but thrombocytopenia can recur in subsequent pregnancies. Severe thrombocytopenia is not observed in newborns so that a conservative management is warranted.