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Tiscia G, Sartori MT, Giuffrida G, et al. Focus on Key Issues in Immune Thrombotic Thrombocytopenic Purpura: Italian Experience of Six Centers. J Clin Med. 2021;10(23)doi: 10.3390/jcm10235702.
Tiscia, G., Sartori, M. T., Giuffrida, G., Ostuni, A., Cascavilla, N., Nicolosi, D., Battista, C., Santeramo, T. M., Melillo, L., Giordano, G., Cappucci, F., Fischetti, L., Chinni, E., Tarantini, G., Cerbo, A., Bertomoro, A., Fabris, F., & Grandone, E. (2021). Focus on Key Issues in Immune Thrombotic Thrombocytopenic Purpura: Italian Experience of Six Centers. Journal of clinical medicine, 10(23), . https://doi.org/10.3390/jcm10235702
Tiscia, Giovanni, et al. "Focus on Key Issues in Immune Thrombotic Thrombocytopenic Purpura: Italian Experience of Six Centers." Journal of clinical medicine vol. 10,23 (2021). doi: https://doi.org/10.3390/jcm10235702
Tiscia G, Sartori MT, Giuffrida G, Ostuni A, Cascavilla N, Nicolosi D, Battista C, Santeramo TM, Melillo L, Giordano G, Cappucci F, Fischetti L, Chinni E, Tarantini G, Cerbo A, Bertomoro A, Fabris F, Grandone E. Focus on Key Issues in Immune Thrombotic Thrombocytopenic Purpura: Italian Experience of Six Centers. J Clin Med. 2021 Dec 04;10(23). doi: 10.3390/jcm10235702. PMID: 34884404.
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J Clin Med. 2021 Dec 04;10(23). doi: 10.3390/jcm10235702.

Focus on Key Issues in Immune Thrombotic Thrombocytopenic Purpura: Italian Experience of Six Centers.

Journal of clinical medicine

Giovanni Tiscia, Maria Teresa Sartori, Gaetano Giuffrida, Angelo Ostuni, Nicola Cascavilla, Daniela Nicolosi, Cosima Battista, Teresa Maria Santeramo, Lorella Melillo, Giulio Giordano, Filomena Cappucci, Lucia Fischetti, Elena Chinni, Giuseppe Tarantini, Anna Cerbo, Antonella Bertomoro, Fabrizio Fabris, Elvira Grandone

Affiliations

  1. Thrombosis and Hemostasis Unit, Fondazione IRCCS Casa Sollievo della Sofferenza, 71013 San Giovanni Rotondo, Italy.
  2. Department of Internal Medicine, University of Padua, 35100 Padua, Italy.
  3. Hematology Division, Department of Clinical and Molecular Biomedicine, University of Catania, 95123 Catania, Italy.
  4. Transfusion Medicine & Blood Bank, University-Hospital of Bari, 70124 Bari, Italy.
  5. Division of Hematology, Fondazione IRCCS Casa Sollievo della Sofferenza, 71013 San Giovanni Rotondo, Italy.
  6. Division of Hematology, "Monsignor Raffaele Dimiccoli" Hospital, 70051 Barletta, Italy.
  7. Division of Hematology, University-Hospital of Foggia, 71122 Foggia, Italy.
  8. Division of Hematology, "Cardarelli" Hospital, 86100 Campobasso, Italy.
  9. Department of Obstetrics and Gynecology, First I.M. Sechenov Moscow State Medical University, 119991 Moscow, Russia.
  10. Department of Obstetrics and Gynecology, University of Foggia, 71122 Foggia, Italy.

PMID: 34884404 DOI: 10.3390/jcm10235702

Abstract

Immune-mediated thrombotic thrombocytopenic purpura is a rare and challenging hematological disease caused by the antibody anti-ADAMTS13. Though the mortality rate has decreased considerably in recent years, fatalities still remain unacceptable. This study aimed at further adding to the existing knowledge of this medical challenge. We enrolled 89 consecutive patients observed in six Italian centers (from 8 August 2013 to 28 May 2021) with a diagnosis of immune-mediated thrombotic thrombocytopenic purpura. Clinical information and blood parameters were collected for all patients. We describe clinical manifestations and laboratory data, possible risk factors and the therapeutic management of first episodes or relapses. A total of 74 first episodes and 19 relapses (median 3 years (interquartile range (IQR): 2-7)) were recorded. Seventy percent of patients enrolled at the first episode showed neurological signs and/or symptoms. All the patients enrolled at the first episode were treated with plasma exchange (median = 12; IQR: 8-19.5) and methylprednisolone (1 mg/kg/day). Rituximab (375 mg/m

Keywords: ADAMTS13; mortality; outcome; relapse; thrombotic thrombocytopenic purpura

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