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Biomedical subjects

A Lattuada

Publications and source records attributed to A Lattuada.

At least 19 recordsLinked to original sources
PubMed1992

A new variant of von Willebrand disease (type II I) with a normal degree of proteolytic cleavage of von Willebrand factor.

A variant of type II von Willebrand disease (vWd) is described in a young woman and her mother with severe lifelong bleeding histories. On electrophoresis with low-resolution agarose gels the plasma of the proband lacked large and intermediate-size multimers of von Willebrand factor (vWF) but the platelet multimeric structure was normal. On high-resolution gels, smaller multimers could be resolved into a broader central band and four satellite bands, which were much fainter than in normal plasma. In the proband plasma, the relative concentrations of proteolytic fragments of the vWF subunit were within the normal laboratory range. Since this variant of vWd appears to differ from those reported hitherto, the designation of type II I is proposed.

Adolescent↗
PubMed1990

Platelet von Willebrand factor assay: results using two methods for platelet lysis.

Two different methods (using Triton X-100 and glycerol) for lysing platelets to measure platelet vWF concentrations were compared directly. The platelet concentration of von Willebrand factor antigen (vWF:Ag) was similar for both methods, whereas ristocetin cofactor activity (Ricof) was higher with Triton than with glycerol. After storing platelet lysates for two months at -80 degrees C vWF:Ag and Ricof concentrations decreased with both methods of lysis. Larger than normal (supranormal) vWF multimeric forms could be visualized in platelet lysates obtained using both methods, with no change of the multimeric pattern during storage. Triton can be recommended as the agent of choice to lyse platelets for measurement of their vWF concentration, but the samples must be assayed within two weeks to avoid decay of Ricof activity.

Blood Platelets↗
PubMed1990

Total body irradiation and high-dose melphalan with bone marrow transplantation at Istituto Nazionale Tumori, Milan, Italy.

The technique of total body irradiation (TBI) developed at Istituto Nazionale Tumori, Milan, Italy, is described. This technique consists of i) administration of 12.5 Gy and 14.85 Gy TBI for autologous and allogeneic bone marrow transplantation respectively; ii) in all cases in vivo dosimetry of absorbed TBI dose; and iii) radiation doses to lungs higher than previously described. As of June 1988, seventeen patients with Hodgkin's disease and four with lymphoblastic lymphoma received TBI and 120-180 mg/m2 melphalan. Respiratory function was prospectively evaluated demonstrating moderate and transient reduction of pulmonary function.

Adolescent↗
PubMed1989

Heterogeneous phenotypes of platelet and plasma von Willebrand factor in obligatory heterozygotes for severe von Willebrand disease.

To characterize the heterogeneity of severe (type III) von Willebrand disease (vWD), plasma and platelet von Willebrand factor antigen (vWF:Ag) and ristocetin cofactor activity (Ricof) were measured in 28 obligatory heterozygotes (ie, parents or children of probands from 15 different kindreds with severe vWD). On the average, heterozygotes had low levels of vWF in both platelets and plasma. There was, however, considerable heterogeneity, with four distinct patterns. Eleven heterozygotes had concordant reduction of vWF:Ag and Ricof in both plasma and platelets; five had low levels of vWF:Ag and Ricof in plasma contrasting with normal levels in platelets; eight had a peculiar pattern, the reverse of the above (ie, low levels in platelets and normal levels in plasma); and in one, both vWF measurements were normal in plasma and platelets. These patterns were genetically determined: they were consistent in four couples of consanguineous heterozygotes and in two couples carrying the same gene deletion. Only the remaining three heterozygotes had no clearly identifiable pattern. Other findings of this study were that although most of the heterozygotes had normal bleeding times, the 7 of 28 who had prolonged bleeding times had concordantly low levels of vWF measurements in both plasma and platelets. In conclusion, this large series of obligatory heterozygotes provides evidence for phenotypic and genotypic heterogeneity of severe vWD.

Bleeding Time↗
PubMed1989

Enhanced proteolysis of plasma von Willebrand factor in thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome.

