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295 results on '"Magrini, U"'

14. Abnormal expression patterns of WT1-as, MEG3 and ANRIL long non-coding RNAs in CD34+ cells from patients with primary myelofibrosis and their clinical correlations

Author

Pennucci, V, Zini, R, Norfo, R, Guglielmelli, P, Bianchi, E, Salati, S, Sacchi, G, Prudente, Z, Tenedini, E, Ruberti, S, Paoli, C, Fanelli, T, Mannarelli, C, Tagliafico, E, Ferrari, S, Vannucchi, Am, Manfredini, R, on behalf of Associazione Italiana per la Ricerca sul Cancro Gruppo Italiano Malattie Mieloproliferative Investigators Collaborators Vannucchi AM, Balliu, M, Bartalucci, N, Bogani, C, Bosi, A, Fjerza, R, Martinelli, S, Pancrazzi, A, Pieri, L, Bisognin, Andrea, Bortoluzzi, Stefania, Coppe, Alessandro, Saccoman, Claudia, Masciulli, A, Giovani, B, Azzan, C, Badalucco, S, Balduini, A, Bonetti, E, Campanelli, R, Catarsi, P, Isgrò, Ma, Lupo, Ml, Magrini, U, Massa, M, Poletto, V, Rosti, V, Villani, L, Cazzola, M, Ambaglio, I, Bernasconi, P, Casetti, Ic, Catricalà, S, Elena, C, Fugazza, E, Gallì, A, Malcovati, L, Milanesi, C, Pascutto, C, Pietra, D, Ripamonti, F, Rossi, M, Rumi, E, Dejana, E, Breviario, F, Corada, M, Erba, Bg, Rambaldi, A, Barbui, T, Ferrari, Ml, Finazzi, G, Finazzi, Mc, Gritti, G, Belotti, C, Boroni, C, Salmoiraghi, S, Amaru, A, Golay, J, Cilloni, D, Campia, V, Carturan, S, Guerrasio, A, Montanari, M, and Zini, R.

Subjects
medicine.medical_specialty, Cancer Research, Antigens, CD34, Biology, Transcriptome, Internal medicine, Hematology, Oncology, Gene expression, medicine, Humans, Myelofibrosis, MEG3, Regulation of gene expression, Reverse Transcriptase Polymerase Chain Reaction, RNA, Prognosis, medicine.disease, Gene Expression Regulation, Neoplastic, Primary Myelofibrosis, Cancer cell, Immunology, Cancer research, RNA, Long Noncoding
Abstract

Long non-coding RNAs (lncRNAs), an emerging group of gene expression regulators in normal and cancer cells, are defined as endogenous RNAs consisting of more than 200 nucleotides and lacking an ope...

Published
2015

15. Alteration of liver enzymes is a feature of the MYH9-related disease syndrome

Author

Pecci A, Biino G, Fierro T, Bozzi V, Mezzasoma A, Patrizia Noris, Ramenghi U, Loffredo G, Fabris F, Momi S, Magrini U, Pirastu M, Savoia A, Balduini C, Gresele P, Myh, Italian Registry For -Releated Diseases, Pecci, A., Biino, G., Fierro, T., Bozzi, V., Mezzasoma, A., Noris, P., Ramenghi, U., Loffredo, G., Fabris, F., Momi, S., Magrini, U., Pirastu, M., Savoia, Anna, Balduini, C., Gresele, P., and Italian Registry for MYH9 releated, Diseases

Subjects
MYH9, Thrombocytopenia, Male, Pathology, MYH9 Mutations, Anatomy and Physiology, Biopsy, lcsh:Medicine, Disease, Biochemistry, Liver Function Tests, may-heggly, Odds Ratio, lcsh:Science, IIA, Child, inherited thrombocytopenias, Aged, 80 and over, Multidisciplinary, medicine.diagnostic_test, Molecular Motor Proteins, Liver Diseases, Fatty liver, fechtner-syndrome, myosin heavy chain, diagnostic algorithm, epstein-syndrome, cell motility, Macrothrombocytopenia, Syndrome, Hematology, Middle Aged, Immunohistochemistry, Malattia MYH9 correlata, Enzymes, Liver, Autosomal Dominant, Child, Preschool, Medicine, Female, Research Article, Adult, Platelets, medicine.medical_specialty, Adolescent, Gastroenterology and Hepatology, Neurological System, Nephropathy, Enzyme Regulation, Young Adult, medicine, Humans, Clinical significance, Abnormalities, Multiple, Biology, Aged, Demography, Clinical Genetics, Myosin Heavy Chains, business.industry, lcsh:R, Case-control study, Infant, Odds ratio, medicine.disease, Case-Control Studies, Mutation, lcsh:Q, business, Liver function tests, Follow-Up Studies, Neuroscience
Abstract

