2,994 results match your criteria: "Mastocytosis Systemic"
Hymenoptera venom allergy in children.
Ital J Pediatr
December 2024
Department of Health Sciences, University of Florence, Florence, 50139, Italy.
From a taxonomic point of view, Hymenoptera are subclassified into families: Apidae, including honeybees (Apis mellifera) and bumblebees (Bombus), and Vespidae, which, in turn, are divided into the subfamilies of Vespinae (wasps, including hornets, vespules, dolichovespules) and Polistinae (paper wasp). Hypersensitivity to Hymenoptera venom can be linked to immunological (IgE-mediated or non-IgE-mediated) and non-immunological mechanisms. Reactions are classified into local reactions, large local reactions, systemic reactions, toxic reactions, and unusual reactions.
View Article and Find Full Text PDF[Endoscopy for the diagnosis of mast cell leukemia with refractory diarrhea: a case report].
Nihon Shokakibyo Gakkai Zasshi
December 2024
Department of Gastroenterology, Fukushima Medical University School of Medicine.
Already a rare presentation in patients with systemic mastocytosis (SM), chronic diarrhea is even rarer in those with mast cell leukemia (MCL), a subtype of SM. We present a case to illustrate the utility of lower gastrointestinal endoscopy with histological examination in the diagnosis of MCL in a patient with chronic diarrhea. A woman in her 70s presented with persistent diarrhea.
View Article and Find Full Text PDFKIT V560D-Mutated Systemic Mastocytosis Associated With High-Risk Myelodysplastic Syndrome: A Unique Case of Systemic Mastocytosis-Associated Hematologic Neoplasm.
Case Rep Hematol
November 2024
Department of Hematology-Oncology, Medical University of South Carolina, Charleston, South Carolina, USA.
Systemic mastocytosis (SM) is a rare hematologic disorder characterized by clonal proliferation of mast cells in the bone marrow and/or other organs. SM-associated hematologic neoplasm (SM-AHN) is one of the advanced SM variants that usually confer a poor prognosis. We present a case of a 75-year-old female patient with SM-AHN, specifically myelodysplastic syndrome (MDS), that harbored a unique KIT mutation KIT V560D, not previously described in the literature in this setting.
View Article and Find Full Text PDFMast cell sarcoma with KIT p.D816V mutation and concurrent systemic mastocytosis.
J Hematop
December 2024
Department of Pathology, University of Colorado Anschutz Medical Campus, Aurora, USA.
Article Synopsis
Recurrent prosthetic mitral valve thrombosis: First report of an unusual feature of systemic mastocytosis, a case report and review of the literature.
Caspian J Intern Med
October 2024
Department of Dermatopathology, Raazi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Article Synopsis
Ocular and palpebral manifestations of mastocytosis: A prospective single-center study.
Ann Dermatol Venereol
November 2024
Departement of Dermatology, Hôpital Privé Francheville, Périgueux, France. Electronic address:
Article Synopsis
Osteoporosis in Systemic Mastocytosis: A Scoping Review.
Medicina (Kaunas)
October 2024
Precision Medicine in the Medical, Surgical and Critical Care Areas, University of Palermo, 90100 Palermo, Italy.
Article Synopsis
Thromboelastography for rapid diagnosis of heparin-like anticoagulant release during anaphylaxis-induced coagulopathy in systemic mastocytosis: a case report.
Clin Med (Lond)
November 2024
King Abdullah International Medical Research Center, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia; Department of Medicine King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia.
Article Synopsis
Sensitive and reliable detection of KIT p.D816V mutation in decalcified archival bone marrow trephines.
Virchows Arch
November 2024
Institute of Pathology, Medical School Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.
Article Synopsis
Demographics, Types of Patient-Reported Allergic Diseases, and Anaphylaxis in Mastocytosis: A Single-Center US Experience.
J Allergy Clin Immunol Pract
November 2024
Department of Internal Medicine, Allergy and Clinical Immunology, University of Michigan, Ann Arbor, Mich. Electronic address:
Article Synopsis
Quantifying Mast Cell and Eosinophil Cellular Density in Skin Biopsy Tissue From Adults With Maculopapular Cutaneous Mastocytosis as Compared With Urticaria and Normal Skin: A Retrospective Histopathologic Study.
Am J Dermatopathol
November 2024
Department of Dermatology, Mayo Clinic, Rochester, MN.
Article Synopsis
Case Report: Multidisciplinary management of a patient with indolent systemic mastocytosis and refractory symptoms.
Front Allergy
October 2024
Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, United States.
Article Synopsis
Patient-Reported Burden of Indolent Systemic Mastocytosis in a Managed Care Organization.
J Allergy Clin Immunol Pract
October 2024
Department of Research and Evaluation, Kaiser Permanente Southern California, Pasadena, Calif.
Article Synopsis
- Indolent systemic mastocytosis (ISM) is the most common form of systemic mastocytosis, and its diagnostic challenges and impact on patients' lives need more examination.
- A survey of 40 adults with ISM revealed a lengthy diagnosis process, averaging over two years, with many patients experiencing severe symptoms that negatively affected their quality of life and work ability.
