2,994 results match your criteria: "Mastocytosis Systemic"
Management of Advanced Systemic Mastocytosis: Clinical Challenges.
J Blood Med
September 2024
Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
Article Synopsis
- - Advanced systemic mastocytosis (AdvSM) is a rare blood cancer characterized by harmful accumulation of abnormal mast cells in various organs, leading to organ damage and decreased life expectancy.
- - The identification of the D816V mutation has spurred the development of targeted therapies like avapritinib, which have improved patients' quality of life but still face management challenges.
- - This review discusses the future of AdvSM treatments, including new KIT inhibitors like elenestinib and bezuclastinib, as well as the role of stem cell transplantation and ongoing clinical management issues.
The challenging differential diagnosis of recurrent flushing episodes: systemic mastocytosis mimicking carcinoid syndrome.
Endokrynol Pol
September 2024
Department of Endocrinology and Radioisotope Therapy, Military Institute of Medicine - National Research Institute, Warsaw, Poland.
Article Synopsis
- The text indicates that a Clinical Vignette is not obligatory for submission or consideration.
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- Understanding of this requirement could help streamline the preparation process for students or professionals in the field.
Unraveling wasp sensitization in a patient with systemic mastocytosis by CAP-inhibition assay.
Allergol Immunopathol (Madr)
September 2024
Allergy Department, Hospital Universitari Vall d´Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
Article Synopsis
- Systemic mastocytosis (SM) is a disorder that can cause severe allergic reactions, especially triggered by insect stings, and diagnosing indolent SM without skin symptoms is not uncommon.* -
- Venom immunotherapy (VIT) effectively reduces the risk of future reactions in patients with indolent SM, and it's crucial to personalize this treatment by distinguishing between true venom allergies and cross-reactivity.* -
- In a case study of a man who experienced anaphylaxis after wasp stings, molecular diagnosis did not clarify his allergies, so a CAP-inhibition assay was necessary, emphasizing the need for precise assessments in hymenoptera venom allergy.*
Clustering of clinical symptoms using large language models reveals low diagnostic specificity of proposed alternatives to consensus mast cell activation syndrome criteria.
J Allergy Clin Immunol
September 2024
Institute for Immunity, Transplantation, and Infection, School of Medicine, Stanford University, Palo Alto, Calif; Department of Medicine, Center for Biomedical Informatics Research, School of Medicine, Stanford University, Palo Alto, Calif.
Article Synopsis
- - The diagnosis of mast cell activation syndrome (MCAS) has risen, but while there are established consortium criteria, there are also alternative criteria that may not be as specific.
- - Researchers used advanced language models to analyze diagnostic probabilities and found that alternative criteria led to broader and less precise diagnoses compared to the consortium criteria.
- - The study concluded that alternative MCAS criteria result in a diverse range of diagnoses, making them less reliable and potentially leading to misclassifications when compared to established conditions like systemic lupus erythematosus.
Solitary Cutaneous Mastocytoma in an Adult Diagnosed on Cytology: A Rare Case Report.
Diagn Cytopathol
January 2025
Department of Dermatology, University College of Medical Sciences and Guru Teg Bahadur Hospital, Delhi University, New Delhi, Delhi, India.
Article Synopsis
- Mastocytosis is a condition where there is an abnormal increase of mast cells in various organs, presenting either on the skin or systemically.
- A case study of a 19-year-old female highlighted a solitary cutaneous mastocytoma, identified by a reddish-brown nodule in her supraclavicular area that was sampled for analysis.
- Fine needle aspiration cytology (FNAC) revealed characteristic cells and granules, leading to a diagnosis of cutaneous mastocytoma, showing FNAC's effectiveness in diagnosing mast cell tumors without needing a tissue biopsy in some situations.
MASTering systemic mastocytosis: Lessons learned from a large patient cohort.
J Allergy Clin Immunol Glob
November 2024
Department of Allergy, Kaiser Permanente Medical Center, San Diego, Calif.
