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

Humans Prognosis Mastocytosis / diagnosis Mastocytosis, Systemic / diagnosis Disease Progression Mastocytosis, Cutaneous Lymphadenopathy
Hepatomegaly Lymphadenopathy Mastocytosis Organomegaly Splenomegaly Systemic mastocytosis

Journal

The journal of allergy and clinical immunology. In practice
ISSN: 2213-2201
Titre abrégé: J Allergy Clin Immunol Pract
Pays: United States
ID NLM: 101597220

Informations de publication

Date de publication:
02 2023
Historique:
received: 10 05 2022
revised: 13 09 2022
accepted: 18 10 2022
pubmed: 21 11 2022
medline: 14 2 2023
entrez: 20 11 2022
Statut: ppublish

Résumé

Organomegaly, including splenomegaly, hepatomegaly, and/or lymphadenopathy, are important diagnostic and prognostic features in patients with cutaneous mastocytosis (CM) or systemic mastocytosis (SM). To investigate the prevalence and prognostic impact of 1 or more organomegalies on clinical course and survival in patients with CM/SM. 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. 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. 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.

Sections du résumé

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.

Identifiants

DOI: 10.1016/j.jaip.2022.10.051 PMID: 36403897
pubmed: 36403897
pii: S2213-2198(22)01197-7
doi: 10.1016/j.jaip.2022.10.051
pii:
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

581-590.e5

Informations de copyright

Copyright © 2022 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

Auteurs

Johannes Lübke (J)

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

Juliana Schwaab (J)

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

Deborah Christen (D)

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

Hanneke Oude Elberink (HO)

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

Bart Span (B)

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

Marek Niedoszytko (M)

Department of Allergology, Medical University of Gdańsk, Gdańsk, Poland.

Aleksandra Gorska (A)

Department of Allergology, Medical University of Gdańsk, Gdańsk, Poland.

Magdalena Lange (M)

Department of Dermatology, Medical University of Gdańsk, Gdańsk, Poland.

Karoline V 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.

Emir 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, Department/University Clinic for Companion Animals and Horses, University of Veterinary Medicine, Vienna, Austria.

Oleksii Solomianyi (O)

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

Irena Angelova-Fischer (I)

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

Roberta Zanotti (R)

Section of Hematology, Department of Medicine, Verona University Hospital, Verona, Italy.

Massimiliano Bonifacio (M)

Section of Hematology, Department of Medicine, Verona University Hospital, Verona, Italy.

Patrizia Bonadonna (P)

Allergy Unit, Verona University Hospital, Verona, Italy.

Khalid 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.

Nikolas 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, Lübeck, Germany.

Sabine Müller (S)

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

Cecelia Perkins (C)

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

Chiara Elena (C)

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

Luca Malcovati (L)

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

Hans Hagglund (H)

Division of Hematology, Department of Medical Sciences, Uppsala University, Uppsala, Sweden.

Mattias Mattsson (M)

Division of Hematology, Department of Medical Sciences, Uppsala University, Uppsala, Sweden.

Roberta Parente (R)

Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.

Judit Varkonyi (J)

Department of Hematology, Semmelweis University, Budapest, Hungary.

Anna Belloni Fortina (AB)

Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.

Francesca Caroppo (F)

Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.

Alexander Zink (A)

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

Knut Brockow (K)

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

Christine Breynaert (C)

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

Dominique Bullens (D)

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

Akif Selim Yavuz (AS)

Division of Hematology, Istanbul Medical School, University of Istanbul, Istanbul, Turkey.

Michael Doubek (M)

University Hospital and Faculty of Medicine, Brno, Czechia.

Vito Sabato (V)

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

Tanja Schug (T)

Department of Dermatology and Venereology, University Hospital Graz, Graz, Austria.

Dietger Niederwieser (D)

University Hospital of Leipzig, Leipzig, Germany.

Karin 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.

Massimo Triggiani (M)

Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.

Jason Gotlib (J)

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

Olivier Hermine (O)

French Reference Center for Mastocytosis (CEREMAST), Hôpital Necker, Assistance Publique Hôpitaux de Paris, Imagine Institute, University Paris Descartes, Paris, France.

Michel Arock (M)

Laboratory of Hematology, Pitié-Salpêtrière Hospital, Paris, France.

Hanneke C Kluin-Nelemans (HC)

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

Jens 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.

Wolfgang R 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.

Peter 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.

Andreas Reiter (A)

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

Mohamad Jawhar (M)

Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany. Electronic address: mohamad.jawhar@medma.uni-heidelberg.de.

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Classifications MeSH

questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Prognostic Impact of Organomegaly in...
questionsmedicales.fr › Diagnostic › Pronostic › Prognostic Impact of Organomegaly in...
questionsmedicales.fr › Maladies du système immunitaire › Troubles de l'activation mastocytaire › Mastocytose › Prognostic Impact of Organomegaly in...
questionsmedicales.fr › Maladies du système immunitaire › Troubles de l'activation mastocytaire › Mastocytose › Mastocytose généralisée › Prognostic Impact of Organomegaly in...
questionsmedicales.fr › États, signes et symptômes pathologiques › Processus pathologiques › Caractéristiques de la maladie › Évolution de la maladie › Prognostic Impact of Organomegaly in...
questionsmedicales.fr › Maladies du système immunitaire › Troubles de l'activation mastocytaire › Mastocytose › Mastocytose cutanée › Prognostic Impact of Organomegaly in...
questionsmedicales.fr › Hémopathies et maladies lymphatiques › Maladies lymphatiques › Lymphadénopathie › Prognostic Impact of Organomegaly in...