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Article: SRC-Family Kinases in Acute Myeloid Leukaemia and Mastocytosis.

Voisset, Edwige / Brenet, Fabienne / Lopez, Sophie / de Sepulveda, Paulo

2020 Volume 12, Issue 7

Abstract: Protein tyrosine kinases have been recognized as important actors of cell transformation and cancer progression, since their discovery as products of viral oncogenes. SRC-family kinases (SFKs) play crucial roles in normal hematopoiesis. Not surprisingly, ...

Abstract	Protein tyrosine kinases have been recognized as important actors of cell transformation and cancer progression, since their discovery as products of viral oncogenes. SRC-family kinases (SFKs) play crucial roles in normal hematopoiesis. Not surprisingly, they are hyperactivated and are essential for membrane receptor downstream signaling in hematological malignancies such as acute myeloid leukemia (AML) and mastocytosis. The precise roles of SFKs are difficult to delineate due to the number of substrates, the functional redundancy among members, and the use of tools that are not selective. Yet, a large num ber of studies have accumulated evidence to support that SFKs are rational therapeutic targets in AML and mastocytosis. These two pathologies are regulated by two related receptor tyrosine kinases, which are well known in the field of hematology: FLT3 and KIT. FLT3 is one of the most frequently mutated genes in AML, while KIT oncogenic mutations occur in 80-90% of mastocytosis. Studies on oncogenic FLT3 and KIT signaling have shed light on specific roles for members of the SFK family. This review highlights the central roles of SFKs in AML and mastocytosis, and their interconnection with FLT3 and KIT oncoproteins.
Language	English
Publishing date	2020-07-21
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2527080-1
ISSN	2072-6694
ISSN	2072-6694
DOI	10.3390/cancers12071996
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Article: Cutting the brakes on hematopoietic regeneration by blocking TGFβ to limit chemotherapy-induced myelosuppression.

Brenet, Fabienne / Scandura, Joseph M

Molecular & cellular oncology

2015 Volume 2, Issue 3, Page(s) e978703

Abstract: Hematopoietic stressors such as infection, bleeding, or toxic injury trigger a hematopoietic adaptation that sacrifices hematopoietic stem and progenitor cell (HSPC) quiescence to meet an urgent need for new blood cell production. Once the hematopoietic ... ...

Abstract	Hematopoietic stressors such as infection, bleeding, or toxic injury trigger a hematopoietic adaptation that sacrifices hematopoietic stem and progenitor cell (HSPC) quiescence to meet an urgent need for new blood cell production. Once the hematopoietic demands are adequately met, homeostasis must be restored. Transforming growth factor β (TGFβ) signaling is a central mediator mandating the return of HSPCs to quiescence after stress. Blockade of TGFβ signaling after hematopoietic stress delays the return of cycling HSPCs to quiescence and in so doing promotes hematopoietic stem cell (HSC) self-renewal and accelerates hematopoietic reconstitution. These findings open the door to new therapeutics that modulate the hematopoietic adaptation to stress. In this review, we will discuss the complex context-dependent activities of TGFβ in hematopoiesis and the potential benefits and limitations of using TGFβ pathway inhibitors to promote multilineage hematopoietic reconstitution after myelosuppressive chemotherapy.
Language	English
Publishing date	2015-04-18
Publishing country	United States
Document type	Journal Article ; Review
ISSN	2372-3556
ISSN	2372-3556
DOI	10.4161/23723556.2014.978703
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Article: Contribution de la voie du TGFβ à la régénération hématopoïétique après chimiothérapie myélosuppressive.

Brenet, Fabienne / Scandura, Joseph M

Medecine sciences : M/S

2013 Volume 29, Issue 11, Page(s) 940–942

Title translation	TGFβ contribution to hematopoietic regeneration after myelosuppressive chemotherapy.
MeSH term(s)	Animals ; Antineoplastic Agents/adverse effects ; Bone Marrow Cells/physiology ; Hematopoiesis/drug effects ; Hematopoiesis/physiology ; Hematopoietic Stem Cells/physiology ; Humans ; Regeneration ; Transforming Growth Factor beta/physiology
Chemical Substances	Antineoplastic Agents ; Transforming Growth Factor beta
Language	French
Publishing date	2013-11-20
Publishing country	France
Document type	News
ZDB-ID	632733-3
ISSN	1958-5381 ; 0767-0974
ISSN (online)	1958-5381
ISSN	0767-0974
DOI	10.1051/medsci/20132911003
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Article ; Online: Molecular basis of mast cell disease.