Article Synopsis
- Systemic mastocytosis (SM) is a rare disease affecting about 32,000 people in the U.S., often misdiagnosed due to vague symptoms and the necessity for invasive tests.
- A study of 116 patients revealed that the majority had indolent SM, with a significant average delay of 58.3 months for diagnosis, and some patients progressed to more advanced forms.
- The findings emphasize the need for better awareness and understanding of SM to improve diagnosis and treatment, suggesting that further studies are necessary to establish effective follow-up care for patients.
Systemic mastocytosis and its alliance with myelodysplastic syndrome.
Indian J Pathol Microbiol
September 2024
Department of Pathology and Lab Medicine, Medanta-The Medicity, Gurugram, Haryana, India.
Characteristics and outcomes associated with CD2 and CD25 expression on bone marrow mast cells in patients with systemic mastocytosis.
Haematologica
August 2024
Université Paris Cité, INSERM UMR-S1151, CNRS UMR-S8253, Institut Necker Enfants Malades, F-75015 Paris, France; Hôpital Necker Enfants-Malades, Laboratoire d'Onco-Hématologie, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris.
Article Synopsis
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Harmonization of Diagnostic Criteria in Mastocytosis for Use in Clinical Practice: WHO vs ICC vs AIM/ECNM.
J Allergy Clin Immunol Pract
December 2024
Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, Mich.
Article Synopsis
- - Mastocytosis is a disorder characterized by an abnormal increase of mast cells in one or more organs, with varying symptoms and outcomes; it's classified into cutaneous, systemic, and MC sarcoma types by the WHO.
- - The disease often involves a specific mutation (D816V) in most systemic mastocytosis cases, affecting life expectancy significantly based on the type, with those having nonadvanced forms generally living near-normal lifespans, while advanced forms have limited life expectancies.
- - Recently, a group of experts proposed updated diagnostic criteria and classifications for mastocytosis, aiming to unify different classifications from previous organizations to improve research and clinical comparisons.
Diagnostic measures in patients with severe insect sting reactions and elevated baseline serum tryptase levels.
Allergol Select
August 2024
Department of Dermatology and Allergy, LMU University Hospital, Munich, Germany.
Article Synopsis
- * All patients had elevated bST levels (ranging from 15.5 to 23.2 µg/L) but no evidence of mastocytosis in the skin, leading to further tests that identified hereditary α-hypertryptasemia in 2 patients and a D816V mutation in 1 patient.
- * The findings highlight the importance of investigating elevated bST levels through minimally invasive tests, as they can reveal underlying conditions and inform treatment decisions for patients with insect venom allergies.
Systemic Mastocytosis Successfully Managed using Cytosorb ® During Cardiopulmonary Bypass for Aortic Valve Replacement.
Ann Card Anaesth
October 2024
Department of Cardiac Surgery, ICH, Valais Hospital, Sion, Switzerland.
Article Synopsis
- A 72-year-old male with systemic mastocytosis underwent aortic valve replacement to address severe aortic insufficiency.
- The anesthesia and peri-operative care focused on avoiding histamine-releasing drugs and using prophylactic medications like methylprednisolone and clemastin.
- A CytoSorb ® cartridge was used in the bypass circuit to perform hemoadsorption during cardiopulmonary bypass, leading to a successful procedure with no adverse events or allergic reactions reported.
Systemic Mastocytosis: State of the Art.
Curr Hematol Malig Rep
October 2024
Department of Haematology, Guy's and St Thomas' NHS Foundation Trust, London, SE1 7EH, UK.
Article Synopsis
- The review discusses the recent advancements in treating Systemic Mastocytosis (SM), emphasizing the significance of targeting the C-KIT D816V mutation as identified by the WHO.
- New therapeutic options like Avapritinib and other potent KIT tyrosine kinase inhibitors (TKIs) have shown promise in achieving long-term remissions and improving patient outcomes with minimal side effects.