BACKGROUND: MYH9-related disease (MYH9-RD) is a rare autosomal dominant genetic syndrome characterized by congenital thrombocytopenia associated with the risk of developing progressive nephropathy, sensorineural deafness, and presenile cataract. During the collection of a large case-series of patients with MYH9-RD we noticed several cases with unexplained elevation of liver enzymes. Our aim was to evaluate if the alteration of liver tests is a feature of the MYH9-RD and to define its clinical significance. METHODS AND FINDINGS: Data concerning liver tests, prospectively recorded in the Italian Registry for MYH9-RD, were collected and compared with those of three control populations: patients with autoimmune thrombocytopenia, patients with inherited thrombocytopenias other than MYH9-RD, and the participants to a large epidemiologic survey in an Italian geographic isolate. Thirty-eight of 75 evaluable MYH9-RD patients (50.7%) showed an elevation of ALT and/or AST, and 17 of 63 (27.0%) an increase of GGT. The increases ranged from 1.9 ± 0.7 to 2.7 ± 1.6 fold the upper normal limit. The prevalence of liver test alterations was significantly higher in MYH9-RD patients than in each of the control populations, with odds ratios ranging from 8.2 (95% CIs 2.2-44.8) to 24.7 (14.8-40.8). Clinical follow-up and more detailed liver studies of a subset of patients, including ultrasound liver scan, liver elastography and liver biopsy in one case, did not show any significant structural damage or evolution towards liver insufficiency. CONCLUSIONS: Elevation of liver enzymes is a frequent and previously unrecognized feature of the MYH9-RD syndrome; however, this defect does not appear to have poor prognostic value.

Published
2012

16. Mutations in the nonmuscle myosin heavy chain IIA gene (MYH9) result in the diverse phenotypes of the May-Hegglin anomaly, Fechtner and Sebastian syndromes

Author

Heath, K.E., Seri, M., Savino, M., Cusano, R., Gangarossa, S., Caridi, G., Bordo, D., Lo Nigro, C., Ghiggeri, G.M., Del Vecchi, M., D'Apolito, M., Iolascon, A., Zelante, L.L., Balduini, C.L., Noris, P., Magrini, U., Belletti, S., Babcock, M., Aliprandis, E., Glucksman, M.J., Bizzaro, N., Desnick, R.J., Ravazzolo, R., Savoia, A., and Martignetti, J.A.

Subjects
Genetic research -- Analysis, Human genetics -- Research, Blood diseases -- Genetic aspects, Genetic disorders -- Research, Biological sciences
Published
2000

17. Clinical effect of driver mutations of JAK2, CALR, or MPL in primary myelofibrosis

Author

Rumi, E, Pietra, D, Pascutto, C, Guglielmelli, P, Martínez Trillos, A, Casetti, I, Colomer, D, Pieri, L, Pratcorona, M, Rotunno, G, Sant'Antonio, E, Bellini, M, Cavalloni, C, Mannarelli, C, Milanesi, C, Boveri, E, Ferretti, V, Astori, C, Rosti, V, Cervantes, F, Barosi, G, Vannucchi, Am, Cazzola, M, Associazione Italiana per la Ricerca sul Cancro Gruppo Italiano Malattie Mieloproliferative Investigators Collaborators Vannucchi AM, Balliu, M, Bartalucci, N, Biamonte, F, Bisognin, A, Bogani, C, Bortoluzzi, S, Bosi, A, Coppe, A, Fanelli, T, Fjerza, R, Loiacono, I, Marchioli, R, Martinelli, S, Masciulli, A, Pancrazzi, A, Paoli, C, Saccoman, C, Spolverini, A, Susini, Mc, Tozzi, L, Azzan, C, Badalucco, S, Balduini, A, Bonetti, E, Campanelli, R, Catarsi, P, Isgrò, Am, Lupo, Ml, Magrini, U, Massa, M, Poletto, V, Villani, L, Ambaglio, I, Bernasconi, P, Casetti, Ic, Catricalà, S, Elena, C, Fugazza, E, Gall, A, Malcovati, L, Ripamonti, F, Rossi, M, Dejana, E, Breviario, F, Corada, M, Erba, Bg, Rambaldi, A, Amaru, A, Barbui, T, Belotti, C, Boroni, C, Ferrari, Ml, Finazzi, G, Finazzi, Mc, Golay, J, Gritti, G, Salmoiraghi, S, Cilloni, Daniela, Campia, V, Carturan, S, Guerrasio, Angelo, Manfredini, R, Bianchi, E, Salati, S, Tagliafico, E, Tenedini, E, and Zini, R.