To examine whether enhanced in vivo proteolysis of von Willebrand factor (vWF) would account for the reported loss of larger multimers in acute thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS), we studied eight patients with acute TTP/HUS whose blood samples were collected into an anticoagulant containing a cocktail of protease inhibitors to impede in vitro proteolysis. In all, enhanced proteolytic degradation of vWF was expressed as a relative decrease in the intact 225-Kd subunit of vWF and a relative increase in the 176-Kd fragment. However, instead of the loss of larger forms of normal multimers reported by other investigators, the plasma of all but one of our patients (whether they had TTP or HUS) contained a set of larger than normal (supranormal) multimers. Hence, although proteolytic fragmentation of vWF was enhanced during acute TTP/HUS, this phenomenon was not associated with the loss of larger multimers. In the five patients who survived the acute disease and underwent plasma exchange (three with HUS and two with chronic relapsing TTP), subunits and fragments returned to normal values, and supranormal multimers were no longer detected in plasma. In conclusion, even though vWF proteolysis is enhanced in acute TTP/HUS, it does not lead to loss of larger multimers.

Adolescent↗
PubMed1989

Supranormal von Willebrand factor multimers in scleroderma.

Platelet adhesion-aggregation reactions play an early and pivotal role in the pathogenesis of systemic sclerosis in scleroderma, but the mechanisms are incompletely understood. We determined whether or not plasma from 11 consecutive patients with scleroderma contained a subset of larger than normal ("supranormal") multimers of von Willebrand factor (vWF) that are potent inducers of platelet aggregation and adhesion. Supranormal multimers were found in all patients on at least one of two different occasions 9 to 12 months apart, whatever the duration and severity of the disease, but in none of the normal controls. Administration of low-dose aspirin (40 mg) to five of the 11 patients for ten days to inhibit the platelet release reaction slightly reduced the amounts of supranormal multimers suggesting that they might originate in part from platelets. Supranormal multimers may contribute to the pathogenesis of systemic sclerosis by inducing platelet aggregation and enhancing adhesion to subendothelium under the conditions of elevated shear stress occurring in the partially occluded vessels of the arterial microcirculation of scleroderma.

Adult↗
PubMed1989

Subcutaneous desmopressin (DDAVP) shortens the bleeding time in uremia.

The intravenous infusion of 1-deamino-8-D-arginine vasopressin (DDAVP) is used as a nontransfusional form of treatment in patients with congenital and acquired bleeding disorders, including patients with uremia associated with prolonged bleeding times. Since uremic patients experience minor bleeding episodes that might be self-managed at home (particularly epistaxis, gingival bleeding, and menorrhagia), we carried out a double-blind, placebo-controlled crossover study in nine uremics to evaluate whether the prolonged bleeding times could be shortened by subcutaneous injections of DDAVP. One hour after administration, the bleeding time was significantly shortened (P less than .01) and became normal in seven of nine patients. After 4 hr, the bleeding time was still shorter than baseline (P less than .01), but in only three patients was it still normal. There was no significant bleeding time change after placebo. When the same patients were treated with the same dose of DDAVP infused intravenously, the bleeding times were not significantly different from those measured after subcutaneous administration. Hence, subcutaneous DDAVP is an alternative method for short-term shortening of the bleeding time in uremia, at least as effective as intravenous DDAVP but with the possibility of self-administration by the patients at home.

Adult↗
PubMed1989

Type II H von Willebrand disease: new structural abnormality of plasma and platelet von Willebrand factor in a patient with prolonged bleeding time and borderline levels of ristocetin cofactor activity.