- The review also highlights the role of molecular profiling in tailoring combination therapies for SM, especially when associated with other hematologic conditions, and presents clinical cases to illustrate these treatment strategies.
Mast Cell Disorders and Hymenoptera Venom-Triggered Anaphylaxis: Evaluation and Management.
J Allergy Clin Immunol Pract
August 2024
Department of Allergy, Hospital Ramón y Cajal, IRYCIS, Madrid, Spain; Spanish Network on Mastocytosis (REMA), Toledo and Salamanca, Salamanca, Spain.
Article Synopsis
- * Screening includes examining the skin, measuring baseline serum tryptase, and using specific scoring systems, with a bone marrow biopsy recommended for higher-risk patients.
- * Treatment for those with both conditions involves long-term immunotherapy for the venom and carrying multiple epinephrine autoinjectors, while future research aims to explore targeted therapies for related anaphylactic reactions.
Risk Factors for Severe Sting Reactions and Side Effects During Venom Immunotherapy.
J Allergy Clin Immunol Pract
August 2024
University Clinic of Respiratory and Allergic Diseases, Golnik, Slovenia; Medical Faculty, University of Ljubljana, Ljubljana, Slovenia.
Article Synopsis
- Understanding what makes some people have serious allergic reactions to stings is important for helping them and their families.
- Some known factors that increase the risk include certain medical conditions, age, and how quickly symptoms appear after a sting.
- Treatment called venom immunotherapy (VIT) can help prevent these reactions, but it may have some side effects, especially in people getting treated for bee stings.
SOHO State of the Art Update and Next Questions: Current and Emerging Therapies for Systemic Mastocytosis.
Clin Lymphoma Myeloma Leuk
June 2024
Department of Leukemia, University of Texas MD Anderson Cancer Center, Houston, TX. Electronic address:
Article Synopsis
- Systemic mastocytosis (SM) is a type of blood cancer characterized by the excessive growth of mast cells in organs like the bone marrow and gastrointestinal tract, primarily caused by a mutation in the KIT gene found in most patients.
- The most common subtype is indolent SM, which can have severe symptoms, while advanced SM has a poor prognosis; recent treatments like KIT inhibitors (e.g., Midostaurin and Avapritinib) have significantly improved patient outcomes and quality of life.
- Investigational drugs such as bezuclastinib and elenestinib are also showing promise in reducing symptoms and mast cell burden, with high effectiveness in various patient groups, particularly
Beyond the Usual Suspects: Unraveling Spleen Mastocytosis in Hypersplenism Differential Diagnosis.
Cureus
August 2024
Hematology, Unidade Local de Saúde de Santo António, Porto, PRT.
Systemic mastocytosis (SM) poses a diagnostic challenge. This hematologic disorder involves abnormal mast cell proliferation and concurrent tissue infiltration. SM clinical presentation is not uniform, with patients displaying a wide array of symptoms related to different organ infiltration and mast cell mediators.
View Article and Find Full Text PDFAggressive systemic mastocytosis (ASM) is an advanced subtype of systemic mastocytosis characterized by organ involvement. In this article, we report a case with ASM in a 54-year-old woman with characteristic findings on computed tomography (CT) and fluorine-18-fluorodeoxyglucose positron emission tomography (F-FDG PET)/CT. Contrast-enhanced CT on admission revealed hepatosplenomegaly, generalized osteosclerosis, colonic edema, edematous thickening of the wall in the ascending colon and edema in the surrounding regions of these organs and mesentery, ileus, subcutaneous edema, periportal collar sign, and multiple mesenteric lymphadenopathies.
View Article and Find Full Text PDFInteractions between eosinophils and IL-5Rα-positive mast cells in nonadvanced systemic mastocytosis.