Subjects
Oncology, Male, Clinical Trials and Observations, Leukocytosis, DNA Mutational Analysis, Kaplan-Meier Estimate, Biochemistry, Risk Factors, hemic and lymphatic diseases, Aged, 80 and over, Leukemia, biology, Incidence (epidemiology), food and beverages, Anemia, Hematology, Middle Aged, Prognosis, Cell Transformation, Neoplastic, Female, medicine.symptom, Receptors, Thrombopoietin, Adult, medicine.medical_specialty, Adolescent, Immunology, Lower risk, Risk Assessment, Young Adult, Internal medicine, medicine, Humans, Myelofibrosis, Aged, Proportional Hazards Models, business.industry, Proportional hazards model, Cell Biology, Janus Kinase 2, medicine.disease, Thrombocytopenia, Primary Myelofibrosis, Mutation, biology.protein, business, Calreticulin
Abstract

We studied the impact of driver mutations of JAK2, CALR, (calreticulin gene) or MPL on clinical course, leukemic transformation, and survival of patients with primary myelofibrosis (PMF). Of the 617 subjects studied, 399 (64.7%) carried JAK2 (V617F), 140 (22.7%) had a CALR exon 9 indel, 25 (4.0%) carried an MPL (W515) mutation, and 53 (8.6%) had nonmutated JAK2, CALR, and MPL (so-called triple-negative PMF). Patients with CALR mutation had a lower risk of developing anemia, thrombocytopenia, and marked leukocytosis compared with other subtypes. They also had a lower risk of thrombosis compared with patients carrying JAK2 (V617F). At the opposite, triple-negative patients had higher incidence of leukemic transformation compared with either CALR-mutant or JAK2-mutant patients. Median overall survival was 17.7 years in CALR-mutant, 9.2 years in JAK2-mutant, 9.1 years in MPL-mutant, and 3.2 years in triple-negative patients. In multivariate analysis corrected for age, CALR-mutant patients had better overall survival than either JAK2-mutant or triple-negative patients. The impact of genetic lesions on survival was independent of current prognostic scoring systems. These observations indicate that driver mutations define distinct disease entities within PMF. Accounting for them is not only relevant to clinical decision-making, but should also be considered in designing clinical trials.

Published
2014

18. Effects of R216Q mutation of GATA-1 on erythropoisis and platelet production

Author

Balduini, Cl, Pecci, A, Loffredo, G, Izzo, P, Noris, P, Grosso, M, Bergamaschi, G, Rosti, V, Magrini, U, Ceresa, If, Conti, V, Poggi, V, Savoia, Anna, Balduini, Cl, Pecci, A, Loffredo, G, Izzo, P, Noris, P, Grosso, M, Bergamaschi, G, Rosti, V, Magrini, U, Ceresa, If, Conti, V, Poggi, V, and Savoia, Anna

Published
2004

20. CD56/neural cell adhesion molecule expression in primary extranodal Ki-1/CD30+ lymphoma. Report of a pediatric case with simultaneous cutaneous and bone localizations

Author

Paulli, M, Boveri, E, Rosso, R, Aricò, M, Kindl, S, Viglio, A, Berti, E, Leithäuser, F, Locatelli, F, Gianelli, U, Beluffi, G, Feller, A, Borroni, G, Magrini, U, BERTI, EMILIO, Magrini, U., Paulli, M, Boveri, E, Rosso, R, Aricò, M, Kindl, S, Viglio, A, Berti, E, Leithäuser, F, Locatelli, F, Gianelli, U, Beluffi, G, Feller, A, Borroni, G, Magrini, U, BERTI, EMILIO, and Magrini, U.

Abstract

We describe the clinicopathologic features of an unusual case of CD30+/CD50+ T-cell lymphoma in a child who presented with simultaneous primary extranodal cutaneous and bone localizations. The expression of CD56 (neural cell adhesion molecule, or NCAM) is rare in non-Hodgkin's lymphomas other than in a group of haematopoietic/lymphoid neoplasms of natural killer and natural killer-like T-cells, which usually involve extranodal sites and often pursue an aggressive clinical behavior. Coexpression of CD30 and CD56 in T-cell lymphomas is exceedingly rare, and its biological significance is unknown. Our patient responded well to an intensive chemotherapy regimen, and she is now in complete remission 4 years after discontinuation of chemotherapy. Expression of NCAM could be regarded as responsible, in part, for the extranodal localization of lymphoma cells; expression of CD56 also might contribute to the definition of a subset of CD30+ lymphomas with distinctive clinicopathologic features