- The study found a strong correlation between symptom severity and physical/mental health scores, highlighting that those with moderate to severe symptoms suffered significantly more from skin and gastrointestinal issues compared to those with mild symptoms.
Detection of Mutations in Systemic Mastocytosis: How, When, and Why.
Int J Mol Sci
October 2024
Department of Clinical and Biological Sciences, University of Turin, Mauriziano Hospital, 10128 Turin, Italy.
Article Synopsis
- * The most common mutation is p.D816V, but there are other less frequent mutations that are typically undetectable with standard testing methods.
- * New, more sensitive detection methods are being developed, and the review discusses how to approach these tests for diagnosing and monitoring mastocytosis patients.
Diarrhea-predominant irritable bowel syndrome as a masquerade for systemic mastocytosis: review article and illustrating case report.
Arch Med Sci
April 2024
Pathology Department, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Can molecular patterns help to classify overlapping entities in myeloid neoplasms?
Histopathology
January 2025
Department of Pathology, Microbiology, and Immunology, University of Nebraska Medical Center, Omaha, USA.
Article Synopsis
Altered B-cell, plasma cell, and antibody immune profiles in blood of patients with systemic mastocytosis.
J Allergy Clin Immunol
October 2024
Cancer Research Center, Department of Medicine and Cytometry Service, University of Salamanca, Salamanca, Spain; Biomedical Research Institute of Salamanca, Salamanca, Spain; Biomedical Research Networking Center Consortium, Madrid, Spain; Spanish Network on Mastocytosis, Toledo and Salamanca, Spain. Electronic address:
Article Synopsis
- Systemic mastocytosis (SM) is a complex disease linked to abnormal mast cells that release mediators affecting the immune environment.
- The study aimed to explore the blood profiles of B-cells, plasma cells, and antibody types in 108 SM patients compared to healthy individuals.
- Results showed increased immature B-cells and elevated IgM and IgD levels in SM patients, alongside decreased plasma cells across various IgH types, with unique immune patterns based on the SM subtype.
Clonal dynamics of aggressive systemic mastocytosis on avapritinib therapy.
Blood Cancer J
October 2024
Versiti Blood Research Institute, Milwaukee, WI, USA.
[Report of six cases with mast cell leukemia and a literature review].
Zhonghua Nei Ke Za Zhi
October 2024
Jiangsu Institute of Hematology, National Clinical Research Center for Hematologic Diseases, the First Affiliated Hospital of Soochow University, Suzhou 215006, China.
Article Synopsis
- A retrospective analysis of six patients with acute mast cell leukemia (MCL) at Soochow University revealed a median age of 54, with various clinical presentations such as hypoalbuminemia, fatigue, and fever, highlighting the complexity of this rare disease.
- Most patients displayed significant abnormalities in specific proteins (CD117, CD30, CD25, CD2) and gene mutations, indicating aggressive disease characteristics and a need for better diagnostic criteria.
- Treatment outcomes were poor, with some patients receiving various therapies like venetoclax and cladribine, but only one achieved partial remission; this emphasizes the urgent need for improved understanding and treatment approaches for MCL.
Indolent systemic mastocytosis (ISM) without skin lesions as a recurrent anaphylaxis: a case report study.
Ann Med Surg (Lond)
October 2024
Neonatal and Children's Health Research Center, Golestan University of Medical Sciences, Gorgan, Iran.
Article Synopsis
- * A 32-year-old male patient experienced recurrent severe allergic reactions (anaphylaxis) and high levels of a substance called tryptase, which indicates mast cell issues, despite not having visible skin symptoms.
- * The condition is rare but can be serious and presents with unusual signs, highlighting the need for better diagnostic methods and treatment options to improve understanding and patient outcomes.
Tryptase: The Silent Witness of Past and Ongoing Systemic Events.
Medicina (Kaunas)
August 2024
Clinic of Allergology and Immunology, University Clinical Centre of Serbia, 11000 Belgrade, Serbia.
Article Synopsis
- Tryptase is a key biomarker for diagnosing severe allergic reactions like anaphylaxis and is also important in assessing other mast cell-related conditions.
- The paper highlights two clinical cases to demonstrate the significance of timely measuring serum tryptase during hypersensitivity reactions and emphasizes its broader clinical uses.
- A detailed literature review identified crucial guidelines for interpreting serum tryptase levels, suggesting that elevated levels indicate a higher risk for severe reactions and necessitate further investigation and management.
Comparison of prognostic scores according to WHO classification in 170 patients with advanced mastocytosis and C-finding treated with midostaurin.
Am J Hematol
November 2024
French Reference Center for Mastocytosis (CEREMAST), Necker-Enfants Malades University Hospital, APHP, Paris, France.
Article Synopsis
- Advanced systemic mastocytosis (AdvSM) is a complex condition linked to poor outcomes, and while midostaurin is the first approved treatment, its long-lasting effectiveness is limited.
- Various prognostic scoring systems like MARS, IPSM, and GPSM have been developed to assess patients' outcomes, but it's essential to tailor these scores to specific AdvSM subtypes for better accuracy.
- A study of patients treated with midostaurin revealed that MARS and AdvSM subtype significantly predict overall survival, identifying five distinct patient subgroups with varying median survival times, highlighting the need for personalized management strategies.