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.

Allergy Unit Verona University Hospital Verona Italy

Department of Allergology Medical University of Gdańsk Gdańsk Poland

Department of Allergology University Medical Center Groningen University of Groningen Groningen The Netherlands

Department of Dermatology and Allergy Biederstein Faculty of Medicine Technische Universität München Munich Germany

Department of Dermatology and Venereology Allergy Center Kepler University Hospital Johannes Kepler University Linz Linz Austria

Department of Dermatology and Venereology University Hospital Graz Graz Austria

Department of Dermatology Faculty of Medicine Medical Center University of Freiburg Freiburg Germany

Department of Dermatology Medical University of Gdańsk Gdańsk Poland

Department of Hematology and Oncology Medical Center Faculty of Medicine University of Freiburg Freiburg Germany; German Cancer Consortium Partner Site Freiburg Freiburg Germany

Department of Hematology and Oncology Medical Center Faculty of Medicine University of Freiburg Freiburg Germany; German Cancer Consortium Partner Site Freiburg Freiburg Germany; Department of Hematology and Oncology Medical Center University of Schleswig Holstein Campus Lübeck Lübeck Germany

Department of Hematology and Oncology University Hospital Mannheim Heidelberg University Mannheim Germany

Department of Hematology Semmelweis University Budapest Hungary

Department of Hematology University Medical Center Groningen University of Groningen Groningen The Netherlands

Department of Internal Medicine 1 Division of Hematology and Hemostaseology Medical University of Vienna Vienna Austria; Ludwig Boltzmann Institute for Hematology and Oncology Medical University of Vienna Vienna Austria

Department of Internal Medicine 1 Division of Hematology and 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 Department University Clinic for Companion Animals and Horses University of Veterinary Medicine Vienna Austria

Department of Molecular Medicine and Department of Hematology Oncology University of Pavia and Fondazione IRCCS Policlinico San Matteo Pavia Italy

Department of Oncology Haematology Haemostaseology and Stem Cell Transplantation University Hospital RWTH Aachen Aachen Germany

Department of Oncology Haematology Haemostaseology and Stem Cell Transplantation University Hospital RWTH Aachen Aachen Germany; Center for Integrated Oncology Aachen Bonn Cologne Düsseldorf Aachen Germany

Division of Allergy and Clinical Immunology University of Salerno Salerno Italy

Division of Allergy Department of Dermatology University of Basel Basel Switzerland; Department of Biomedicine University Hospital Basel and University of Basel Basel Switzerland

Division of Hematology Department of Medical Sciences Uppsala University Uppsala Sweden

Division of Hematology Department of Medicine Stanford University School of Medicine Stanford California USA

Division of Hematology Istanbul Medical School University of Istanbul Istanbul Turkey

Faculty of Medicine and Health Sciences Department of Immunology Allergology Rheumatology University of Antwerp and Antwerp University Hospital Antwerpen Belgium

French Reference Center for Mastocytosis Hôpital Necker Assistance Publique Hôpitaux de Paris Imagine Institute University Paris Descartes Paris France

KU Leuven Department of Microbiology Immunology and Transplantation Allergy and Clinical Immunology Research Group and MASTeL University Hospitals Leuven Leuven Belgium

Laboratory of Hematology Pitié Salpêtrière Hospital Paris France

Pediatric Dermatology Unit Department of Medicine University of Padova Padova Italy

Section of Hematology Department of Medicine Verona University Hospital Verona Italy

University Clinic for Hematology and Oncology Kepler University Hospital Johannes Kepler University Linz Austria

University Hospital and Faculty of Medicine Brno Czechia

University Hospital of Leipzig Leipzig Germany

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Prognostic impact of expression of CD2, CD25, and/or CD30 in/on mast cells in systemic mastocytosis: a registry study of the European Competence Network on Mastocytosis