LIVIVO - The Search Portal for Life Sciences

zur deutschen Oberfläche wechseln
Advanced search

Search results

Result 1 - 10 of total 92

Search options

  1. Article: Bone Marrow Mastocytosis: A Diagnostic Challenge.

    Zanotti, Roberta / Tanasi, Ilaria / Bernardelli, Andrea / Orsolini, Giovanni / Bonadonna, Patrizia

    Journal of clinical medicine

    2021  Volume 10, Issue 7

    Abstract: Bone marrow mastocytosis (BMM) represents a provisional, indolent subvariant of systemic mastocytosis (SM). Utilizing WHO criteria, BMM requires bone marrow (BM) involvement and the absence of mastocytosis skin lesions. BMM is characterized by male sex ... ...

    Abstract Bone marrow mastocytosis (BMM) represents a provisional, indolent subvariant of systemic mastocytosis (SM). Utilizing WHO criteria, BMM requires bone marrow (BM) involvement and the absence of mastocytosis skin lesions. BMM is characterized by male sex prevalence, a slight increase of serum tryptase levels, low BM mast cells (MC) burden, and an indolent clinical course. BMM shows a strong correlation with severe anaphylaxis, mainly due to an IgE-mediated allergy to bee or wasp venom and, less frequently, to unexplained (idiopathic) anaphylaxis. Furthermore, BMM is often associated with osteoporosis which could be the only presenting symptom of the disease. BMM is an undervalued disease as serum tryptase levels are not routinely measured in the presence of unexplained osteoporosis or anaphylaxis. Moreover, BMM patients are often symptom-free except for severe allergic reactions. These factors, along with typical low BM MCs infiltration, may contribute to physicians overlooking BMM diagnosis, especially in medical centers that lack appropriately sensitive diagnostic techniques. This review highlights the need for a correct diagnostic pathway to diagnose BMM in patients with suspected symptoms but lacking typical skin lesions, even in the case of normal serum tryptase levels. Early diagnosis may prevent potential life-threatening anaphylaxis or severe skeletal complications.
    Language English
    Publishing date 2021-04-01
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2662592-1
    ISSN 2077-0383
    ISSN 2077-0383
    DOI 10.3390/jcm10071420
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  2. Article ; Online: Safety of local anesthesia and prevalence of hypersensitivity reactions in adult patients with clonal mast cell diseases: A retrospective single-center study.

    Tanasi, Ilaria / Olivieri, Elisa / Oberti, Margherita / Lucchini, Giuseppe / Furci, Fabiana / Zanotti, Roberta / Bonadonna, Patrizia

    The journal of allergy and clinical immunology. In practice

    2021  Volume 9, Issue 8, Page(s) 3224–3226

    MeSH term(s) Adult ; Anesthesia, Local ; Drug Hypersensitivity/diagnosis ; Drug Hypersensitivity/epidemiology ; Humans ; Hypersensitivity ; Mast Cells ; Mastocytosis/diagnosis ; Mastocytosis/epidemiology ; Prevalence ; Retrospective Studies
    Language English
    Publishing date 2021-04-20
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2843237-X
    ISSN 2213-2201 ; 2213-2198
    ISSN (online) 2213-2201
    ISSN 2213-2198
    DOI 10.1016/j.jaip.2021.03.051
    Database MEDical Literature Analysis and Retrieval System OnLINE

    Full text online

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 7086: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

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

    Mannelli, Francesco / Crupi, Francesca / Zanotti, Roberta / Pagano, Livio / Rapezzi, Davide / Tanasi, Ilaria / Criscuolo, Marianna / Bonifacio, Massimiliano / Fresa, Alberto / Guglielmelli, Paola / Vannucchi, Alessandro M

    Therapeutic advances in hematology

    2023  Volume 14, Page(s) 20406207231205643

    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- ...

    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
    Language English
    Publishing date 2023-11-02
    Publishing country England
    Document type Journal Article
    ZDB-ID 2585183-4
    ISSN 2040-6215 ; 2040-6207
    ISSN (online) 2040-6215
    ISSN 2040-6207
    DOI 10.1177/20406207231205643
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  4. Article ; Online: Bone Disease in Mastocytosis.

