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Your search keyword '"Kilbertus, Alex"' showing total 3 results
Start Over Author "Kilbertus, Alex" Publisher med univ graz, dept dermatol & venereol, graz, austria.
3 results on '"Kilbertus, Alex"'

1. Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin

Author

Fuchs, David, Kilbertus, Alex, Kofler, Karin, von Bubnoff, Nikolas, Shoumariyeh, Khalid, Zanotti, Roberta, Bonadonna, Patrizia, Scaffidi, Luigi, Doubek, Michael, Elberink, Hanneke Oude, Span, Lambert F. R., Hermine, Olivier, Elena, Chiara, Benvenuti, Pietro, Yavuz, Akif Selim, Brockow, Knut, Zink, Alexander, Aberer, Elisabeth, Gorska, Aleksandra, Romantowski, Jan, Hadzijusufovic, Emir, Fortina, Anna Belloni, Caroppo, Francesca, Perkins, Cecelia, Illerhaus, Anja, Panse, Jens, Vucinic, Vladan, Jawhar, Mohamad, Sabato, Vito, Triggiani, Massimo, Parente, Roberta, Bergström, Anna, Breynaert, Christine, Gotlib, Jason, Reiter, Andreas, Hartmann, Karin, Niedoszytko, Marek, Arock, Michel, Kluin-Nelemans, Hanneke C., Sperr, Wolfgang R., Greul, Rosemarie, Valent, Peter, Fuchs, David, Kilbertus, Alex, Kofler, Karin, von Bubnoff, Nikolas, Shoumariyeh, Khalid, Zanotti, Roberta, Bonadonna, Patrizia, Scaffidi, Luigi, Doubek, Michael, Elberink, Hanneke Oude, Span, Lambert F. R., Hermine, Olivier, Elena, Chiara, Benvenuti, Pietro, Yavuz, Akif Selim, Brockow, Knut, Zink, Alexander, Aberer, Elisabeth, Gorska, Aleksandra, Romantowski, Jan, Hadzijusufovic, Emir, Fortina, Anna Belloni, Caroppo, Francesca, Perkins, Cecelia, Illerhaus, Anja, Panse, Jens, Vucinic, Vladan, Jawhar, Mohamad, Sabato, Vito, Triggiani, Massimo, Parente, Roberta, Bergström, Anna, Breynaert, Christine, Gotlib, Jason, Reiter, Andreas, Hartmann, Karin, Niedoszytko, Marek, Arock, Michel, Kluin-Nelemans, Hanneke C., Sperr, Wolfgang R., Greul, Rosemarie, and Valent, Peter

Abstract

BACKGROUND: Mastocytosis in adults often presents with skin lesions. A bone marrow biopsy is necessary to confirm or exclude the presence of systemic mastocytosis (SM) in these cases. When a bone marrow biopsy is not performed, the provisional diagnosis is mastocytosis in the skin (MIS). No generally accepted scoring system has been established to estimate the risk of SM in these patients. OBJECTIVE: To develop a risk score to predict SM in adults with MIS. METHODS: We examined 1145 patients with MIS from the European Competence Network on Mastocytosis Registry who underwent a bone marrow biopsy. A total of 944 patients had SM and 201 patients had cutaneous mastocytosis; 63.7% were female, and 36.3% were male. Median age was 44 +/- 13.3 years. The median serum tryptase level amounted to 29.3 +/- 81.9 ng/mL. We established a multivariate regression model using the whole population of patients as a training and validation set (bootstrapping). A risk score was developed and validated with receiver-operating curves. RESULTS: In the multivariate model, the tryptase level (P < .001), constitutional/cardiovascular symptoms (P = .014), and bone symptoms/osteoporosis (P < .001) were independent predictors of SM (P < .001; sensitivity, 90.7%; specificity, 69.1%). A 6-point risk score was established (risk, 10.7%-98.0%) and validated. CONCLUSIONS: Using a large data set of the European Competence Network on Mastocytosis Registry, we created a risk score to predict the presence of SM in patients with MIS. Although the score will need further validation in independent cohorts, our score seems to discriminate safely between patients with SM and with pure cutaneous mastocytosis. (C) 2020 American Academy of Allergy, Asthma & Immunology

Published
2021
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2. Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification

Author

Trizuljak, Jakub, Sperr, Wolfgang R., Nekvindova, Lucie, Elberink, Hanneke O., Gleixner, Karoline, V, Gorska, Aleksandra, Lange, Magdalena, Hartmann, Karin, Illerhaus, Anja, Bonifacio, Massimiliano, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Fortina, Anna B., Shoumariyeh, Khalid, Jawhar, Mohamad, Zanotti, Roberta, Bonadonna, Patrizia, Caroppo, Francesca, Zink, Alexander, Triggiani, Massimo, Parente, Roberta, von Bubnoff, Nikolas, Yavuz, Akif S., Hägglund, Hans, Mattsson, Mattias, Panse, Jens, Jaekel, Nadja, Kilbertus, Alex, Hermine, Olivier, Arock, Michel, Fuchs, David, Sabato, Vito, Brockow, Knut, Bretterklieber, Agnes, Niedoszytko, Marek, van Anrooij, Bjorn, Reiter, Andreas, Gotlib, Jason, Kluin-Nelemans, Hanneke C., Mayer, Jiri, Doubek, Michael, Valent, Peter, Trizuljak, Jakub, Sperr, Wolfgang R., Nekvindova, Lucie, Elberink, Hanneke O., Gleixner, Karoline, V, Gorska, Aleksandra, Lange, Magdalena, Hartmann, Karin, Illerhaus, Anja, Bonifacio, Massimiliano, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Fortina, Anna B., Shoumariyeh, Khalid, Jawhar, Mohamad, Zanotti, Roberta, Bonadonna, Patrizia, Caroppo, Francesca, Zink, Alexander, Triggiani, Massimo, Parente, Roberta, von Bubnoff, Nikolas, Yavuz, Akif S., Hägglund, Hans, Mattsson, Mattias, Panse, Jens, Jaekel, Nadja, Kilbertus, Alex, Hermine, Olivier, Arock, Michel, Fuchs, David, Sabato, Vito, Brockow, Knut, Bretterklieber, Agnes, Niedoszytko, Marek, van Anrooij, Bjorn, Reiter, Andreas, Gotlib, Jason, Kluin-Nelemans, Hanneke C., Mayer, Jiri, Doubek, Michael, and Valent, Peter

