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 Elena; Luca 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

doi:10.1016/j.jaip.2022.10.051

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 Elena

Luca 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

Department of Dermatology and Allergology

Universitätsmedizin Mannheim
University Hospital
University Medical Center Groningen
Medical Unversity of Gdansk
Medical University of Vienna
University of Veterinary Medicine Vienna
Kepler Universitätsklinikum
Azienda Ospedaliera Universitaria Integrata Verona
University of Freiburg
German Cancer Research Center
Universitätsklinikum Schleswig-Holstein Campus Lübeck
Stanford University School of Medicine
Fondazione IRCCS Policlinico San Matteo
Uppsala University
University of Salerno
Semmelweis University
University of Padova
Technical University of Munich
KU Leuven
Cerrahpasa Medical School
Faculty of Medicine of Masaryk University Brno and University Hospital
University of Antwerp and Antwerp University Hospital
Abteilung für Psychiatrie
University Hospital Leipzig
University of Basel
University Hospital Basel
Hôpital Necker Enfants Malades
AP-HP
University Hospital Cologne

Research output: Contribution to journal › Article › peer-review

7 Scopus citations

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.

Original language	English
Pages (from-to)	581-590.e5
Journal	Journal of Allergy and Clinical Immunology: In Practice
Volume	11
Issue number	2
DOIs	https://doi.org/10.1016/j.jaip.2022.10.051
State	Published - Feb 2023

Keywords

Hepatomegaly
Lymphadenopathy
Mastocytosis
Organomegaly
Splenomegaly
Systemic mastocytosis

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10.1016/j.jaip.2022.10.051

Cite this

Lübke, J., Schwaab, J., Christen, D., Elberink, H. O., Span, B., Niedoszytko, M., Gorska, A., Lange, M., Gleixner, K. V., Hadzijusufovic, E., Solomianyi, O., Angelova-Fischer, I., Zanotti, R., Bonifacio, M., Bonadonna, P., Shoumariyeh, K., von Bubnoff, N., Müller, S., Perkins, C., ... Jawhar, M. (2023). Prognostic Impact of Organomegaly in Mastocytosis: An Analysis of the European Competence Network on Mastocytosis. Journal of Allergy and Clinical Immunology: In Practice, 11(2), 581-590.e5. https://doi.org/10.1016/j.jaip.2022.10.051

@article{69aaecd2dc1740eabb3d25123beef8b7,

title = "Prognostic Impact of Organomegaly in Mastocytosis: An Analysis of the European Competence Network on Mastocytosis",

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.",

keywords = "Hepatomegaly, Lymphadenopathy, Mastocytosis, Organomegaly, Splenomegaly, Systemic mastocytosis",

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

note = "Publisher Copyright: {\textcopyright} 2022 American Academy of Allergy, Asthma \& Immunology",

year = "2023",

month = feb,

doi = "10.1016/j.jaip.2022.10.051",

language = "English",

volume = "11",

pages = "581--590.e5",

journal = "Journal of Allergy and Clinical Immunology: In Practice",

issn = "2213-2198",

publisher = "American Academy of Allergy, Asthma and Immunology",

number = "2",

}

Lübke, J, Schwaab, J, Christen, D, Elberink, HO, Span, B, Niedoszytko, M, Gorska, A, Lange, M, Gleixner, KV, Hadzijusufovic, E, Solomianyi, O, Angelova-Fischer, I, Zanotti, R, Bonifacio, M, Bonadonna, P, Shoumariyeh, K, von Bubnoff, N, Müller, S, Perkins, C, Elena, C, Malcovati, L, Hagglund, H, Mattsson, M, Parente, R, Varkonyi, J, Fortina, AB, Caroppo, F, Zink, A, Brockow, K, Breynaert, C, Bullens, D, Yavuz, AS, Doubek, M, Sabato, V, Schug, T, Niederwieser, D, Hartmann, K, Triggiani, M, Gotlib, J, Hermine, O, Arock, M, Kluin-Nelemans, HC, Panse, J, Sperr, WR, Valent, P, Reiter, A & Jawhar, M 2023, 'Prognostic Impact of Organomegaly in Mastocytosis: An Analysis of the European Competence Network on Mastocytosis', Journal of Allergy and Clinical Immunology: In Practice, vol. 11, no. 2, pp. 581-590.e5. https://doi.org/10.1016/j.jaip.2022.10.051

TY - JOUR

T1 - Prognostic Impact of Organomegaly in Mastocytosis

T2 - An Analysis of the European Competence Network on Mastocytosis

AU - Lübke, Johannes

AU - Schwaab, Juliana

AU - Christen, Deborah

AU - Elberink, Hanneke Oude

AU - Span, Bart

AU - Niedoszytko, Marek

AU - Gorska, Aleksandra

AU - Lange, Magdalena

AU - Gleixner, Karoline V.

AU - Hadzijusufovic, Emir

AU - Solomianyi, Oleksii

AU - Angelova-Fischer, Irena

AU - Zanotti, Roberta

AU - Bonifacio, Massimiliano

AU - Bonadonna, Patrizia

AU - Shoumariyeh, Khalid

AU - von Bubnoff, Nikolas

AU - Müller, Sabine

AU - Perkins, Cecelia

AU - Elena, Chiara

AU - Malcovati, Luca

AU - Hagglund, Hans

AU - Mattsson, Mattias

AU - Parente, Roberta

AU - Varkonyi, Judit

AU - Fortina, Anna Belloni

AU - Caroppo, Francesca

AU - Zink, Alexander

AU - Brockow, Knut

AU - Breynaert, Christine

AU - Bullens, Dominique

AU - Yavuz, Akif Selim

AU - Doubek, Michael

AU - Sabato, Vito

AU - Schug, Tanja

AU - Niederwieser, Dietger

AU - Hartmann, Karin

AU - Triggiani, Massimo

AU - Gotlib, Jason

AU - Hermine, Olivier

AU - Arock, Michel

AU - Kluin-Nelemans, Hanneke C.

AU - Panse, Jens

AU - Sperr, Wolfgang R.

AU - Valent, Peter

AU - Reiter, Andreas

AU - Jawhar, Mohamad

PY - 2023/2

Y1 - 2023/2

N2 - 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.

AB - 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.

KW - Hepatomegaly

KW - Lymphadenopathy

KW - Mastocytosis

KW - Organomegaly

KW - Splenomegaly

KW - Systemic mastocytosis

UR - https://www.scopus.com/pages/publications/85143857828

U2 - 10.1016/j.jaip.2022.10.051

DO - 10.1016/j.jaip.2022.10.051

M3 - Article

C2 - 36403897

AN - SCOPUS:85143857828

SN - 2213-2198

VL - 11

SP - 581-590.e5

JO - Journal of Allergy and Clinical Immunology: In Practice

JF - Journal of Allergy and Clinical Immunology: In Practice

IS - 2

ER -

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

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Keywords

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