    Orsolini, Giovanni / Viapiana, Ombretta / Rossini, Maurizio / Bonifacio, Massimiliano / Zanotti, Roberta

    Immunology and allergy clinics of North America

    2018  Volume 38, Issue 3, Page(s) 443–454

    Abstract: Systemic mastocytosis can give very different bone pictures: from osteosclerosis to osteoporosis. Osteoporosis is one of the most frequent manifestations particularly in adults and the most clinical relevant. It is often complicated by a high recurrence ... ...

    Abstract Systemic mastocytosis can give very different bone pictures: from osteosclerosis to osteoporosis. Osteoporosis is one of the most frequent manifestations particularly in adults and the most clinical relevant. It is often complicated by a high recurrence of mainly vertebral fragility fractures. The main factor of bone loss is the osteoclast with a relative or absolute predominance of bone resorption. The RANK-RANKL pathway seems of key importance, but histamine and other cytokines also play a significant role in the process. The predominance of resorption made bisphosphonates, as anti-resorptive drugs, the most rational treatment of bone involvement in systemic mastocytosis.
    MeSH term(s) Adult ; Animals ; Bone Density Conservation Agents/therapeutic use ; Bone Diseases/immunology ; Bone Diseases/therapy ; Bone Resorption ; Diphosphonates/metabolism ; Humans ; Mastocytosis, Systemic/immunology ; Mastocytosis, Systemic/therapy ; Osteoclasts/physiology ; RANK Ligand/metabolism ; Receptor Activator of Nuclear Factor-kappa B/metabolism ; Signal Transduction
    Chemical Substances Bone Density Conservation Agents ; Diphosphonates ; RANK Ligand ; Receptor Activator of Nuclear Factor-kappa B
    Language English
    Publishing date 2018-06-09
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 92606-1
    ISSN 1557-8607 ; 0889-8561
    ISSN (online) 1557-8607
    ISSN 0889-8561
    DOI 10.1016/j.iac.2018.04.013
    Database MEDical Literature Analysis and Retrieval System OnLINE

    Full text online

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Se 710: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  5. Article: A Multidisciplinary Diagnostic Approach Reveals a Higher Prevalence of Indolent Systemic Mastocytosis: 15-Years' Experience of the GISM Network.

    Zanotti, Roberta / Bonifacio, Massimiliano / Isolan, Cecilia / Tanasi, Ilaria / Crosera, Lara / Olivieri, Francesco / Orsolini, Giovanni / Schena, Donatella / Bonadonna, Patrizia

    Cancers

    2021  Volume 13, Issue 24

    Abstract: Systemic mastocytosis (SM) and other adult clonal mast cell disorders (CMD) are often underestimated, and their epidemiology data are scarce. We aimed at evaluating the impact of the activity of the Interdisciplinary Group for Study of Mastocytosis (GISM) ...

    Abstract Systemic mastocytosis (SM) and other adult clonal mast cell disorders (CMD) are often underestimated, and their epidemiology data are scarce. We aimed at evaluating the impact of the activity of the Interdisciplinary Group for Study of Mastocytosis (GISM) of Verona on the prevalence and incidence of CMD. We examined the data of 502 adult patients diagnosed with CMD and residing in the Veneto Region, consecutively referred to GISM between 2006 and 2020. SM was diagnosed in 431 cases, while 71 patients had cutaneous mastocytosis or other CMD. Indolent SM represented the most frequent SM variant (91.0%), mainly with the characteristics of bone marrow mastocytosis (54.8%). The prevalence of SM in the adult population of the Veneto region and of the Verona province was 10.2 and 17.2/100,000 inhabitants, respectively. The mean incidence of new SM cases in Verona was 1.09/100,000 inhabitants/year. Hymenoptera venom allergy was the main reason (50%) leading to the CMD diagnosis. Osteoporosis, often complicated by fragility fractures, was present in 35% of cases, even in young patients, especially males. Our data show a higher prevalence and incidence of SM than previously reported, confirming that reference centers with multidisciplinary approach are essential for the recognition and early diagnosis of CMD.
    Language English
    Publishing date 2021-12-20
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2527080-1
    ISSN 2072-6694
    ISSN 2072-6694
    DOI 10.3390/cancers13246380
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  6. Article: Systemic Mastocytosis: Multidisciplinary Approach.

    Zanotti, Roberta / Tanasi, Ilaria / Crosera, Lara / Bonifacio, Massimiliano / Schena, Donatella / Orsolini, Giovanni / Mastropaolo, Francesca / Tebaldi, Morena / Olivieri, Elisa / Bonadonna, Patrizia

    Mediterranean journal of hematology and infectious diseases

    2021  Volume 13, Issue 1, Page(s) e2021068

    Abstract: Systemic mastocytosis (SM) is a heterogeneous group of diseases that affect almost exclusively adults and are defined by the proliferation and accumulation of clonal mast cells (MC) in various tissues. Disease subtypes range from indolent to rare ... ...

