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  • Trizuljak, JakubCEITEC Masaryk Univ, Univ Hosp, Dept Hematol & Oncol, Brno, Czech Republic. (author)

Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification

  • Article/chapterEnglish2020

Publisher, publication year, extent ...

  • 2020-03-16
  • Wiley,2020
  • printrdacarrier

Numbers

  • LIBRIS-ID:oai:DiVA.org:uu-420523
  • https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-420523URI
  • https://doi.org/10.1111/all.14248DOI

Supplementary language notes

  • Language:English
  • Summary in:English

Part of subdatabase

Classification

  • Subject category:ref swepub-contenttype
  • Subject category:art swepub-publicationtype

Notes

  • Background: In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM.Methods: We employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM.Results: We found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9% of progressions in typical ISM group, 1.7% in BMM, and 9.4% in SSM). Progressions to advanced SM were observed in 2.9% of these patients. In contrast, six patients with CM (1.7%) converted to ISM and no definitive progression to advanced SM was found. No significant differences in OS and event-free survival (EFS) were found when comparing ISM, BMM, and SSM. Higher risk of both progression and death was significantly associated with male gender, worse performance status, and organomegaly.Conclusion: Our data confirm the clinical impact of the WHO classification that separates ISM from CM and from other SM variants.

Subject headings and genre

Added entries (persons, corporate bodies, meetings, titles ...)

  • Sperr, Wolfgang R.Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria. (author)
  • Nekvindova, LucieMasaryk Univ, Inst Biostat & Anal Ltd, Spinoff Co, Brno, Czech Republic. (author)
  • Elberink, Hanneke O.Univ Groningen, Univ Med Ctr Groningen, Dept Allergol, Groningen, Netherlands. (author)
  • Gleixner, Karoline, VMed Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria. (author)
  • Gorska, AleksandraMed Univ Gdansk, Dept Allergol, Gdansk, Poland. (author)
  • Lange, MagdalenaMed Univ Gdansk, Dept Dermatol Venereol & Allergol, Gdansk, Poland. (author)
  • Hartmann, KarinUniv Basel, Dept Dermatol, Div Allergy, Basel, Switzerland.;Univ Cologne, Dept Dermatol, Cologne, Germany. (author)
  • Illerhaus, AnjaUniv Cologne, Dept Dermatol, Cologne, Germany. (author)
  • Bonifacio, MassimilianoUniv Verona, Dept Med, Sect Hematol, Verona, Italy. (author)
  • Perkins, CeceliaStanford Univ, Dept Med, Div Hematol, Sch Med,Stanford Canc Inst, Stanford, CA 94305 USA. (author)
  • Elena, ChiaraFdn IRCCS Policlin San Matteo, Div Hematol, Pavia, Italy.;Univ Pavia, Pavia, Italy. (author)
  • Malcovati, LucaFdn IRCCS Policlin San Matteo, Div Hematol, Pavia, Italy.;Univ Pavia, Pavia, Italy. (author)
  • Fortina, Anna B.Univ Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy. (author)
  • Shoumariyeh, KhalidUniv Freiburg, Fac Med, Med Ctr, Dept Hematol Oncol & Stem Cell Transplantat, Freiburg, Germany. (author)
  • Jawhar, MohamadHeidelberg Univ, Univ Med Mannheim, Med Klin 3, Hamatol & Onkol, Mannheim, Germany. (author)
  • Zanotti, RobertaUniv Verona, Dept Med, Sect Hematol, Verona, Italy. (author)
  • Bonadonna, PatriziaVerona Univ Hosp, Allergy Unit, Verona, Italy. (author)
  • Caroppo, FrancescaUniv Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy. (author)
  • Zink, AlexanderTech Univ Munich, Sch Med, Dept Dermatol & Allergy Biederstein, Munich, Germany. (author)
  • Triggiani, MassimoUniv Salerno, Div Allergy & Clin Immunol, Salerno, Italy. (author)
  • Parente, RobertaUniv Salerno, Div Allergy & Clin Immunol, Salerno, Italy. (author)
  • von Bubnoff, NikolasUniv Freiburg, Fac Med, Med Ctr, Dept Hematol Oncol & Stem Cell Transplantat, Freiburg, Germany. (author)
  • Yavuz, Akif S.Istanbul Univ, Istanbul Med Sch, Div Hematol, Istanbul, Turkey. (author)
  • Hägglund, HansUppsala universitet,Hematologi(Swepub:uu)hanha689 (author)
  • Mattsson, MattiasUppsala universitet,Hematologi(Swepub:uu)matma431 (author)
  • Panse, JensUniv Hosp RWTH Aachen, Dept Oncol Haematol Haemostaseol & Stem Cell Tran, Aachen, Germany. (author)
  • Jaekel, NadjaUniv Hosp Halle, Dept Internal Med 4, Halle, Germany. (author)
  • Kilbertus, AlexJohannes Kepler Univ Linz, Kepler Univ Hosp, Dept Dermatol & Venereol, Linz, Austria. (author)
  • Hermine, OlivierUniv Paris 05, Hop Necker, Assistance Publ Hop Paris APHP,INSERM U1123,Sorbo, Ctr Natl Reference Mastocytoses Imagine,Dept Hema, Paris, France. (author)
  • Arock, MichelPitle Salpetriere Hosp, Lab Hematol, Paris, France. (author)
  • Fuchs, DavidJohannes Kepler Univ Linz, Kepler Univ Hosp, Dept Internal Med Hematol & Oncol 3, Linz, Austria. (author)
  • Sabato, VitoUniv Antwerp, Fac Med & Hlth Sci, Dept Immunol Allergol Rheumatol, Antwerp, Belgium.;Antwerp Univ Hosp, Antwerp, Belgium. (author)
  • Brockow, KnutTech Univ Munich, Sch Med, Dept Dermatol & Allergy Biederstein, Munich, Germany. (author)
  • Bretterklieber, AgnesMed Univ Graz, Dept Dermatol & Venereol, Graz, Austria. (author)
  • Niedoszytko, MarekMed Univ Gdansk, Dept Allergol, Gdansk, Poland. (author)
  • van Anrooij, BjornUniv Groningen, Univ Med Ctr Groningen, Dept Allergol, Groningen, Netherlands. (author)
  • Reiter, AndreasHeidelberg Univ, Univ Med Mannheim, Med Klin 3, Hamatol & Onkol, Mannheim, Germany. (author)
  • Gotlib, JasonStanford Univ, Dept Med, Div Hematol, Sch Med,Stanford Canc Inst, Stanford, CA 94305 USA. (author)
  • Kluin-Nelemans, Hanneke C.Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands. (author)
  • Mayer, JiriCEITEC Masaryk Univ, Univ Hosp, Dept Hematol & Oncol, Brno, Czech Republic. (author)
  • Doubek, MichaelCEITEC Masaryk Univ, Univ Hosp, Dept Hematol & Oncol, Brno, Czech Republic. (author)
  • Valent, PeterMed Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria. (author)
  • CEITEC Masaryk Univ, Univ Hosp, Dept Hematol & Oncol, Brno, Czech Republic.Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria. (creator_code:org_t)

Related titles

  • In:Allergy. European Journal of Allergy and Clinical Immunology: Wiley75:8, s. 1927-19380105-45381398-9995

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