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1. The clinical experience of compassionate use program for avapritinib: implications for drug positioning in the therapeutic scenario of systemic mastocytosis

Author

Mannelli, F., Crupi, F., Zanotti, R., Pagano, Livio, Rapezzi, D., Tanasi, I., Criscuolo, Marianna, Bonifacio, M., Fresa, Alberto, Guglielmelli, P., Vannucchi, A. M., Pagano L. (ORCID:0000-0001-8287-928X), Criscuolo M., Fresa A., Mannelli, F., Crupi, F., Zanotti, R., Pagano, Livio, Rapezzi, D., Tanasi, I., Criscuolo, Marianna, Bonifacio, M., Fresa, Alberto, Guglielmelli, P., Vannucchi, A. M., Pagano L. (ORCID:0000-0001-8287-928X), Criscuolo M., and Fresa A.

Abstract

In systemic mastocytosis, cytoreductive treatment is indicated for advanced systemic mastocytosis (AdvSM) variants. The treatment scenario is rapidly diversifying especially with the introduction of KIT tyrosine kinase inhibitors. Avapritinib is a second-generation potent and selective inhibitor of the mutant KIT D816V that, based on the results of pivotal clinical trials, was approved for the treatment of adults with AdvSM by the regulatory agencies US FDA and EMA. The present article reports the experience of treating SM patients with avapritinib in an Italian compassionate use program. The data from our case series confirm the drug as being active after multiple lines of treatment allowing rapid achievement of profound responses, making it also an effective bridging strategy to allogeneic transplant in eligible patients. However, the anticipated wider use of avapritinib in the near future will require careful monitoring of side effects, especially in heavily pretreated patients.

Published
2023

2. Consolidation Radiotherapy Could Be Safely Omitted in Advanced Hodgkin Lymphoma With Large Nodal Mass in Complete Metabolic Response After ABVD: Final Analysis of the Randomized GITIL/FIL HD0607 Trial

Author

Gallamini, A, Rossi, A, Patti, C, Picardi, M, Romano, A, Cantonetti, M, Oppi, S, Viviani, S, Bolis, S, Trentin, L, Gini, G, Battistini, R, Chauvie, S, Sorasio, R, Pavoni, C, Zanotti, R, Cimminiello, M, Schiavotto, C, Viero, P, Mulé, A, Fallanca, F, Ficola, U, Tarella, C, Guerra, L, Rambaldi, A, Gallamini, Andrea, Rossi, Andrea, Patti, Caterina, Picardi, Marco, Romano, Alessandra, Cantonetti, Maria, Oppi, Sara, Viviani, Simonetta, Bolis, Silvia, Trentin, Livio, Gini, Guido, Battistini, Roberta, Chauvie, Stephane, Sorasio, Roberto, Pavoni, Chiara, Zanotti, Roberta, Cimminiello, Michele, Schiavotto, Corrado, Viero, Piera, Mulé, Antonino, Fallanca, Federico, Ficola, Umberto, Tarella, Corrado, Guerra, Luca, Rambaldi, Alessandro, Gallamini, A, Rossi, A, Patti, C, Picardi, M, Romano, A, Cantonetti, M, Oppi, S, Viviani, S, Bolis, S, Trentin, L, Gini, G, Battistini, R, Chauvie, S, Sorasio, R, Pavoni, C, Zanotti, R, Cimminiello, M, Schiavotto, C, Viero, P, Mulé, A, Fallanca, F, Ficola, U, Tarella, C, Guerra, L, Rambaldi, A, Gallamini, Andrea, Rossi, Andrea, Patti, Caterina, Picardi, Marco, Romano, Alessandra, Cantonetti, Maria, Oppi, Sara, Viviani, Simonetta, Bolis, Silvia, Trentin, Livio, Gini, Guido, Battistini, Roberta, Chauvie, Stephane, Sorasio, Roberto, Pavoni, Chiara, Zanotti, Roberta, Cimminiello, Michele, Schiavotto, Corrado, Viero, Piera, Mulé, Antonino, Fallanca, Federico, Ficola, Umberto, Tarella, Corrado, Guerra, Luca, and Rambaldi, Alessandro

