Prognostic Impact of Organomegaly in Mastocytosis: An Analysis of the European Competence Network on Mastocytosis

Johannes Lübke, Juliana Schwaab, Deborah Christen, Hanneke Oude Elberink, Bart Span, Marek Niedoszytko, Aleksandra Gorska, Magdalena Lange, Karoline V. Gleixner, Emir Hadzijusufovic, Oleksii Solomianyi, Irena Angelova-Fischer, Roberta Zanotti, Massimiliano Bonifacio, Patrizia Bonadonna, Khalid Shoumariyeh, Nikolas von Bubnoff, Sabine Müller, Cecelia Perkins, Chiara ElenaLuca Malcovati, Hans Hagglund, Mattias Mattsson, Roberta Parente, Judit Varkonyi, Anna Belloni Fortina, Francesca Caroppo, Alexander Zink, Knut Brockow, Christine Breynaert, Dominique Bullens, Akif Selim Yavuz, Michael Doubek, Vito Sabato, Tanja Schug, Dietger Niederwieser, Karin Hartmann, Massimo Triggiani, Jason Gotlib, Olivier Hermine, Michel Arock, Hanneke C. Kluin-Nelemans, Jens Panse, Wolfgang R. Sperr, Peter Valent, Andreas Reiter, Mohamad Jawhar*

*Corresponding author for this work
1 Citation (Scopus)


Background: Organomegaly, including splenomegaly, hepatomegaly, and/or lymphadenopathy, are important diagnostic and prognostic features in patients with cutaneous mastocytosis (CM) or systemic mastocytosis (SM). Objectives: To investigate the prevalence and prognostic impact of 1 or more organomegalies on clinical course and survival in patients with CM/SM. Methods: Therefore, 3155 patients with CM (n = 1002 [32%]) or SM (n = 2153 [68%]) enrolled within the registry of the European Competence Network on Mastocytosis were analyzed. Results: Overall survival (OS) was adversely affected by the number of organomegalies (OS: #0 vs #1 hazard ratio [HR], 4.9; 95% CI, 3.4-7.1, P < .001; #1 vs #2 HR, 2.1, 95% CI, 1.4-3.1, P < .001; #2 vs #3 HR, 1.7, 95% CI, 1.2-2.5, P = .004). Lymphadenopathy was frequently detected in patients with smoldering SM (SSM, 18 of 60 [30%]) or advanced SM (AdvSM, 137 of 344 [40%]). Its presence confered an inferior outcome in patients with AdvSM compared with patients with AdvSM without lymphadenopathy (median OS, 3.8 vs 2.6 years; HR, 1.6; 95% CI, 1.2-2.2; P = .003). OS was not different between patients having organomegaly with either ISM or SSM (median, 25.5 years vs not reached; P = .435). At time of disease progression, a new occurrence of any organomegaly was observed in 17 of 40 (43%) patients with ISM, 4 of 10 (40%) patients with SSM, and 33 of 86 (38%) patients with AdvSM, respectively. Conclusions: Organomegalies including lymphadenopathy are often found in SSM and AdvSM. ISM with organomegaly has a similar course and prognosis compared with SSM. The number of organomegalies is adversely associated with OS. A new occurrence of organomegaly in all variants of SM may indicate disease progression.

Original languageEnglish
JournalJournal of Allergy and Clinical Immunology: In Practice
Issue number2
Pages (from-to)581-590.e5
Publication statusPublished - 02.2023

Research Areas and Centers

  • Research Area: Luebeck Integrated Oncology Network (LION)
  • Centers: University Cancer Center Schleswig-Holstein (UCCSH)


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