    Abstract Systemic mastocytosis (SM) is a heterogeneous group of diseases that affect almost exclusively adults and are defined by the proliferation and accumulation of clonal mast cells (MC) in various tissues. Disease subtypes range from indolent to rare aggressive forms. Although SM is classified as a rare disease, it is believed to be likely underdiagnosed. Major signs and symptoms mainly depend on MC activation and less frequent organ infiltration, typical of more aggressive variants. Diagnosis may be challenging, and symptoms can be aspecific and involve several organs. Therefore, it is advisable to refer patients to specialized centers, having sufficient knowledge of the disease, sensitive diagnostic procedures, offering a personalized and multidisciplinary diagnostic approach, including at least hematological, allergological, dermatological, and rheumatological evaluations. A precise and timely diagnosis is required for: a) adequate counseling of patients and their physicians; b) beginning of symptomatic treatment (anti-mediator therapy); c) prevention of severe manifestations of the disease (i.e., recurrent anaphylaxis, osteoporosis, and bone fractures); d) cytoreductive treatment of advanced SM variants. This review summarizes the disease's main manifestations and describes the ideal diagnostic approach for adult patients with suspected SM, giving physicians the main notions for correct patient diagnosis and management. This review also highlights the importance of a multidisciplinary approach in this very complex disease.
    Language English
    Publishing date 2021-11-01
    Publishing country Italy
    Document type Journal Article ; Review
    ZDB-ID 2674750-9
    ISSN 2035-3006
    ISSN 2035-3006
    DOI 10.4084/MJHID.2021.068
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  7. Article ; Online: Mast Cell Disorders In Drug Hypersensitivity.

    Bonadonna, Patrizia / Bonifacio, Massimiliano / Zanotti, Roberta

    Current pharmaceutical design

    2016  Volume 22, Issue 45, Page(s) 6862–6869

    Abstract: Mastocytosis is a clonal disease characterized by proliferation and accumulation of mast cells (MC) in different tissues, preferentially skin and bone marrow, leading to a wide variety of clinical manifestations, mainly caused by the inappropriate ... ...

    Abstract Mastocytosis is a clonal disease characterized by proliferation and accumulation of mast cells (MC) in different tissues, preferentially skin and bone marrow, leading to a wide variety of clinical manifestations, mainly caused by the inappropriate release of MC mediators. As a consequence, patients with mastocytosis may experience symptoms due to massive MC activation and release of mediators. Anaphylaxis is the most frequent manifestation of this phenomenon. Drugs are possible triggers of anaphylaxis in patients with mastocytosis, even though the association between mastocytosis and drug anaphylaxis does not appear to be as strong as anaphylaxis after hymenoptera sting; nevertheless, MC disorders might be ruled out in cases of severe systemic reactions to drugs. Moreover, the risk of perioperative anaphylaxis in adults appears high, mainly in patients with indolent systemic mastocytosis regardless of skin involvement. Such risk is probably lower in patients who have never experienced anaphylaxis and/or have tolerated previous general anaesthesia. However, data published about drug anaphylaxis in patients with MC disorders are scanty and currently it is not possible to provide clear recommendations.
    Language English
    Publishing date 2016
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 1304236-1
    ISSN 1873-4286 ; 1381-6128
    ISSN (online) 1873-4286
    ISSN 1381-6128
    DOI 10.2174/1381612822666160928121857
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 4714: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  8. Article ; Online: Hymenoptera Allergy and Mast Cell Activation Syndromes.

    Bonadonna, Patrizia / Bonifacio, Massimiliano / Lombardo, Carla / Zanotti, Roberta

    Current allergy and asthma reports

    2016  Volume 16, Issue 1, Page(s) 5

    Abstract: Mast cell activation syndrome (MCAS) can be diagnosed in patients with recurrent, severe symptoms from mast cell (MC)-derived mediators, which are transiently increased in serum and are attenuated by mediator-targeting drugs. When KIT-mutated, clonal MC ... ...