Soucie, Erinn / Brenet, Fabienne / Dubreuil, Patrice

Molecular immunology

2014 Volume 63, Issue 1, Page(s) 55–60

Abstract: Mastocytosis is an incurable and sometimes fatal haematological disorder grossly described as the accumulation of abnormal mast cells in the bone marrow and other organs causing tissue and organ damage. The clinical manifestations of this disease are ... ...

Abstract	Mastocytosis is an incurable and sometimes fatal haematological disorder grossly described as the accumulation of abnormal mast cells in the bone marrow and other organs causing tissue and organ damage. The clinical manifestations of this disease are extremely variable; disease phenotypes range from indolent to aggressive, and often present with associated non-mast cell haematological disorders (AHNMD), mainly myeloproliferative neoplasm and myelodysplastic syndromes. Recent efforts to genetically dissect the mechanisms that define aggressive and non-aggressive mastocytosis have generated a list of recurrent somatic mutations in mastocytosis patients that are associated with and may predict the evolution towards aggressive disease phenotypes. Here we review these mutations and discuss the molecular mechanisms associated with these mutations in an effort to better understand the biology of this disease and to predict its onset and evolution, with the ultimate goal of devising new and improved treatment strategies.
MeSH term(s)	DNA-Binding Proteins/genetics ; Dioxygenases ; Humans ; Isocitrate Dehydrogenase/genetics ; Mast Cells/immunology ; Mastocytosis/genetics ; Mastocytosis/immunology ; Mutation ; Nuclear Proteins/genetics ; Phosphoproteins/genetics ; Proto-Oncogene Proteins/genetics ; Proto-Oncogene Proteins c-kit/genetics ; RNA Splicing Factors ; Repressor Proteins/genetics ; Ribonucleoprotein, U2 Small Nuclear/genetics ; Ribonucleoproteins/genetics ; Serine-Arginine Splicing Factors ; Splicing Factor U2AF
Chemical Substances	ASXL1 protein, human ; DNA-Binding Proteins ; Nuclear Proteins ; Phosphoproteins ; Proto-Oncogene Proteins ; RNA Splicing Factors ; Repressor Proteins ; Ribonucleoprotein, U2 Small Nuclear ; Ribonucleoproteins ; SF3B1 protein, human ; Splicing Factor U2AF ; U2AF1 protein, human ; SRSF2 protein, human (147153-65-9) ; Serine-Arginine Splicing Factors (170974-22-8) ; IDH2 protein, human (EC 1.1.1.41) ; Isocitrate Dehydrogenase (EC 1.1.1.41) ; Dioxygenases (EC 1.13.11.-) ; TET2 protein, human (EC 1.13.11.-) ; Proto-Oncogene Proteins c-kit (EC 2.7.10.1)
Language	English
Publishing date	2014-04-24
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	424427-8
ISSN	1872-9142 ; 0161-5890
ISSN (online)	1872-9142
ISSN	0161-5890
DOI	10.1016/j.molimm.2014.03.013
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Article: Molecular basis of mast cell disease

Soucie, Erinn / Fabienne Brenet / Patrice Dubreuil

Molecular Immunology. 2015 Jan., v. 63

2015

Abstract	Mastocytosis is an incurable and sometimes fatal haematological disorder grossly described as the accumulation of abnormal mast cells in the bone marrow and other organs causing tissue and organ damage. The clinical manifestations of this disease are extremely variable; disease phenotypes range from indolent to aggressive, and often present with associated non-mast cell haematological disorders (AHNMD), mainly myeloproliferative neoplasm and myelodysplastic syndromes. Recent efforts to genetically dissect the mechanisms that define aggressive and non-aggressive mastocytosis have generated a list of recurrent somatic mutations in mastocytosis patients that are associated with and may predict the evolution towards aggressive disease phenotypes. Here we review these mutations and discuss the molecular mechanisms associated with these mutations in an effort to better understand the biology of this disease and to predict its onset and evolution, with the ultimate goal of devising new and improved treatment strategies.
Keywords	bone marrow cells ; evolution ; mast cells ; patients ; phenotype ; somatic mutation
Language	English
Dates of publication	2015-01
Size	p. 55-60.
Publishing place	Elsevier Ltd
Document type	Article
ZDB-ID	424427-8
ISSN	1872-9142 ; 0161-5890
ISSN (online)	1872-9142
ISSN	0161-5890
DOI	10.1016/j.molimm.2014.03.013
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Article ; Online: Megakaryocyte TGFβ1 partitions erythropoiesis into immature progenitor/stem cells and maturing precursors.