Abstract

PURPOSE To investigate the role of consolidation radiotherapy (cRT) in advanced-stage Hodgkin lymphoma (HL) presenting at baseline with a large nodal mass (LNM) in complete metabolic response after doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) chemotherapy. PATIENTS AND METHODS Advanced-stage (IIB-IVB) HL patients, enrolled in the HD 0607 trial (Clinicaltrial.gov identifier NCT00795613), with both a negative PET after two (PET-2) and six (PET-6) ABVD cycles, who presented at baseline with an LNM, defined as a nodal mass with the largest diameter $ 5 cm, were prospectively randomly assigned to receive cRT over the LNM or no further treatment (NFT). RESULTS Among 296 randomly assigned patients, the largest diameter of LNM at baseline was 5-7 cm in 101 (34%; subgroup A) and 8-10 cm in 96 (32%; subgroup B), whereas classic bulky (diameter. 10 cm) was detected in 99 (33%; subgroup C). Two hundred eighty patients (88%) showed a postchemotherapy RM. The median dose of cRT was 30.6 Gy (range, 24-36 Gy). After a median follow-up of 5.9 years (range, 0.5-10 years), the 6-year progression-free survival rate of patients who underwent cRT or NFT was, respectively, 91% (95% CI, 84% to 99%) and 95% (95% CI, 89% to 100%; P 5.62) in subgroup A; 98% (95% CI, 93% to 100%) and 90% (95% CI, 80% to 100%; P 5.24) in subgroup B; 89% (95% CI, 81% to 98%) and 86% (95% CI, 77% to 96%; P 5.53) in subgroup C (classic bulky). CONCLUSION cRT could be safely omitted in patients with HL presenting with an LNM and a negative PET-2 and PET-6 scan, irrespective from the LNM size detected at baseline.

Published
2020

3. SETD2 and histone H3 lysine 36 methylation deficiency in advanced systemic mastocytosis

Author

Martinelli, G, Mancini, M, De Benedittis, C, Rondoni, M, Papayannidis, C, Manfrini, M, Meggendorfer, M, Calogero, R, Guadagnuolo, V, Fontana, M. C, Bavaro, L, Padella, A, Zago, E, Pagano, L, Zanotti, R, Scaffidi, L, Specchia, G, Albano, F, Merante, S, Elena, C, Savini, P, Gangemi, D, Tosi, P, Ciceri, F, Poletti, G, Riccioni, L, Morigi, F, Delledonne, M, Haferlach, T, Cavo, M, Valent, P, Soverini, S., Pagano, L (ORCID:0000-0001-8287-928X), Martinelli, G, Mancini, M, De Benedittis, C, Rondoni, M, Papayannidis, C, Manfrini, M, Meggendorfer, M, Calogero, R, Guadagnuolo, V, Fontana, M. C, Bavaro, L, Padella, A, Zago, E, Pagano, L, Zanotti, R, Scaffidi, L, Specchia, G, Albano, F, Merante, S, Elena, C, Savini, P, Gangemi, D, Tosi, P, Ciceri, F, Poletti, G, Riccioni, L, Morigi, F, Delledonne, M, Haferlach, T, Cavo, M, Valent, P, Soverini, S., and Pagano, L (ORCID:0000-0001-8287-928X)

Abstract

The molecular basis of advanced systemic mastocytosis (SM) is not fully understood and despite novel therapies the prognosis remains dismal. Exome sequencing of an index-patient with mast cell leukemia (MCL) uncovered biallelic loss-of-function mutations in the SETD2 histone methyltransferase gene. Copy-neutral loss-of-heterozygosity at 3p21.3 (where SETD2 maps) was subsequently found in SM patients and prompted us to undertake an in-depth analysis of SETD2 copy number, mutation status, transcript expression and methylation levels, as well as functional studies in the HMC-1 cell line and in a validation cohort of 57 additional cases with SM, including MCL, aggressive SM and indolent SM. Reduced or no SETD2 protein expression-and consequently, H3K36 trimethylation-was found in all cases and inversely correlated with disease aggressiveness. Proteasome inhibition rescued SETD2 expression and H3K36 trimethylation and resulted in marked accumulation of ubiquitinated SETD2 in SETD2-deficient patients but not in patients with near-normal SETD2 expression. Bortezomib and, to a lesser extent, AZD1775 alone or in combination with midostaurin induced apoptosis and reduced clonogenic growth of HMC-1 cells and of neoplastic mast cells from advanced SM patients. Our findings may have implications for prognostication of SM patients and for the development of improved treatment approaches in advanced SM.

Published
2018

4. Age at disease onset has a major impact on clinical characteristics and course of mastocytosis

Author

Rabenhorst, A., Sperr, W., Elberink, J. Oude, Lange, M., Gleixner, K., Brazzelli, V., Fontana, E., Vos, B., Gorska, A., Reiter, A., Schwaab, J., Zanotti, R., Bonadonna, P., Triggiani, M., Gotlib, J., Doubek, M., Jakob, T., Kilbertus, A., Sabato, V., Zink, A., Bretterklieber, A., Jaekel, N., Sotlar, K., Horny, H., Fortina, A. Belloni, Merante, S., Niedoszytko, M., Kluin-Nelemans, H., Valent, P., Hartmann, K., Rabenhorst, A., Sperr, W., Elberink, J. Oude, Lange, M., Gleixner, K., Brazzelli, V., Fontana, E., Vos, B., Gorska, A., Reiter, A., Schwaab, J., Zanotti, R., Bonadonna, P., Triggiani, M., Gotlib, J., Doubek, M., Jakob, T., Kilbertus, A., Sabato, V., Zink, A., Bretterklieber, A., Jaekel, N., Sotlar, K., Horny, H., Fortina, A. Belloni, Merante, S., Niedoszytko, M., Kluin-Nelemans, H., Valent, P., and Hartmann, K.

