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Start Over Author "Pontiggia S" Topic 03 medical and health sciences
2 results on '"Pontiggia S"'

1. The features of acquired thrombotic thrombocytopenic purpura occurring at advanced age

Author

Agosti, P, Mancini, I, Artoni, A, Ferrari, B, Pontiggia, S, Trisolini, Sm, Facchini, L, Peyvandi, F, Capria, S, Codeluppi, K, Rinaldi, E, Pastore, D, Campus, S, Podda, Ra, Caria, C, Caddori, A, Nicolosi, D, Giuffrida, G, Agostini, V, Roncarati, U, Mannarella, C, Fragasso, A, Podda, Gm, Birocchi, S, Cerbone, Am, Tufano, A, Loffredo, G, Menna, G, Pizzuti, M, Ronchi, M, De Fanti, A, Amarri, S, Defina, M, Bocchia, M, Ceru, S, Gattillo, S, Agosti, P., Mancini I., Artoni A., Ferrari B., Pontiggia S., Trisolini S. M., Facchini L., Peyvandi F., and Tufano, A.

Subjects
medicine.medical_specialty, Thrombotic microangiopathy, ADAMTS13 Protein, Hemorrhage, Disease, 030204 cardiovascular system & hematology, Kidney, Adamts13 activity, 03 medical and health sciences, 0302 clinical medicine, Elderly, Older patients, Internal medicine, medicine, Humans, Registries, Acute thrombotic thrombocytopenic purpura management, ADAMTS13, Rare disease, Aged, Acquired Thrombotic Thrombocytopenic Purpura, Purpura, Thrombotic Thrombocytopenic, business.industry, Hematology, Microangiopathic hemolytic anemia, medicine.disease, Cross-Sectional Studies, 030220 oncology & carcinogenesis, Female, business
Abstract

Introduction: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare life-threatening thrombotic microangiopathy (TMA) affecting more frequently women of 30–50 years of age. There is scarce information on the clinical features of aTTP occurring in the elderly. Our goal was to evaluate the impact of an elderly-onset disease on the expression, severity and management of aTTP. Materials and methods: We performed a cross-sectional study of patients enrolled in the Milan TTP Registry (www.ttpdatabase.org) after a first acute episode of aTTP from January 2002 to March 2018. The aTTP diagnosis was suspected on the basis of the presence of thrombocytopenia and microangiopathic hemolytic anemia with no alternative causes, and was confirmed centrally by a severe plasma deficiency of ADAMTS13 activity (

Published
2020

2. The HLA Variant rs6903608 Is Associated with Disease Onset and Relapse of Immune-Mediated Thrombotic Thrombocytopenic Purpura in Caucasians

Author

Clara Mannarella, Erminia Rinaldi, Katia Codeluppi, Anna Maria Cerbone, Sergio Amarri, Daniela Nicolosi, Michele Pizzuti, Luca Facchini, Alessandro De Fanti, Michela Ronchi, Emanuela Pappalardo, Barbara Ferrari, Monica Bocchia, Gaetano Giuffrida, Vanessa Agostini, Cinzia Caria, Silvia Maria Trisolini, Salvatore Gattillo, Elisa Giacomini, Gian Marco Podda, Aldo Caddori, Saveria Capria, Antonella Tufano, Silvia Cerù, Marzia Defina, Alberto Fragasso, Roberta Gualtierotti, Andrea Artoni, Simona Campus, Flora Peyvandi, Silvia Pontiggia, Umberto Roncarati, Ilaria Mancini, Giuseppe Menna, Domenico Pastore, Frits R. Rosendaal, Simone Birocchi, Mancini, I., Giacomini, E., Pontiggia, S., Artoni, A., Ferrari, B., Pappalardo, E., Gualtierotti, R., Trisolini, S. M., Capria, S., Facchini, L., Codeluppi, K., Rinaldi, E., Pastore, D., Campus, S., Caria, C., Caddori, A., Nicolosi, D., Giuffrida, G., Agostini, V., Roncarati, U., Mannarella, C., Fragasso, A., Podda, G. M., Birocchi, S., Cerbone, A. M., Tufano, A., Menna, G., Pizzuti, M., Ronchi, M., De Fanti, A., Amarri, S., Defina, M., Bocchia, M., Ceru, S., Gattillo, S., Rosendaal, F. R., and Peyvandi, F.

Subjects
medicine.medical_specialty, Thrombotic microangiopathy, ADAMTS13, HLA, autoimmune disease, genotyping, relapse, risk factor, thrombotic thrombocytopenic purpura, Thrombotic thrombocytopenic purpura, lcsh:Medicine, Human leukocyte antigen, 030204 cardiovascular system & hematology, Gastroenterology, Article, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, hemic and lymphatic diseases, medicine, Risk factor, business.industry, lcsh:R, Absolute risk reduction, General Medicine, Odds ratio, medicine.disease, business, 030215 immunology, Cohort study
Abstract

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening thrombotic microangiopathy caused by severe ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin motifs 13) deficiency, recurring in 30&ndash, 50% of patients. The common human leukocyte antigen (HLA) variant rs6903608 was found to be associated with prevalent iTTP, but whether this variant is associated with disease relapse is unknown. To estimate the impact of rs6903608 on iTTP onset and relapse, we performed a case-control and cohort study in 161 Italian patients with a first iTTP episode between 2002 and 2018, and in 456 Italian controls. Variation in rs6903608 was strongly associated with iTTP onset (homozygotes odds ratio (OR) 4.68 (95% confidence interval (CI) 2.67 to 8.23), heterozygotes OR 1.64 (95%CI 0.95 to 2.83)), which occurred over three years earlier for each extra risk allele (&beta, &minus, 3.34, 95%CI &minus, 6.69 to 0.02). Of 153 survivors (median follow-up 4.9 years (95%CI 3.7 to 6.1)), 44 (29%) relapsed. The risk allele homozygotes had a 46% (95%CI 36 to 57%) absolute risk of relapse by year 6, which was significantly higher than both heterozygotes (22% (95%CI 16 to 29%)) and reference allele homozygotes (30% (95%CI 23 to 39%)). In conclusion, HLA variant rs6903608 is a risk factor for both iTTP onset and relapse. This newly identified biomarker may help with recognizing patients at high risk of relapse, who would benefit from close monitoring or intensified immunosuppressive therapy.

Published
2020

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