Di Giandomenico, Silvana / Kermani, Pouneh / Mollé, Nicole / Yabut, Maria Mia / Abu-Zeinah, Ghaith / Stephens, Thomas / Messali, Nassima / Schreiner, Ryan / Brenet, Fabienne / Rafii, Shahin / Scandura, Joseph M

Blood

2020 Volume 136, Issue 9, Page(s) 1044–1054

Abstract: Erythropoietin (EPO) provides the major survival signal to maturing erythroid precursors (EPs) and is essential for terminal erythropoiesis. Nonetheless, progenitor cells can irreversibly commit to an erythroid fate well before EPO acts, risking ... ...

Abstract	Erythropoietin (EPO) provides the major survival signal to maturing erythroid precursors (EPs) and is essential for terminal erythropoiesis. Nonetheless, progenitor cells can irreversibly commit to an erythroid fate well before EPO acts, risking inefficiency if these progenitors are unneeded to maintain red blood cell (RBC) counts. We identified a new modular organization of erythropoiesis and, for the first time, demonstrate that the pre-EPO module is coupled to late EPO-dependent erythropoiesis by megakaryocyte (Mk) signals. Disrupting megakaryocytic transforming growth factor β1 (Tgfb1) disorganized hematopoiesis by expanding the pre-EPO pool of progenitor cells and consequently triggering significant apoptosis of EPO-dependent EPs. Similarly, pharmacologic blockade of TGFβ signaling in normal mice boosted the pre-EPO module, leading to apoptosis of EPO-sensitive EPs. Subsequent treatment with low-dose EPO triggered robust RBC production in both models. This work reveals modular regulation of erythropoiesis and offers a new strategy for overcoming chronic anemias.
MeSH term(s)	Animals ; Apoptosis/drug effects ; Bone Marrow/pathology ; Erythroid Precursor Cells/cytology ; Erythroid Precursor Cells/metabolism ; Erythropoiesis/physiology ; Erythropoietin/pharmacology ; Gene Knockout Techniques ; Hematopoietic Stem Cells/cytology ; Hematopoietic Stem Cells/metabolism ; Immunophenotyping ; Megakaryocyte-Erythroid Progenitor Cells/cytology ; Megakaryocyte-Erythroid Progenitor Cells/metabolism ; Megakaryocytes/cytology ; Megakaryocytes/metabolism ; Mice ; Mice, Inbred C57BL ; Mice, Transgenic ; Radiation Chimera ; Recombinant Proteins/pharmacology ; Transforming Growth Factor beta1/antagonists & inhibitors ; Transforming Growth Factor beta1/genetics ; Transforming Growth Factor beta1/pharmacology ; Transforming Growth Factor beta1/physiology
Chemical Substances	EPO protein, human ; Recombinant Proteins ; Tgfb1 protein, mouse ; Transforming Growth Factor beta1 ; Erythropoietin (11096-26-7)
Language	English
Publishing date	2020-05-19
Publishing country	United States
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
ZDB-ID	80069-7
ISSN	1528-0020 ; 0006-4971
ISSN (online)	1528-0020
ISSN	0006-4971
DOI	10.1182/blood.2019003276
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Article ; Online: GlcNAc is a mast-cell chromatin-remodeling oncometabolite that promotes systemic mastocytosis aggressiveness.

Agopian, Julie / Da Costa, Quentin / Nguyen, Quang Vo / Scorrano, Giulia / Kousteridou, Paraskevi / Yuan, Min / Chelbi, Rabie / Goubard, Armelle / Castellano, Remy / Maurizio, Julien / Teodosio, Cristina / De Sepulveda, Paulo / Asara, John M / Orfao, Alberto / Hermine, Olivier / Dubreuil, Patrice / Brenet, Fabienne

Blood

2021 Volume 138, Issue 17, Page(s) 1590–1602

Abstract: Systemic mastocytosis (SM) is a KIT-driven hematopoietic neoplasm characterized by the excessive accumulation of neoplastic mast cells (MCs) in various organs and, mainly, the bone marrow (BM). Multiple genetic and epigenetic mechanisms contribute to the ...

