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Prognostic Impact of Organomegaly in Mastocytosis: An Analysis of the European Competence Network on Mastocytosis.
Lübke, Johannes; Schwaab, Juliana; Christen, Deborah; Elberink, Hanneke Oude; Span, Bart; Niedoszytko, Marek; Gorska, Aleksandra; Lange, Magdalena; Gleixner, Karoline V; Hadzijusufovic, Emir; Solomianyi, Oleksii; Angelova-Fischer, Irena; Zanotti, Roberta; Bonifacio, Massimiliano; Bonadonna, Patrizia; Shoumariyeh, Khalid; von Bubnoff, Nikolas; Müller, Sabine; Perkins, Cecelia; Elena, Chiara; Malcovati, Luca; Hagglund, Hans; Mattsson, Mattias; Parente, Roberta; Varkonyi, Judit; Fortina, Anna Belloni; Caroppo, Francesca; Zink, Alexander; Brockow, Knut; Breynaert, Christine; Bullens, Dominique; Yavuz, Akif Selim; Doubek, Michael; Sabato, Vito; Schug, Tanja; Niederwieser, Dietger; Hartmann, Karin; Triggiani, Massimo; Gotlib, Jason; Hermine, Olivier; Arock, Michel; Kluin-Nelemans, Hanneke C; Panse, Jens; Sperr, Wolfgang R; Valent, Peter; Reiter, Andreas; Jawhar, Mohamad.
Affiliation
  • Lübke J; Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.
  • Schwaab J; Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.
  • Christen D; Department of Oncology, Haematology, Haemostaseology and Stem Cell Transplantation, University Hospital RWTH Aachen, Aachen, Germany.
  • Elberink HO; Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
  • Span B; Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
  • Niedoszytko M; Department of Allergology, Medical University of Gdansk, Gdansk, Poland.
  • Gorska A; Department of Allergology, Medical University of Gdansk, Gdansk, Poland.
  • Lange M; Department of Dermatology, Medical University of Gdansk, Gdansk, Poland.
  • Gleixner KV; Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.
  • Hadzijusufovic E; Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria; Internal Medicine Small Animals, University Clinic for Small Animals,
  • Solomianyi O; University Clinic for Hematology and Oncology, Kepler University Hospital, Johannes Kepler University, Linz, Austria.
  • Angelova-Fischer I; Department of Dermatology and Venereology, Allergy Center, Kepler University Hospital, Johannes Kepler University Linz, Linz, Austria.
  • Zanotti R; Section of Hematology, Department of Medicine, Verona University Hospital, Verona, Italy.
  • Bonifacio M; Section of Hematology, Department of Medicine, Verona University Hospital, Verona, Italy.
  • Bonadonna P; Allergy Unit, Verona University Hospital, Verona, Italy.
  • Shoumariyeh K; Department of Hematology and Oncology, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany; German Cancer Consortium (DKTK) Partner Site Freiburg, Freiburg, Germany.
  • von Bubnoff N; Department of Hematology and Oncology, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany; German Cancer Consortium (DKTK) Partner Site Freiburg, Freiburg, Germany; Department of Hematology and Oncology, Medical Center, University of Schleswig-Holstein, Campus Lübeck, Lüb
  • Müller S; Department of Dermatology, Faculty of Medicine, Medical Center, University of Freiburg, Freiburg, Germany.
  • Perkins C; Division of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, California, USA.
  • Elena C; Department of Molecular Medicine and Department of Hematology Oncology, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
  • Malcovati L; Department of Molecular Medicine and Department of Hematology Oncology, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
  • Hagglund H; Division of Hematology, Department of Medical Sciences, Uppsala University, Uppsala, Sweden.
  • Mattsson M; Division of Hematology, Department of Medical Sciences, Uppsala University, Uppsala, Sweden.
  • Parente R; Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
  • Varkonyi J; Department of Hematology, Semmelweis University, Budapest, Hungary.
  • Fortina AB; Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.
  • Caroppo F; Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.
  • Zink A; Department of Dermatology and Allergy Biederstein, Faculty of Medicine, Technische Universität München, Munich, Germany.
  • Brockow K; Department of Dermatology and Allergy Biederstein, Faculty of Medicine, Technische Universität München, Munich, Germany.
  • Breynaert C; KU Leuven Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group and MASTeL, University Hospitals Leuven, Leuven, Belgium.
  • Bullens D; KU Leuven Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group and MASTeL, University Hospitals Leuven, Leuven, Belgium.
  • Yavuz AS; Division of Hematology, Istanbul Medical School, University of Istanbul, Istanbul, Turkey.
  • Doubek M; University Hospital and Faculty of Medicine, Brno, Czechia.
  • Sabato V; Faculty of Medicine and Health Sciences, Department of Immunology-Allergology-Rheumatology, University of Antwerp and Antwerp University Hospital, Antwerpen, Belgium.
  • Schug T; Department of Dermatology and Venereology, University Hospital Graz, Graz, Austria.
  • Niederwieser D; University Hospital of Leipzig, Leipzig, Germany.
  • Hartmann K; Division of Allergy, Department of Dermatology, University of Basel, Basel, Switzerland; Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland.
  • Triggiani M; Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
  • Gotlib J; Division of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, California, USA.
  • Hermine O; French Reference Center for Mastocytosis (CEREMAST), Hôpital Necker, Assistance Publique Hôpitaux de Paris, Imagine Institute, University Paris Descartes, Paris, France.
  • Arock M; Laboratory of Hematology, Pitié-Salpêtrière Hospital, Paris, France.
  • Kluin-Nelemans HC; Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
  • Panse J; Department of Oncology, Haematology, Haemostaseology and Stem Cell Transplantation, University Hospital RWTH Aachen, Aachen, Germany; Center for Integrated Oncology, Aachen Bonn Cologne Düsseldorf (CIO ABCD), Aachen, Germany.
  • Sperr WR; Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.
  • Valent P; Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.
  • Reiter A; Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.
  • Jawhar M; Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany. Electronic address: mohamad.jawhar@medma.uni-heidelberg.de.
J Allergy Clin Immunol Pract ; 11(2): 581-590.e5, 2023 02.
Article in En | MEDLINE | ID: mdl-36403897
ABSTRACT

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.
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Full text: 1 Database: MEDLINE Main subject: Mastocytosis / Mastocytosis, Cutaneous / Mastocytosis, Systemic / Lymphadenopathy Type of study: Prognostic_studies / Risk_factors_studies Limits: Humans Language: En Year: 2023 Type: Article
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Full text: 1 Database: MEDLINE Main subject: Mastocytosis / Mastocytosis, Cutaneous / Mastocytosis, Systemic / Lymphadenopathy Type of study: Prognostic_studies / Risk_factors_studies Limits: Humans Language: En Year: 2023 Type: Article