Published
1997

21. Mutations in MYH9 result in the May-Hegglin anomaly, and Fechtner and Sebastian syndromes. The May-Heggllin/Fechtner Syndrome Consortium

Author

SERI M, CUSANO R, GANGAROSSA S, CARIDI G, BORDO D, LO NIGRO C, GHIGGERI GM, RAVAZZOLO R, SAVINO M, DEL VECCHIO M, D'APOLITO M, ZELANTE LL, SAVOIA A, BALDUINI CL, NORIS P, MAGRINI U, BELLETTI S, HEATH KE, BABCOCK M, GLUCKSMAN MJ, ALIPRANDIS E, BIZZARO N, DESNICK RJ, MARTIGNETTI J.A., IOLASCON, ACHILLE, Seri, M, Cusano, R, Gangarossa, S, Caridi, G, Bordo, D, LO NIGRO, C, Ghiggeri, Gm, Ravazzolo, R, Savino, M, DEL VECCHIO, M, D'Apolito, M, Iolascon, Achille, Zelante, Ll, Savoia, A, Balduini, Cl, Noris, P, Magrini, U, Belletti, S, Heath, Ke, Babcock, M, Glucksman, Mj, Aliprandis, E, Bizzaro, N, Desnick, Rj, and Martignetti, J. A.

Subjects
Models, Molecular, Cytoplasm, Genotype, Neutrophils, Protein Conformation, Chromosomes, Human, Pair 22, Hearing Loss, Sensorineural, Molecular Sequence Data, Mutation, Missense, Myosins, Crystallography, X-Ray, Cataract, Leukocytes, Animals, Humans, Amino Acid Sequence, Alleles, Nephritis, Myosin Heavy Chains, Sequence Homology, Amino Acid, Molecular Motor Proteins, Muscle, Smooth, Syndrome, Thrombocytopenia, Protein Structure, Tertiary, Phenotype, Mutation, Blood Platelet Disorders, Chickens
Abstract

The autosomal dominant, giant-platelet disorders, May-Hegglin anomaly (MHA; MIM 155100), Fechtner syndrome (FTNS; MIM 153640) and Sebastian syndrome (SBS), share the triad of thrombocytopenia, large platelets and characteristic leukocyte inclusions ('Döhle-like' bodies). MHA and SBS can be differentiated by subtle ultrastructural leukocyte inclusion features, whereas FTNS is distinguished by the additional Alport-like clinical features of sensorineural deafness, cataracts and nephritis. The similarities between these platelet disorders and our recent refinement of the MHA (ref. 6) and FTNS (ref. 7) disease loci to an overlapping region of 480 kb on chromosome 22 suggested that all three disorders are allelic. Among the identified candidate genes is the gene encoding nonmuscle myosin heavy chain 9 (MYH9; refs 8-10), which is expressed in platelets and upregulated during granulocyte differentiation. We identified six MYH9 mutations (one nonsense and five missense) in seven unrelated probands from MHA, SBS and FTNS families. On the basis of molecular modelling, the two mutations affecting the myosin head were predicted to impose electrostatic and conformational changes, whereas the truncating mutation deleted the unique carboxy-terminal tailpiece. The remaining missense mutations, all affecting highly conserved coiled-coil domain positions, imparted destabilizing electrostatic and polar changes. Thus, our results suggest that mutations in MYH9 result in three megakaryocyte/platelet/leukocyte syndromes and are important in the pathogenesis of sensorineural deafness, cataracts and nephritis.

Published
2000

22. High frequency of endothelial colony forming cells marks a non-active myeloproliferative neoplasm with high risk of splanchnic vein thrombosis

Author

Rosti, V, Bonetti, E, Bergamaschi, G, Campanelli, R, Guglielmelli, P, Maestri, M, Magrini, U, Massa, M, Tinelli, C, Viarengo, G, Villani, L, Primignani, M, Vannucchi, Am, Frassoni, F, Barosi, G, Agimm, Investigators, INCLUDING VANNUCCHI AM, Antonioli, E, Bartalucci, N, Biamonte, F, Bogani, C, Bosi, A, Fjerza, R, Malevolti, E, Pancrazzi, A, Pieri, L, Spolverini, A, Susini, Mc, Tozzi, L, Bortoluzzi, Stefania, Bisognin, A, Coppe, A, Marchioli, R, Azzan, C, Badalucco, S, Balduini, A, Carolei, A, Currao, M, Isgrã’, Ma, Lupo, Ml, Magni, V, Cazzola, M, Bernasconi, P, Boggi, S, Elena, C, Gallãœ, A, Malcovati, L, Pascutto, C, Passamonti, F, Pietra, D, Rumi, E, Dejana, E, Corada, M, Giannotta, M, Rambaldi, A, Ferrari, Ml, Finazzi, G, Finazzi, Mc, Magri, M, Quaresmini, G, Montalvo, Ml, Ricci, C, Salmoiraghi, S, Spinelli, O, Amaru, A, Golay, J, Cilloni, D, Arruga, F, Bracco, E, Carturan, S, Gaidano, V, Guerrasio, A, Pradotto, M, Manfredini, R, Bianchi, E, Montanari, M, Salati, S, Tagliafico, E, Tenedini, E, and Zini, R.