    Abstract Mast cell activation syndrome (MCAS) can be diagnosed in patients with recurrent, severe symptoms from mast cell (MC)-derived mediators, which are transiently increased in serum and are attenuated by mediator-targeting drugs. When KIT-mutated, clonal MC are detected in these patients, a diagnosis of primary MCAS can be made. Severe systemic reactions to hymenoptera venom (HV) represent the most common form of anaphylaxis in patients with mastocytosis. Patients with primary MCAS and HV anaphylaxis are predominantly males and do not have skin lesions in the majority of cases, and anaphylaxis is characterized by hypotension and syncope in the absence of urticaria and angioedema. A normal value of tryptase (≤11.4 ng/ml) in these patients does not exclude a diagnosis of mastocytosis. Patients with primary MCAS and HV anaphylaxis have to undergo lifelong venom immunotherapy, in order to prevent further potentially fatal severe reactions.
    MeSH term(s) Allergens/immunology ; Anaphylaxis/immunology ; Animals ; Arthropod Venoms/adverse effects ; Arthropod Venoms/immunology ; Female ; Humans ; Hymenoptera/immunology ; Male ; Mast Cells/immunology ; Mastocytosis/diagnosis ; Mastocytosis/immunology ; Syndrome ; Tryptases/blood
    Chemical Substances Allergens ; Arthropod Venoms ; Tryptases (EC 3.4.21.59)
    Language English
    Publishing date 2016-01
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2057370-4
    ISSN 1534-6315 ; 1529-7322
    ISSN (online) 1534-6315
    ISSN 1529-7322
    DOI 10.1007/s11882-015-0582-5
    Database MEDical Literature Analysis and Retrieval System OnLINE

    Full text online

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 5548: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  9. Article ; Online: Comparison between indolent systemic mastocytosis and clonal mast cell disease not meeting WHO diagnostic criteria: A nationwide multicenter retrospective analysis.

    Sciumè, Mariarita / Serpenti, Fabio / Zanotti, Roberta / Bonadonna, Patrizia / Tanasi, Ilaria / Crosera, Lara / Elena, Chiara / Mannelli, Francesco / Crupi, Francesca / Papayannidis, Cristina / Sartor, Chiara / Soverini, Simona / Rondoni, Michela / Eller-Vainicher, Cristina / Pravettoni, Valerio / Rivolta, Federica / Alberti Violetti, Silvia / Croci, Giorgio Alberto / Migliorini, Anna Chiara /
    Bolli, Niccolò / Giannarelli, Diana / Grifoni, Federica Irene

    Hematological oncology

    2024  Volume 42, Issue 3, Page(s) e3277

    MeSH term(s) Humans ; Mastocytosis, Systemic/diagnosis ; Mastocytosis, Systemic/pathology ; Retrospective Studies ; Male ; Female ; Middle Aged ; Aged ; World Health Organization ; Adult
    Language English
    Publishing date 2024-04-30
    Publishing country England
    Document type Letter ; Multicenter Study ; Comparative Study
    ZDB-ID 604884-5
    ISSN 1099-1069 ; 0278-0232
    ISSN (online) 1099-1069
    ISSN 0278-0232
    DOI 10.1002/hon.3277
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 1816: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  10. Article: A case of high-risk AML in a patient with advanced systemic mastocytosis.

    Fazio, Manlio / Vetro, Calogero / Markovic, Uroš / Duminuco, Andrea / Parisi, Marina / Maugeri, Cinzia / Mauro, Elisa / Parrinello, Nunziatina Laura / Stagno, Fabio / Villari, Loredana / Triolo, Anna Maria / Stella, Stefania / Palumbo, Giuseppe A / Di Raimondo, Francesco / Romano, Alessandra / Zanotti, Roberta

    Clinical case reports

    2023  Volume 11, Issue 7, Page(s) e7134

    Abstract: Aggressive SM + AML has limited therapeutic options. Even a strong combination of decitabine-venetoclax-midostaurin has a transient effect on AML and a mitigated effect on SM. Larger series are required to identify the best therapeutic strategy. ...

    Abstract Aggressive SM + AML has limited therapeutic options. Even a strong combination of decitabine-venetoclax-midostaurin has a transient effect on AML and a mitigated effect on SM. Larger series are required to identify the best therapeutic strategy.
    Language English
    Publishing date 2023-07-17
    Publishing country England
    Document type Case Reports
    ZDB-ID 2740234-4
    ISSN 2050-0904
    ISSN 2050-0904
    DOI 10.1002/ccr3.7134
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

To top