Abstract

Background: In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM. Methods: We employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM. Results: We found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9% of progressions in typical ISM group, 1.7% in BMM, and 9.4% in SSM). Progressions to advanced SM were observed in 2.9% of these patients. In contrast, six patients with CM (1.7%) converted to ISM and no definitive progression to advanced SM was found. No significant differences in OS and event-free survival (EFS) were found when comparing ISM, BMM, and SSM. Higher risk of both progression and death was significantly associated with male gender, worse performance status, and organomegaly. Conclusion: Our data confirm the clinical impact of the WHO classification that separates ISM from CM and from other SM variants.

Published
2020
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3. Prognostic impact of eosinophils in mastocytosis : analysis of 2350 patients collected in the ECNM Registry

Author

Kluin-Nelemans, Hanneke C., Reiter, Andreas, Illerhaus, Anja, van Anrooij, Bjorn, Hartmann, Karin, Span, Lambertus F. R., Gorska, Aleksandra, Niedoszytko, Marek, Lange, Magdalena, Scaffidi, Luigi, Zanotti, Roberta, Bonadonna, Patrizia, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Shoumariyeh, Khalid, von Bubnoff, Nikolas, Parente, Roberta, Triggiani, Massimo, Schwaab, Juliana, Jawhar, Mohamad, Caroppo, Francesca, Fortina, Anna Belloni, Brockow, Knut, Zink, Alexander, Fuchs, David, Kilbertus, Alex, Yavuz, Akif Selim, Doubek, Michael, Mattsson, Mattias, Hägglund, Hans, Panse, Jens, Sabato, Vito, Aberer, Elisabeth, Niederwieser, Dietger, Breynaert, Christine, Varkonyi, Judit, Kennedy, Vanessa, Lortholary, Olivier, Jakob, Thilo, Hermine, Olivier, Rossignol, Julien, Arock, Michel, Gotlib, Jason, Valent, Peter, Sperr, Wolfgang R., Kluin-Nelemans, Hanneke C., Reiter, Andreas, Illerhaus, Anja, van Anrooij, Bjorn, Hartmann, Karin, Span, Lambertus F. R., Gorska, Aleksandra, Niedoszytko, Marek, Lange, Magdalena, Scaffidi, Luigi, Zanotti, Roberta, Bonadonna, Patrizia, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Shoumariyeh, Khalid, von Bubnoff, Nikolas, Parente, Roberta, Triggiani, Massimo, Schwaab, Juliana, Jawhar, Mohamad, Caroppo, Francesca, Fortina, Anna Belloni, Brockow, Knut, Zink, Alexander, Fuchs, David, Kilbertus, Alex, Yavuz, Akif Selim, Doubek, Michael, Mattsson, Mattias, Hägglund, Hans, Panse, Jens, Sabato, Vito, Aberer, Elisabeth, Niederwieser, Dietger, Breynaert, Christine, Varkonyi, Judit, Kennedy, Vanessa, Lortholary, Olivier, Jakob, Thilo, Hermine, Olivier, Rossignol, Julien, Arock, Michel, Gotlib, Jason, Valent, Peter, and Sperr, Wolfgang R.

Abstract

Systemic mastocytosis (SM) is frequently associated with eosinophilia. To examine its prevalence and clinical impact in all WHO classification-based subcategories, we analyzed eosinophil counts in 2350 mastocytosis patients using the dataset of the European Competence Network on Mastocytosis. Ninety percent of patients had normal eosinophil counts, 6.8% mild eosinophilia (0.5-1.5x10(9)/l), and 3.1% hypereosinophilia (HE; >1.5x10(9)/l). Eosinophilia/HE were mainly present in patients with advanced SM (17%/19%), and only rarely recorded in patients with indolent and smoldering SM (5%/1%), and some patients with cutaneous mastocytosis. The eosinophil count correlated with organomegaly, dysmyelopoiesis, and the WHO classification, but not with mediator-related symptoms or allergy. Eosinophilia at diagnosis had a strong prognostic impact (p<0.0001) on overall survival (OS) and progression-free survival (PFS), with a 10-year OS of 19% for patients with HE, 70% for those with mild eosinophilia, and 88% for patients with normal eosinophil counts. In 89% of patients with follow-up data (n=1430, censored at start of cytoreductive therapy), eosinophils remained stable. In those with changing eosinophil counts (increase/decrease or mixed pattern), OS and PFS were inferior compared with patients with stable eosinophil counts. In conclusion, eosinophilia and HE are more prevalent in advanced SM and are predictors of a worse outcome.

Published
2020
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