Published
2017

5. Impact of allergic disorders and anaphylaxis in mastocytosis: Preliminary data from a study of the European Competence Network on Mastocytosis (ECNM) registry

Author

Niedoszytko, M., Gorska, A., Nedoszytko, B., Rabenhorst, A., Sperr, W., Elberink, Oude J., Lange, M., Gleixner, K., Brazzelli, V, Fontana, E., Vos, B., Reiter, A., Schwaab, J., Zanotti, R., Bonadonna, P., Triggiani, M., Gotlib, J., Doubek, M., Jakob, T., Fuchs, D., Sabato, V, Zink, A., Bretterklieber, A., Jaekel, N., Sotlar, K., Horny, H., Fortina, Belloni A., Merante, S., Kluin-Nelemans, H., Valent, P., Hartmann, K., Niedoszytko, M., Gorska, A., Nedoszytko, B., Rabenhorst, A., Sperr, W., Elberink, Oude J., Lange, M., Gleixner, K., Brazzelli, V, Fontana, E., Vos, B., Reiter, A., Schwaab, J., Zanotti, R., Bonadonna, P., Triggiani, M., Gotlib, J., Doubek, M., Jakob, T., Fuchs, D., Sabato, V, Zink, A., Bretterklieber, A., Jaekel, N., Sotlar, K., Horny, H., Fortina, Belloni A., Merante, S., Kluin-Nelemans, H., Valent, P., and Hartmann, K.

Published
2017

6. The body mass index is an indicator of advanced systemic mastocytosis: preliminary results from a registry project of the European Competence Network on Mastocytosis (ECNM) registry

Author

Gorska, A., Gruchala-Niedoszytko, M., Niedoszytko, M., Rabenhorst, A., Sperr, W., Elberink, Oude J., Lange, M., Gleixner, K., Brazzelli, V, Fontana, E., Vos, B., Reiter, A., Schwaab, J., Zanotti, R., Bonadonna, P., Triggiani, M., Gotlib, J., Doubek, M., Jakob, T., Fuchs, D., Sabato, V, Zink, A., Bretterklieber, A., Jaekel, N., Sotlar, K., Horny, H., Fortina, Belloni A., Merante, S., Kluin-Nelemans, H., Valent, P., Hartmann, K., Gorska, A., Gruchala-Niedoszytko, M., Niedoszytko, M., Rabenhorst, A., Sperr, W., Elberink, Oude J., Lange, M., Gleixner, K., Brazzelli, V, Fontana, E., Vos, B., Reiter, A., Schwaab, J., Zanotti, R., Bonadonna, P., Triggiani, M., Gotlib, J., Doubek, M., Jakob, T., Fuchs, D., Sabato, V, Zink, A., Bretterklieber, A., Jaekel, N., Sotlar, K., Horny, H., Fortina, Belloni A., Merante, S., Kluin-Nelemans, H., Valent, P., and Hartmann, K.

Published
2017

7. Age at disease onset has a major impact on clinical characteristics and course of mastocytosis

Author

Rabenhorst, A., Sperr, W., Elberink, J. Oude, Lange, M., Gleixner, K., Brazzelli, V., Fontana, E., Vos, B., Gorska, A., Reiter, A., Schwaab, J., Zanotti, R., Bonadonna, P., Triggiani, M., Gotlib, J., Doubek, M., Jakob, T., Kilbertus, A., Sabato, V., Zink, A., Bretterklieber, A., Jaekel, N., Sotlar, K., Horny, H., Fortina, A. Belloni, Merante, S., Niedoszytko, M., Kluin-Nelemans, H., Valent, P., Hartmann, K., Rabenhorst, A., Sperr, W., Elberink, J. Oude, Lange, M., Gleixner, K., Brazzelli, V., Fontana, E., Vos, B., Gorska, A., Reiter, A., Schwaab, J., Zanotti, R., Bonadonna, P., Triggiani, M., Gotlib, J., Doubek, M., Jakob, T., Kilbertus, A., Sabato, V., Zink, A., Bretterklieber, A., Jaekel, N., Sotlar, K., Horny, H., Fortina, A. Belloni, Merante, S., Niedoszytko, M., Kluin-Nelemans, H., Valent, P., and Hartmann, K.

