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The difficulty to diagnose and treat a rare disease: response to midostaurin in a case of aggressive systemic mastocytosis conditioning bulky lymphadenopathy
  • +11
  • Mariarita Sciumé,
  • Fabio Serpenti,
  • Simona Muratori,
  • Valerio Pravettoni,
  • Fabio Massimo Ulivieri,
  • Giorgio Alberto Croci,
  • Anna Chiara Migliorini,
  • Andrea Esposito,
  • Maria Cecilia Goldaniga,
  • Giorgia Natascia Saporiti,
  • Giulia Galassi,
  • Luca Baldini,
  • Francesco Onida,
  • Federica Irene Grifoni
Mariarita Sciumé
Hematology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
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Fabio Serpenti
Università degli Studi di Milano, Milan, Italy
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Simona Muratori
Dermatology Unit, Department of Physiopathology and Transplantation, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Università di Milano
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Valerio Pravettoni
U.O.C. General Medicine - Immunology and Allergology, Fondazione Ca' Granda IRCCS, Ospedale Maggiore Policlinico, Università di Milano
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Fabio Massimo Ulivieri
Nuclear Medicine, Bone Metabolic Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Università di Milano
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Giorgio Alberto Croci
Division of Pathology, Department of Pathophysiology and Transplantation, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
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Anna Chiara Migliorini
Laboratorio di ematologia, Servizio di immunoematologia e trasfusionale, Fondazione Ca' Granda IRCCS Ospedale Maggiore Policlinico
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Andrea Esposito
Division of Radiology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
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Maria Cecilia Goldaniga
Hematology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
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Giorgia Natascia Saporiti
Hematology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
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Giulia Galassi
Hematology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
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Luca Baldini
Hematology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
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Francesco Onida
Hematology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
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Federica Irene Grifoni
Hematology Unit, Fondazione Ca' Granda IRCCS, Ospedale Maggiore Policlinico, Università di Milano
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Abstract

Systemic mastocytosis (SM) can be extremely heterogeneous and its treatment should be highly individualized. We reported a description of clinical management of an aggressive SM characterized at diagnosis by multiple organ involvement. The patient underwent to different lines of treatment, from standard chemotherapy to the novel tyrosine kinase inhibitor midostaurin.

Peer review status:UNDER REVIEW

06 Jun 2020Submitted to Clinical Case Reports
12 Jun 2020Assigned to Editor
12 Jun 2020Submission Checks Completed
25 Jun 2020Reviewer(s) Assigned