In this study a new variant of type II von Willebrand disease is identified by multimeric analyses of increasing resolving power. Prior to multimeric analysis, the patient was misdiagnosed as carrying an undefined abnormality in platelet function because of his normal von Willebrand factor antigen (vWF:Ag) and low borderline ristocetin cofactor (Ricof) levels. Absence of the largest multimers from the patient's plasma and platelets was shown in a low-resolution system, but all the multimers were present in his relatives. An abnormality in the complex multimeric structure was demonstrated in both plasma and platelets with high-resolution agarose gels. The plasma of the proband and of several family members shows a broader central band with a minor, faster moving satellite band differing from the typical "triplet pattern" observed with normal plasma. Platelets show a "doublet" that runs with a mobility different from the "doublet" in normals. Therefore the proband may be either a homozygote or double heterozygote for this new abnormality. Treatment with desmopressin (DDAVP) on several occasions corrected the prolonged bleeding time of the patient only transiently. Factor VIII increased significantly, but vWF:Ag and Ricof responded poorly. We conclude that this vWF abnormality is different from those observed in the other variants (II A-G) previously described. Therefore the proposed designation for this new variant is type II H.

Adult↗
PubMed1989

Advanced neuroblastoma: results of two treatment programs including sequential hemibody irradiation.

Results of two consecutive treatment programs for advanced neuroblastoma, including sequential hemibody irradiation, are analyzed and compared. The first treatment program (I-TP) included one single-fraction (7 Gy) irradiation to the upper and lower halves of the body as consolidation of remission achieved by previous chemotherapy with CDDP and VP16. A fractionated technique (2 Gy daily for 4 consecutive days to each hemibody) was used in the second treatment program (II-TP) for children in remission following a combination of CDDP + VP16 and ADM + VCR + CTX. In both treatment programs, chemotherapy was continued according to the same pre-radiation regimen following the two sessions of hemibody irradiation. Overall response rate to pre-radiation chemotherapy was 84% and 60% for I-TP and II-TP, respectively. Thirty-month overall progression-free survival was 0 for I-TP and 20% for II-TP. No treatment-related fatalities occurred. In the subsets of patients who reached complete or good partial remission during the pre-radiation chemotherapeutic phase, 30-month progression-free survival in I-TP and II-TP was 0 and 33%, respectively. The role of fractionated hemibody irradiation in prolonging the progression-free survival can be inferred.

Adolescent↗
PubMed1989

[Treatment technic and clinical dosimetry in whole-body irradiation].

At the Istituto Nazionale Tumori, Milan, total-body irradiation (TBI) is delivered by a 15 MV linear accelerator, with two lateral opposed beams. Maximum build-up at the skin is achieved by lateral slabs of perspex 3 cm thick. Attenuation filters or bolus are used for dose compensation, or reduction, to the head and lungs. The dose delivered to clinically relevant anatomic regions is determined by "in vivo" dosimetry. For this purpose, calibrated diodes are employed, which are positioned at the entrance and at the exit of the beams. "In vivo" dosimetry data show our TBI technique to allow an homogeneous irradiation of all body areas, with maximum deviation of the mean dose value from reference point dose of -11% in the posterior abdomen, at the spinal cord shielded by arms.

Humans↗
PubMed1989

[The prognostic significance of anaplastic variants in patients with testicular seminoma subjected to exclusive radiotherapy].

Three-hundred and sixty-six patients affected with seminoma testis (234 stage I, 85 stage II low, 47 advanced diseases) were treated between January 1968 and December 1985. Overall incidence of anaplastic lesions (43 patients) was 12%, progressively increasing with stage of the disease (9% stage I, 13% stage II low, 21% advanced disease). Only 343 patients submitted to exclusive curative/prophylactic radiotherapy were studied: 35 of them were anaplastic (22 stage I, 11 stage II low, 2 advanced disease). Radiation therapy was always performed regardless of the histologic subtype. Recurrences occurred in 14% of anaplastic tumors (5/35) and in 7.5% of classical seminomas (23/308). There was no statistically-significant difference between global and stage-by-stage failures. After literature review and complete analysis of the patterns of recurrence, the authors stress the lack of evidence suggesting a different radioresponsiveness of anaplastic versus classical seminomas; nonetheless, a more accurate staging is recommended for anaplastic variants, since they tend to appear in the advanced stages of the disease.

Adult↗
PubMed1988

Combined modality treatment for stage I-II non-Hodgkin's lymphomas: CVP versus BACOP chemotherapy.