Abstract	Systemic mastocytosis (SM) is a KIT-driven hematopoietic neoplasm characterized by the excessive accumulation of neoplastic mast cells (MCs) in various organs and, mainly, the bone marrow (BM). Multiple genetic and epigenetic mechanisms contribute to the onset and severity of SM. However, little is known to date about the metabolic underpinnings underlying SM aggressiveness, which has thus far impeded the development of strategies to leverage metabolic dependencies when existing KIT-targeted treatments fail. Here, we show that plasma metabolomic profiles were able to discriminate indolent from advanced forms of the disease. We identified N-acetyl-d-glucosamine (GlcNAc) as the most predictive metabolite of SM severity. High plasma levels of GlcNAc in patients with advanced SM correlated with the activation of the GlcNAc-fed hexosamine biosynthesis pathway in patients BM aspirates and purified BM MCs. At the functional level, GlcNAc enhanced human neoplastic MCs proliferation and promoted rapid health deterioration in a humanized mouse model of SM. In addition, in the presence of GlcNAc, immunoglobulin E-stimulated MCs triggered enhanced release of proinflammatory cytokines and a stronger acute response in a mouse model of passive cutaneous anaphylaxis. Mechanistically, elevated GlcNAc levels promoted the transcriptional accessibility of chromatin regions that contain genes encoding mediators of receptor tyrosine kinases cascades and inflammatory responses, thus leading to a more aggressive phenotype. Therefore, GlcNAc is an oncometabolite driver of SM aggressiveness. This study suggests the therapeutic potential for targeting metabolic pathways in MC-related diseases to manipulate MCs effector functions.
MeSH term(s)	Acetylglucosamine/analysis ; Acetylglucosamine/metabolism ; Adult ; Animals ; Chromatin Assembly and Disassembly ; Disease Progression ; Humans ; Mast Cells/metabolism ; Mast Cells/pathology ; Mastocytosis, Systemic/genetics ; Mastocytosis, Systemic/metabolism ; Mastocytosis, Systemic/pathology ; Metabolome ; Mice, SCID ; Prospective Studies ; Mice
Chemical Substances	Acetylglucosamine (V956696549)
Language	English
Publishing date	2021-05-11
Publishing country	United States
Document type	Journal Article ; Multicenter Study ; Research Support, Non-U.S. Gov't
ZDB-ID	80069-7
ISSN	1528-0020 ; 0006-4971
ISSN (online)	1528-0020
ISSN	0006-4971
DOI	10.1182/blood.2020008948
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Article ; Online: Histological characterization of liver involvement in systemic mastocytosis.

Rossignol, Julien / Canioni, Danielle / Aouba, Achille / Bulai-Livideanu, Cristina / Barete, Stéphane / Lancesseur, Charles / Polivka, Laura / Madrange, Marine / Ballul, Thomas / Neuraz, Antoine / Greco, Celine / Agopian, Julie / Brenet, Fabienne / Dubreuil, Patrice / Lemal, Richard / Tournilhac, Olivier / Terriou, Louis / Launay, David / Bouillet, Laurence /

Gourguechon, Clément / Frenzel, Laurent / Meni, Cécile / Gaudy-Marqueste, Caroline / Gousseff, Marie / Le Mouel, Edwige / Hamidou, Mohamed / Neel, Antoine / Ranta, Dana / Jaussaud, Roland / Guilpain, Philippe / Molina, Thierry J / Bruneau, Julie / Lhermitte, Ludovic / Garcelon, Nicolas / Javier, Rose-Marie / Pelletier, Fabien / Castelain, Florence / Retornaz, Frederique / Cabrera, Quentin / Zunic, Patricia / Gourin, Marie Pierre / Wierzbicka-Hainaut, Ewa / Viallard, Jean François / Lavigne, Christian / Hoarau, Cyrille / Durieu, Isabelle / Heiblig, Maël / Dimicoli-Salazar, Sophie / Torregrosa-Diaz, Jose M / Soria, Angèle / Arock, Michel / Lortholary, Olivier / Bodemer, Christine / Pol, Stanislas / Mallet, Vincent / Hermine, Olivier / Damaj, Ghandi

Liver international : official journal of the International Association for the Study of the Liver

2024

Abstract: Background and aims: Systemic mastocytosis (SM) is characterized by the accumulation of atypical mast cells (MCs) in organs. Liver histology of SM has been marginally described and accurate histological classification is critical, given the consequences ...