Subjects
Male, Pathology, myeloproliferative neoplasm, Gastroenterology, Cohort Studies, Hematologic Cancers and Related Disorders, splanchnic vein thrombosis, Hemoglobins, Polycythemia vera, Molecular Cell Biology, Odds Ratio, Splanchnic Circulation, Polycythemia Vera, Aged, 80 and over, Venous Thrombosis, Thrombocytosis, Likelihood Functions, Multidisciplinary, Hematology, Middle Aged, Venous thrombosis, Oncology, Medicine, Female, Cellular Types, Research Article, Adult, medicine.medical_specialty, Clinical Pathology, Science, Sensitivity and Specificity, Myeloproliferative Disorders, Diagnostic Medicine, Internal medicine, medicine, Humans, Myelofibrosis, Biology, Myeloproliferative neoplasm, Aged, Essential thrombocythemia, business.industry, Endothelial Cells, Cancers and Neoplasms, Odds ratio, medicine.disease, Thrombocytopenia, Cross-Sectional Studies, Splanchnic vein thrombosis, business, Biomarkers, General Pathology
Abstract

Increased mobilization of circulating endothelial progenitor cells may represent a new biological hallmark of myeloproliferative neoplasms. We measured circulating endothelial colony forming cells (ECFCs) in 106 patients with primary myelofibrosis, fibrotic stage, 49 with prefibrotic myelofibrosis, 59 with essential thrombocythemia or polycythemia vera, and 43 normal controls. Levels of ECFC frequency for patient's characteristics were estimated by using logistic regression in univariate and multivariate setting. The sensitivity, specificity, likelihood ratios, and positive predictive value of increased ECFC frequency were calculated for the significantly associated characteristics. Increased frequency of ECFCs resulted independently associated with history of splanchnic vein thrombosis (adjusted odds ratio = 6.61, 95% CI = 2.54-17.16), and a summary measure of non-active disease, i.e. hemoglobin of 13.8 g/dL or lower, white blood cells count of 7.8×10(9)/L or lower, and platelet count of 400×10(9)/L or lower (adjusted odds ratio = 4.43, 95% CI = 1.45-13.49) Thirteen patients with splanchnic vein thrombosis non associated with myeloproliferative neoplasms were recruited as controls. We excluded a causal role of splanchnic vein thrombosis in ECFCs increase, since no control had elevated ECFCs. We concluded that increased frequency of ECFCs represents the biological hallmark of a non-active myeloproliferative neoplasm with high risk of splanchnic vein thrombosis. The recognition of this disease category copes with the phenotypic mimicry of myeloproliferative neoplasms. Due to inherent performance limitations of ECFCs assay, there is an urgent need to arrive to an acceptable standardization of ECFC assessment.

Published
2010

24. [Molecular biology, basic research and diagnosis of Hirschsprung's disease]

Author

Martucciello, Giuseppe, Luinetti, O., Romano, P., and Magrini, U.

Subjects
diagnosis/embryology/genetics, Diagnosis, Differential, Mutation, Proto-Oncogene Proteins c-ret, Humans, genetics, Hirschsprung Disease, Differential, Gastrointestinal Motility, genetics, Hirschsprung Disease, diagnosis/embryology/genetics, Humans, Mutation, Proto-Oncogene Proteins c-ret, Gastrointestinal Motility
Published
2007

25. Il Gruppo Italiano di Emolinfopatologia (G.I.E.): una esigenza per le nuove e complesse problematiche tecnico-organizzative della moderna diagnostica emolinfopatologica

Author

Paulli, M, Artusi, T, Baroni, Cd, Carbone, A, Coggi, G, DI LOLLO, S, Facchetti, F, Falini, B, Franco, V, Gambacorta, M, LA ROCCA VM, Leoncini, L, Magrini, U, Maiorana, Antonino, Menestrina, F, Novero, D, Palestro, G, Pescarmona, E, Cantucci, M, STRACCA PANSA, V, Truini, M, and Pileri, S.