This paper reports the 5-year results of a prospective randomized study beginning in 1976 on 177 evaluable patients with pathologic Stage I-IE and II-IIE non-Hodgkin's lymphomas with diffuse histology according to the Rappaport classification. Treatment consisted of either CVP or BACOP chemotherapy (3 cycles) followed by regional radiotherapy (40 to 50 Gy) and further cycles of either combination. In both arms, complete remission at the end of combined treatment was high (CVP 93%, BACOP 98%) regardless of age, stage or bulky disease. At 5 years, the comparative freedom from first progression was 62% for CVP vs 78% for BACOP (p = 0.02), respectively. Clinically relevant differences favoring BACOP chemotherapy were essentially documented in patients with large cell lymphomas (International Working Formulation), those with Stage II having more than three involved anatomical sites, bulky disease and age over 60 years. Recurrence within radiation fields was documented in only 5% of complete responders. Combined treatment was, in general, well tolerated particularly when BACOP was used. In only 2 patients given CVP post radiation cutaneous fibrosis was documented. Second solid tumors were detected in 4 patients. One patient started on CVP died because of brain stem necrosis after 45 Gy. We conclude that in Stage I-II patients with nodal and extranodal diffuse non-Hodgkin's lymphomas, particularly large cell lymphomas, combined modality approach with primary Adriamycin and bleomycin containing regimen, such as BACOP, followed by adjuvant radiotherapy offers high chances of cure with minimal toxicity.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1979

Childhood non-Hodgkin Malignant lymphomas: a clinicopathologic retrospective study.

Between 1968 and 1975, 44 evaluable children under 16 years of age with the histologic diagnosis of non-Hodgkin malignant lymphoma (ML) were treated at the Istituto Nazionale Tumori of Milan. Histologic diagnoses were reclassified as follows: 13 lymphoblastic (others) ML, 15 convoluted cell type lymphoblastic ML, 9 Burkitt type ML, and 7 immunoblastic ML. Only 36% of the patients had stage I and II disease. At diagnosis 25% showed malignant cells in the bone marrow smears. Bone marrow infiltration was particularly frequent in the convoluted cell type lymphoblastic ML and in the lymphoblastic (others) ML subgroups. Burkitt type ML frequently was associated with abdominal lesions and subsequently a high incidence of central nervous system involvement. Patients with stage I and II ML were encountered mostly in the immunoblastic ML subgroup. After 1973 more intensive chemotherapy plus radiotherapy seems to have slightly improved the survival of the patients, except in the Burkitt type ML Subgroup.

Adolescent↗
PubMed1977

Lymphangiography in patients with ovarian epithelial cancer: an evaluation of 289 consecutive cases.

From January, 1973, to June, 1976, 226 patients with palpable ovarian masses were evaluated preoperatively by lymphography. Histology showed 166 cases of malignant epithelial tumors, 26 benign tumors, and 34 malignant special tumors (not included in this report). Furthermore, the group of patients included 99 recurrences of ovarian epithelial cancer and 24 patients who underwent restaging diagnostic procedures without clinical evidence of disease. Lymphography was negative in all patients with benign tumors. In the 289 cases of epithelial cancer, lymphangiography gave evidence of nodal metastases in 88 (30%). When the histological subtype was considered, the highest incidence of metastases was in undifferentiated carcinoma (50%) and the lowest, in mesonephroid carcinoma (14%). According to the stage before lymphography, nodal metastases were found in 8% of Stage I, 0% of Stage II, 29% of Stage III, and 53% of Stage IV cases. The incidence of metastases was 46% in patients studied for recurrent disease and 17% in patients studied for restaging. Fifty-four percent of patients had metastases only in the pelvic nodes and 18% only in the para-aortic chains; in 28% both chains were involved simultaneously. Bilateral involvement was found in 63% of the positive cases. Retroperitoneal node biopsies were performed in 68 patients (36%). The radiologic/histologic correlation was 100% in the lymphangiographically positive cases; 81% in the negative cases, with nine false-negative reports; and 87% in all cases.

Carcinoma↗