Abstract	Background and aims: Systemic mastocytosis (SM) is characterized by the accumulation of atypical mast cells (MCs) in organs. Liver histology of SM has been marginally described and accurate histological classification is critical, given the consequences of aggressive SM diagnosis. We aimed to describe the histological features associated with liver SM using updated tools. Methods: Using the database of the French Reference Centre for Mastocytosis, we retrospectively identified patients with a liver biopsy (LB) and a diagnosis of SM. All LB procedures were performed according to the local physician in charge and centrally reviewed by an expert pathologist. Results: A total of 28 patients were included: 6 had indolent SM, 9 had aggressive SM, and 13 had SM with an associated hematologic neoplasm. Twenty-five (89%) patients presented hepatomegaly, and 19 (68%) had portal hypertension. The LB frequently showed slight sinusoid dilatation (82%). Fibrosis was observed in 3/6 indolent SM and in almost all advanced SM cases (21/22), but none of them showed cirrhosis. A high MC burden (>50 MCs/high-power field) was correlated with elevated blood alkaline phosphatase levels (p = .030). The presence of portal hypertension was associated with a higher mean fibrosis grade (1.6 vs. 0.8 in its absence; p = .026). In advanced SM, the presence of nodular regenerative hyperplasia (NRH) was associated with decreased overall survival (9.5 vs. 46.3 months, p = .002). Conclusions: MC infiltration induced polymorphic hepatic lesions and the degree of fibrosis is associated with portal hypertension. NRH identifies a poor prognosis subgroup of patients with advanced SM. Assessing liver histology can aid in SM prognostic evaluation.
Language	English
Publishing date	2024-03-30
Publishing country	United States
Document type	Journal Article
ZDB-ID	2102783-3
ISSN	1478-3231 ; 1478-3223
ISSN (online)	1478-3231
ISSN	1478-3223
DOI	10.1111/liv.15913
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Article: Tips and Tricks and Clinical Outcome of Cryopreserved Human Amniotic Membrane Application for the Management of Medication-Related Osteonecrosis of the Jaw (MRONJ): A Pilot Study.

Odet, Stéphane / Meyer, Christophe / Gaudet, Camille / Weber, Elise / Quenot, Julie / Derruau, Stéphane / Laurence, Sebastien / Bompy, Lisa / Girodon, Marine / Chatelain, Brice / Mauprivez, Cédric / Brenet, Esteban / Kerdjoudj, Halima / Zwetyenga, Narcisse / Marchetti, Philippe / Hatzfeld, Anne-Sophie / Toubeau, David / Pouthier, Fabienne / Lafarge, Xavier /

Redl, Heinz / Fenelon, Mathilde / Fricain, Jean-Christophe / Di Pietro, Roberta / Ledouble, Charlotte / Gualdi, Thomas / Parmentier, Anne-Laure / Louvrier, Aurélien / Gindraux, Florelle

Frontiers in bioengineering and biotechnology

2022 Volume 10, Page(s) 936074

Abstract: Medication-related osteonecrosis of the jaw (MRONJ) is a complication of certain pharmacological treatments such as bisphosphonates, denosumab, and angiogenesis inhibitors. There are currently no guidelines on its management, particularly in advanced ... ...

Abstract	Medication-related osteonecrosis of the jaw (MRONJ) is a complication of certain pharmacological treatments such as bisphosphonates, denosumab, and angiogenesis inhibitors. There are currently no guidelines on its management, particularly in advanced stages. The human amniotic membrane (hAM) has low immunogenicity and exerts anti-inflammatory, antifibrotic, antimicrobial, antiviral, and analgesic effects. It is a source of stem cells and growth factors promoting tissue regeneration. hAM acts as an anatomical barrier with suitable mechanical properties (permeability, stability, elasticity, flexibility, and resorbability) to prevent the proliferation of fibrous tissue and promote early neovascularization at the surgical site. In oral surgery, hAM stimulates healing and facilitates the proliferation and differentiation of epithelial cells in the oral mucosa and therefore its regeneration. We proposed using cryopreserved hAM to eight patients suffering from cancer (11 lesions) with stage 2-3 MRONJ on a compassionate use basis. A collagen sponge was added in some cases to facilitate hAM grafting. One or three hAMs were applied and one patient had a reapplication. Three patients had complete closure of the surgical site with proper epithelialization at 2 weeks, and two of them maintained it until the last follow-up. At 1 week after surgery, three patients had partial wound dehiscence with partial healing 3 months later and two patients had complete wound dehiscence. hAM reapplication led to complete healing. All patients remained asymptomatic with excellent immediate significant pain relief, no infections, and a truly positive impact on the patients' quality of life. No adverse events occurred. At 6 months of follow-up, 80% of lesions had complete or partial wound healing (30 and 50%, respectively), while 62.5% of patients were in stage 3. Radiological evaluations found that 85.7% of patients had stable bone lesions (
Language	English
Publishing date	2022-07-22
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2719493-0
ISSN	2296-4185
ISSN	2296-4185
DOI	10.3389/fbioe.2022.936074
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Article ; Online: Pathophysiologic implications of elevated prevalence of hereditary alpha-tryptasemia in all mastocytosis subtypes.