Subjects
Emolinfopatologia, diagnostica
Published
2006

27. Mutations in MYH9 in May-Hegglin anomaly, and Fechtner and Sebastian syndromes

Author

Seri, M, Cusano, R, Gangarossa, S, Caridi, G, Bordo, D, LO NIGRO, C, Ghiggeri, Gm, Ravazzolo, Roberto, Savino, M, DEL VECCHIO, M, D'Apolito, M, Iolascon, A, Zelante, Ll, Savoia, A, Balduini, Cl, Noris, P, Magrini, U, Belletti, S, Heath, Ke, Babcock, M, Glucksman, Mj, Aliprandis, E, Bizzaro, N, Desnick, Rj, and Martignetti, J. A.

Published
2000

28. ESSENTIAL THROMBOCYTHAEMIA:PROGNOSTIC FACTORS IN THE ITALIANSERIES OF TWO THOUSAND PATIENTS

Author

Gugliotta, L., Lazzarino, M., Marchioli, R., Ambrosetti, A., Baravelli, S., Bazzan, M., Cacciola, E., Calori, R., CIOCCA VASINO, A., DE VIVO, A., Fiacchini, M., Finazzi, G., Gargantini, L., Grossi, A., Iannaccaro, P. G., Leva, T., Magrini, U., Marfisi, M. R., Martinelli, V., Mazzucconi, M. G., Morelli, A., Novarino, A., Palmieri, F., Pogliani, E., Radaelli, F., Randi, L., REGE CAMBRIN, G., Ronco, F., Ruggeri, M., Rupoli, S., Russo, D., Sacchi, Stefano, Valdrè, L., Vianelli, N., Rodeghiero, F., Barbui, T., and Tura, S.

Subjects
nd
Published
1999

30. Essential Thrombocythaemia: prognostic factors in the italian series of two thousand patients

Author

Gugliotta, L, Lazzarino, M, Marchioli R, R., Ambrosetti, A, Baravelli, S, Bazzan, M, Cacciola, Emma, Calori, R, Ciocca Vasino, A, De Vivo, A, Fiacchini, M, Finazzi, G, Gargantini, L, Grossi, A, Iannacaro, Pg, Leva, T, Magrini, U, Marfisi, Mr, Martinelli, V, Mazzucconi, Mg, Morelli, A, Novarino, A, Palmieri, F, Pogliani, E, Radaelli, F, Randi, L, Rege Cambrin, G, Ronco, F, Ruggeri, M, Rupoli, S, Russo, D, Sacchi, S, Valdrè, L, Vianelli, N, Rodeghiero, F, Barbui, T, and for the GIMMC, Tura S.

Published
1999

31. Subcutaneous 'lipoma-like' B-cell lymphoma associated with HCV infection: a new presentation of primary extranodal marginal zone B-cell lymphoma of MALT

Author

Paulli, M, Arcaini, L, Lucioni, M, Boveri, E, Capello, D, Passamonti, F, Merli, M, Rattotti, S, Rossi, D, Riboni, R, Berti, E, Magrini, U, Bruno, R, Gaidano, G, Lazzarino, M, BERTI, EMILIO, Lazzarino, M., Paulli, M, Arcaini, L, Lucioni, M, Boveri, E, Capello, D, Passamonti, F, Merli, M, Rattotti, S, Rossi, D, Riboni, R, Berti, E, Magrini, U, Bruno, R, Gaidano, G, Lazzarino, M, BERTI, EMILIO, and Lazzarino, M.

Abstract

Background: Hepatitis C virus (HCV) infection has been linked to lymphoproliferative disorders. Marginal zone B-cell lymphoma (MZL) represents one of the most frequent lymphoma subtypes associated with HCV infection. We describe an unusual subset of HCV-associated MZL characterized by subcutaneous presentation. Materials and methods: A series of 12 HCV-positive patients presenting with subcutaneous nodules that revealed lymphoma infiltration at biopsy. Molecular analysis of immunoglobulin heavy chain (IGH) gene rearrangement and FISH investigations for t(11;18)(q21;q21) and t(14;18)(q32;q21) were carried out in nine patients. Results: The 12 patients (median age 69.5 years), all with positive HCV serology, presented with single or multiple subcutaneous nodules resembling lipomas. Histologically the lesions showed lymphoid infiltrates, consistent with extranodal MZL of mucosa-associated lymphoid tissue (MALT). Functional IGH gene rearrangements were identified in nine tested patients, with somatic mutations in 82%, indicating a histogenesis from germinal center-experienced B cells. The t(11;18) was found in two of nine cases. Staging did not show any other lymphoma localization. In two patients, a response was achieved with antiviral treatment. Extracutaneous spread to MALT sites occurred in a case. Conclusions: Our observations expand the spectrum of HCV-associated lymphomas to include a subset of extranodal MZL characterized by a novel primary 'lipoma-like' subcutaneous presentation and indolent clinical course.