Published
2017

8. The body mass index is an indicator of advanced systemic mastocytosis: preliminary results from a registry project of the European Competence Network on Mastocytosis (ECNM) registry

Author

Gorska, A., Gruchala-Niedoszytko, M., Niedoszytko, M., Rabenhorst, A., Sperr, W., Elberink, Oude J., Lange, M., Gleixner, K., Brazzelli, V, Fontana, E., Vos, B., Reiter, A., Schwaab, J., Zanotti, R., Bonadonna, P., Triggiani, M., Gotlib, J., Doubek, M., Jakob, T., Fuchs, D., Sabato, V, Zink, A., Bretterklieber, A., Jaekel, N., Sotlar, K., Horny, H., Fortina, Belloni A., Merante, S., Kluin-Nelemans, H., Valent, P., Hartmann, K., Gorska, A., Gruchala-Niedoszytko, M., Niedoszytko, M., Rabenhorst, A., Sperr, W., Elberink, Oude J., Lange, M., Gleixner, K., Brazzelli, V, Fontana, E., Vos, B., Reiter, A., Schwaab, J., Zanotti, R., Bonadonna, P., Triggiani, M., Gotlib, J., Doubek, M., Jakob, T., Fuchs, D., Sabato, V, Zink, A., Bretterklieber, A., Jaekel, N., Sotlar, K., Horny, H., Fortina, Belloni A., Merante, S., Kluin-Nelemans, H., Valent, P., and Hartmann, K.

Published
2017

9. Impact of allergic disorders and anaphylaxis in mastocytosis: Preliminary data from a study of the European Competence Network on Mastocytosis (ECNM) registry

Author

Niedoszytko, M., Gorska, A., Nedoszytko, B., Rabenhorst, A., Sperr, W., Elberink, Oude J., Lange, M., Gleixner, K., Brazzelli, V, Fontana, E., Vos, B., Reiter, A., Schwaab, J., Zanotti, R., Bonadonna, P., Triggiani, M., Gotlib, J., Doubek, M., Jakob, T., Fuchs, D., Sabato, V, Zink, A., Bretterklieber, A., Jaekel, N., Sotlar, K., Horny, H., Fortina, Belloni A., Merante, S., Kluin-Nelemans, H., Valent, P., Hartmann, K., Niedoszytko, M., Gorska, A., Nedoszytko, B., Rabenhorst, A., Sperr, W., Elberink, Oude J., Lange, M., Gleixner, K., Brazzelli, V, Fontana, E., Vos, B., Reiter, A., Schwaab, J., Zanotti, R., Bonadonna, P., Triggiani, M., Gotlib, J., Doubek, M., Jakob, T., Fuchs, D., Sabato, V, Zink, A., Bretterklieber, A., Jaekel, N., Sotlar, K., Horny, H., Fortina, Belloni A., Merante, S., Kluin-Nelemans, H., Valent, P., and Hartmann, K.

Published
2017

10. Drug hypersensitivity in clonal mast cell disorders: ENDA/EAACI position paper

Author

Bonadonna, P, Pagani, M, Aberer, W, Bilò, Mb, Brockow, K, Oude Elberink, H, Garvey, L, Mosbech, H, Romano, Antonino, Zanotti, R, Torres, Mj, Romano, Antonino (ORCID:0000-0003-3762-2084), Bonadonna, P, Pagani, M, Aberer, W, Bilò, Mb, Brockow, K, Oude Elberink, H, Garvey, L, Mosbech, H, Romano, Antonino, Zanotti, R, Torres, Mj, and Romano, Antonino (ORCID:0000-0003-3762-2084)

Abstract

Mastocytosis is a clonal disorder characterized by the proliferation and accumulation of mast cells (MC) in different tissues, with a preferential localization in skin and bone marrow (BM). The excess of MC in mastocytosis as well as the increased releasability of MC may lead to a higher frequency and severity of immediate hypersensitivity reactions. Mastocytosis in adults is associated with a history of anaphylaxis in 22-49%. Fatal anaphylaxis has been described particularly following hymenoptera stings, but also occasionally after the intake of drugs such as nonsteroidal anti-inflammatory drugs, opioids and drugs in the perioperative setting. However, data on the frequency of drug hypersensitivity in mastocytosis and vice versa are scarce and evidence for an association appears to be limited. Nevertheless, clonal MC disorders should be ruled out in cases of severe anaphylaxis: basal serum tryptase determination, physical examination for cutaneous mastocytosis lesions, and clinical characteristics of anaphylactic reaction might be useful for differential diagnosis. In this position paper, the ENDA group performed a literature search on immediate drug hypersensitivity reactions in clonal MC disorders using MEDLINE, EMBASE, and Cochrane Library, reviewed and evaluated the literature in five languages using the GRADE system for quality of evidence and strength of recommendation.

Published
2015

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