J Allergy Clin Immunol
December 2024
University of Paris, Institut Imagine, INSERM, Paris, France; French Reference Center for Mastocytosis (CEREMAST), Hôpital Necker-Enfants Malades, AP-HP, Paris, France; Department of Hematology, Hôpital Necker-Enfants Malades, AP-HP, Paris, France. Electronic address:
Background: Bidirectional interactions between eosinophils and mast cells (MCs) have been reported in various allergic diseases. Bone marrow (BM) eosinophilia, and to a lesser extent blood eosinophilia, is common in systemic mastocytosis (SM), but its significance remains unknown.
Objective: We described blood and BM eosinophil characteristics in SM.
Increased expression of formyl peptide receptor-1 by basophils from patients with mastocytosis.
J Allergy Clin Immunol Glob
November 2024
Mast Cell Biology Section, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md.
Background: Symptoms in patients with systemic mastocytosis (SM) are associated with an increase in mast cell burden and release of mast cell-derived mediators. The most frequent presentation of SM is indolent SM (ISM), with moderate symptoms and prognosis. Basophil numbers in these patients are generally normal.
View Article and Find Full Text PDFEffect of midostaurin on the pharmacokinetics of P-gp, BCRP, and CYP2D6 substrates: assessing potential drug-drug interactions in healthy participants : Brief title: Drug-drug interaction of midostaurin.
Cancer Chemother Pharmacol
October 2024
Novartis Pharma AG, Basel, CH-4002, Switzerland.
Purpose: Midostaurin, approved for FLT3-mutated acute myeloid leukemia and advanced systemic mastocytosis, is mainly metabolized by cytochrome P450 (CYP) 3A4. Midostaurin exhibited potential inhibitory effects on P-glycoprotein (P-gp), breast cancer resistance protein (BCRP), organic anion-transporting polyprotein 1B1, and CYP2D6 in in vitro studies. This study investigated the pharmacokinetic (PK) effects of midostaurin on P-gp (digoxin), BCRP (rosuvastatin) and CYP2D6 (dextromethorphan) substrates in healthy adults.
View Article and Find Full Text PDFClinical and biological characteristics associated with bronchial or pulmonary abnormalities on chest CT imaging in patients with systemic mastocytosis.
Clin Transl Allergy
August 2024
Department of Respiratory and Allergic Diseases, Toulouse University Hospital Center, Toulouse, France.
Utility of KIT Mutations in Myeloid Neoplasms Without Documented Systemic Mastocytosis to Detect Hidden Mast Cells in Bone Marrow.
Clin Lymphoma Myeloma Leuk
November 2024
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX. Electronic address:
Background: KIT p.D816 mutation is strongly associated with systemic mastocytosis (SM). Next-generation sequencing (NGS) is now routinely performed in almost all bone marrow sample and KIT mutations are detected from patients who are not known or suspected to have SM.
View Article and Find Full Text PDFSubcellular expression of CD30 in cutaneous mastocytosis-An important factor for targeted treatment.
J Cutan Pathol
November 2024
Kempf und Pfaltz Histologische Diagnostik, Zürich, Switzerland.
Background: The subcellular distribution of CD30 on mast cells and the presence of eosinophils in cutaneous mastocytosis require further investigation, especially as the cell surface expression of CD30 is critical for the therapeutic response of systemic mastocytosis to brentuximab vedotin.
Objective: Investigation of 147 biopsy specimens from 143 patients with cutaneous mastocytosis for mast cell density and distribution, frequency of CD30 expression, CD30 staining patterns, and presence and distribution of eosinophils. Correlation with clinical patterns.
Prevalence and impact of the M541L variant in patients with mastocytosis.
Oncotarget
July 2024
Mast Cell Biology Section, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA.
Article Synopsis
- The study investigates the prevalence of the M541L mutation in pediatric and adult patients with mastocytosis, including comparisons to controls and patients with idiopathic anaphylaxis.
- The M541L variant was found in 19 individuals, mostly linked to systemic mastocytosis and often associated with the D816V mutation.
- No significant differences in symptoms or blood parameters were observed between patients with and without the M541L variant, emphasizing its limited impact on clinical outcomes in mastocytosis.