Published
2010

32. Primary cutaneous large B-cell lymphoma of the leg: Histogenetic analysis of a controversial clinicopathologic entity

Author

Paulli, M, Viglio, A, Vivenza, D, Capello, D, Rossi, D, Riboni, R, Lucioni, M, Incardona, P, Boveri, E, Bellosta, M, Orlandi, E, Borroni, G, Lazzarino, M, Berti, E, Alessi, E, Magrini, U, Gaidano, G, BERTI, EMILIO, Gaidano, G., Paulli, M, Viglio, A, Vivenza, D, Capello, D, Rossi, D, Riboni, R, Lucioni, M, Incardona, P, Boveri, E, Bellosta, M, Orlandi, E, Borroni, G, Lazzarino, M, Berti, E, Alessi, E, Magrini, U, Gaidano, G, BERTI, EMILIO, and Gaidano, G.

Abstract

This study analyzes the pathologic and molecular features of 5 cases of primary cutaneous large B-cell lymphoma of the leg (PCLBCL-leg), recently included in the European Organization for Research and Treatment of Cancer (EORTC) classification of primary cutaneous lymphoma. PCLBCL-leg accounts for 5% to 10% of all primary cutaneous B-cell lymphoma (PCBCL), usually affects elderly patients and carries a worse prognosis than other forms of PCBCL. It has been proposed that the malignant cells of PCLBCL-leg originate from germinal center (GC)-related cells, but their effective normal counterpart is unclear, and the rationale behind the inclusion of this lymphoma as a separate entity is based on its prognosis rather than on its proved histogenesis. All of our cases of PCLBCL-leg morphologically resembled diffuse large B-cell lymphoma (DLBCL), but to better define their histogenesis, we also analyzed various phenotypic and genotypic markers, including mutations of the Ig and of BCL,6 genes, as well as expression of the bcl-6, MUM1, and CD138/syndecan-1 proteins. Immunohistochemically, all of our cases stained for the 1,26/CD20cy and CD79a antigens and expressed the bcl-2, bcl-6, and MUM-1 proteins but were negative for both the CD10/CALLA and CD138 antigens. With respect to molecular analysis, the lymphoma population of all PCLBCL-leg carried hypermutation of Ig genes, and all but I case also harbored mutations of the BCL-6 gene. Our results indicate that PCLBCL-leg are similar both under the morphofunctional and molecular profiles to most DLBCL of other sites. Thus, caution seems justified before definitely considering PCLBCL of the leg as a distinct entity. Copyright 2002, Elsevier Science (USA). All rights reserved.

Published
2002

36. Cutaneous CD30+ lymphoproliferative disorders: expression of bcl-2 and proteins of the tumor necrosis factor receptor superfamily

Author

Paulli, M, Berti, E, Boveri, E, Kindl, S, Bonoldi, E, Gambini, C, Rosso, R, Borroni, G, Straccapansa, V, Magrini, U, Decoteau, J, Krammer, P, Möller, P, Kadin, M, DeCoteau, J, Kadin, M., BERTI, EMILIO, Paulli, M, Berti, E, Boveri, E, Kindl, S, Bonoldi, E, Gambini, C, Rosso, R, Borroni, G, Straccapansa, V, Magrini, U, Decoteau, J, Krammer, P, Möller, P, Kadin, M, DeCoteau, J, Kadin, M., and BERTI, EMILIO

Abstract

The spectrum of CD30+ cutaneous lymphoproliferative disorders is characterized by the histology of a high-grade lymphoma but frequent clinical regression of skin lesions in lymphomatoid papulosis (LyP) and occasional regression in CD30+ large cell lymphomas (LCLs). A recent study shows that apoptosis may be a significant mechanism of regression of LyP (Arch Dermatol 133:828-833, 1997). Therefore, we studied expression of proteins that induce apoptosis, including CD27, CD40, CD95, and nerve growth factor receptor (NGF-R), as well as anti-apoptotic protein bcl-2 in skin lesions from 25 patients within the spectrum of CD30+ cutaneous lymphoma. Our results show consistent expression of CD95 (APO-1/Fas), but rare or absent expression of CD27, CD40, and NGF-R on tumor cells from both regressing LyP lesions and nonregressing CD30+ lymphomas. Bcl-2 was expressed at low levels in LyP and at high levels in pleomorphic CD30+ lymphomas. These results indicate that, in addition to CD30, CD95 expression is preferentially expressed at high levels in all cutaneous CD30+ lymphomas and suggest that CD95 may play a role in the regression of CD30+ skin lesions. Expression of bcl-2 appears to protect tumor cells from apoptosis in CD30+ lymphoproliferative disorders.