Polivka, Laura / Madrange, Marine / Bulai-Livideanu, Cristina / Barete, Stéphane / Ballul, Thomas / Neuraz, Antoine / Greco, Celine / Agopian, Julie / Brenet, Fabienne / Dubreuil, Patrice / Burdet, Charles / Lemal, Richard / Tournilhac, Olivier / Terriou, Louis / Launay, David / Bouillet, Laurence / Gourguechon, Clément / Damaj, Ghandi / Frenzel, Laurent /

Meni, Cécile / Bouktit, Hassiba / Collange, Anne Florence / Gaudy-Marqueste, Caroline / Gousseff, Marie / Le Mouel, Edwige / Hamidou, Mohamed / Neel, Antoine / Ranta, Dana / Jaussaud, Roland / Guilpain, Philippe / Canioni, Danielle / Molina, Thierry Jo / Bruneau, Julie / Lhermitte, Ludovic / Garcelon, Nicolas / Javier, Rose-Marie / Pelletier, Fabien / Castelain, Florence / Retornaz, Frederique / Cabrera, Quentin / Zunic, Patricia / Gourin, Marie Pierre / Wierzbicka-Hainaut, Ewa / Viallard, Jean François / Lavigne, Christian / Hoarau, Cyrille / Durieu, Isabelle / Heiblig, Maël / Dimicoli-Salazar, Sophie / Torregrosa-Diaz, Jose Miguel / Soria, Angèle / Arock, Michel / Lortholary, Olivier / Bodemer, Christine / Hermine, Olivier / Rossignol, Julien

The Journal of allergy and clinical immunology

2023 Volume 153, Issue 1, Page(s) 349–353.e4

Abstract: Background: Mastocytosis and monoclonal mast cell (MC) activation syndrome (MMAS) are heterogeneous conditions characterized by the accumulation of atypical MCs. Despite the recurrent involvement of KIT mutations, the pathophysiologic origin of ... ...

Abstract	Background: Mastocytosis and monoclonal mast cell (MC) activation syndrome (MMAS) are heterogeneous conditions characterized by the accumulation of atypical MCs. Despite the recurrent involvement of KIT mutations, the pathophysiologic origin of mastocytosis and MMAS is unclear. Although hereditary α-tryptasemia (HαT, related to TPSAB1 gene duplication) is abnormally frequent in these diseases, it is not known whether the association is coincidental or causal. Objective: We evaluated the prevalence of HαT in all mastocytosis subtypes and MMAS and assessed the pathophysiologic association with HαT. Methods: Clinical data, laboratory data, KIT mutations, TPSAB1 duplication (assessed by droplet digital PCR), and HαT prevalence were retrospectively recorded for all patients with mastocytosis and MMAS registered in the French national referral center database and compared to a control cohort. To increase the power of our analysis for advanced systemic mastocytosis (advSM), we pooled our cohort with literature cases. Results: We included 583 patients (27 with MMAS and 556 with mastocytosis). The prevalence of HαT in mastocytosis was 12.6%, significantly higher than in the general population (5.7%, P = .002) and lower than in MMAS (33.3%, P = .02). HαT Conclusion: Here we confirm the increase incidence of anaphylaxis in HαT
MeSH term(s)	Humans ; Mastocytosis, Systemic/epidemiology ; Mastocytosis, Systemic/genetics ; Mastocytosis, Systemic/pathology ; Retrospective Studies ; Prevalence ; Mastocytosis/epidemiology ; Mastocytosis/genetics ; Mastocytosis/pathology ; Anaphylaxis/pathology ; Mast Cells/pathology ; Tryptases/genetics
Chemical Substances	Tryptases (EC 3.4.21.59)
Language	English
Publishing date	2023-08-24
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	121011-7
ISSN	1097-6825 ; 1085-8725 ; 0091-6749
ISSN (online)	1097-6825 ; 1085-8725
ISSN	0091-6749
DOI	10.1016/j.jaci.2023.08.015
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