Published
1998

38. Sinus histiocytosis with massive lymphoadenopathy (Rosai-Dorfman disease). Clinico-pathological analysis of a paediatric case

Author

Paulli, M, Locatelli, Franco, Kindl, S, Boveri, E, Facchetti, F, Porta, F, Rosso, R, Nespoli, L, Magrini, U, Locatelli, F (ORCID:0000-0002-7976-3654), Paulli, M, Locatelli, Franco, Kindl, S, Boveri, E, Facchetti, F, Porta, F, Rosso, R, Nespoli, L, Magrini, U, and Locatelli, F (ORCID:0000-0002-7976-3654)

Abstract

Histochemical and immunohistochemical studies performed in only a few cases of sinus histiocytosis with massive lymphoadenopathy (SHML) indicated that SHML cells belong to the macrophage - histiocyte system, though their exact origin is still uncertain. We analyzed the morphological, antigenic and enzymatic characteristics of the histiocyte-like cells in one paediatric case of SHML (also named Rosai-Dorfman disease). The SHML cells expressed the S-100 protein, lectins concanavalin A, peanut agglutinin and monocyte-macrophage related antigens CD 11c, CD 14, CD 33, CD 68 and LN 5. Reactivity with other anti-macrophage antibodies (MAC 387, lysozyme, alpha-1 anti-chymotrypsin) was variable. The CD1a antigen was present only in scattered cells, whereas HLA-DR and the HLA-DR associated invariant chain were absent. Cytochemistry demonstrated an intense activity of acid phosphatase and non specific esterase of SHML cells. A large amount of medium sized mononuclear cells were located in the sinuses and intersinusoidal tissue. Our findings suggest that SHML cells have intermediate features between phagocytes and Langerhans cells/interdigitating reticulum cells. The heterogeneity of marker expression on SHML cells might be related to the local content of factors (e.g., cytokines), capable of modulating the phenotype of monocyted and derived cells.

Published
1992

39. Treatment outcome and prognostic factors for primary mediastinal (thymic) B-cell lymphoma: a multicenter study of 106 patients.

Author

Lazzarino, M, primary, Orlandi, E, additional, Paulli, M, additional, Sträter, J, additional, Klersy, C, additional, Gianelli, U, additional, Gargantini, L, additional, Rousset, M T, additional, Gambacorta, M, additional, Marra, E, additional, Lavabre-Bertrand, T, additional, Magrini, U, additional, Manegold, C, additional, Bernasconi, C, additional, and Möller, P, additional

Published
1997
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40. Primary Mediastinal B-Cell Lymphoma: Update of its Clinicopathologic Features

Author

Paulli, M., primary, Lazzarino, M., additional, Gianelli, U., additional, Sträuter, E., additional, Orlandi, E., additional, Klersy, C., additional, Viglio, A., additional, Rosso, R., additional, Gambacorta, M., additional, Rousset, T., additional, Morra, E., additional, Lavabre-Bertrand, T., additional, Bernasconi, C., additional, Manegold, C., additional, Magrini, U., additional, and Möller, P., additional

Published
1997
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45. Myelofibrosis with myeloid metaplasia: clinical and haematological parameters predicting survival in a series of 133 patients

Author

Visani, G., primary, Finelli, C., additional, Castelli, U., additional, Petti, M. C., additional, Ricci, P., additional, Vianelli, N., additional, Gianni, L., additional, Zuffa, E., additional, Aloe Spiriti, M. A., additional, Latagliata, R., additional, Pileri, S., additional, Magrini, U., additional, Gugliotta, L., additional, Morra, E., additional, Bernasconi, C., additional, Mandelli, F., additional, and Tura, S., additional

Published
1990
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49. On Building Hygrometry: Influence of Internal and Exterior Structures.

Author

Isetti, C., Magrini, U., and Nannei, E.

Abstract

Maximum limit of vapor concentration of the inside air that does not create mould growth and condensation on the surface of exterior walls has been analyzed in non-steady state conditions.The calculations have been carried out for three different exterior walls and various air changes.Results show that both thermal storage capacity of the walls and of the inter nal structures affect the level of the permissible vapour concentration inside the dwelling. [ABSTRACT FROM PUBLISHER]

Published
1984
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