7,397 results match your criteria Mastocytosis


Relevance of the endoscopic evaluation in the diagnosis of Bladder Pain Syndrome/Interstitial Cystitis.

Urology 2020 Jun 30. Epub 2020 Jun 30.

Urology Clinic, University of Udine and University Hospital "Santa Maria della Misericordia" - Udine - Italy.

Objective: In 2008 the European Society for the Study of Interstitial Cystitis (ESSIC) established the diagnostic criteria and classification of Bladder Pain Syndrome/Interstitial Cystitis (BPS/IC), based on clinical features and cystoscopy results. The present study aims to assess the relevance of the endoscopic evaluation in clinically suspected cases of BPS/IC, using ESSIC criteria.

Methods: . Read More

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http://dx.doi.org/10.1016/j.urology.2020.06.032DOI Listing

No evidence of vertical transmission of SARS-CoV-2 after induction of labour in an immune-suppressed SARS-CoV-2-positive patient.

BMJ Case Rep 2020 Jun 30;13(6). Epub 2020 Jun 30.

Department of Obstetrics and Gynaecology, Erasmus Medical Center, Rotterdam, Zuid-Holland, The Netherlands

We present a case of a 38+1 weeks pregnant patient (G1P0) with a proven COVID-19 infection, who was planned for induction of labour because of pre-existent hypertension, systemic lupus erythematosus, respiratory problem of coughing and mild dyspnoea without fever during the COVID-19 pandemic in March 2020. To estimate the risk of vertical transmission of Severe Acute Respiratory Syndrome CoronaVirus 2 (SARS-CoV-2) during labour and delivery, we collected oropharyngeal, vaginal, urinary, placental and neonatal PCRs for SARS-CoV-2 during the period of admission. All PCRs, except for the oropharyngeal, were negative and vertical transmission was not observed. Read More

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http://dx.doi.org/10.1136/bcr-2020-235581DOI Listing

Ripretinib: First Approval.

Authors:
Sohita Dhillon

Drugs 2020 Jun 23. Epub 2020 Jun 23.

Springer Nature, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

Ripretinib (QINLOCK™) is a novel type II tyrosine switch control inhibitor being developed by Deciphera Pharmaceuticals for the treatment of KIT proto-oncogene receptor tyrosine kinase (KIT)-driven and/or platelet derived growth factor receptor A (PDGFRA)-driven cancers, including gastrointestinal stromal tumour (GIST). Ripretinib inhibits KIT and PDGFRA kinase, including wild-type, primary and secondary mutations, as well as other kinases, such as PDGFRB, TIE2, VEGFR2 and BRAF. In May 2020, oral ripretinib received its first approval in the USA for the treatment of adult patients with advanced GIST who have received prior treatment with ≥ 3 kinase inhibitors, including imatinib. Read More

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http://dx.doi.org/10.1007/s40265-020-01348-2DOI Listing

Top-Down Proteomics of Human Saliva Discloses Significant Variations of the Protein Profile in Patients with Mastocytosis.

J Proteome Res 2020 Jul 6. Epub 2020 Jul 6.

Dipartimento di Scienze della Vita e dell'Ambiente, Università di Cagliari, 09124 Cagliari, Italy.

Mastocytosis is a myeloproliferative neoplasm causing abnormal clonal mast cell accumulation in different tissues, such as skin and bone marrow. A cutaneous subtype (CM) is distinguished from a systemic one (SM); SM patients can be grouped into SM with (SM+C) or without (SM-C) additional cutaneous lesions, and their classification is often challenging. This study was purposed to highlight variations in the salivary proteome of patients with different mastocytosis subtypes and compared to healthy controls. Read More

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http://dx.doi.org/10.1021/acs.jproteome.0c00207DOI Listing

Evolution of clonal dynamics and differential response to targeted therapy in a case of systemic mastocytosis with associated myelodysplastic syndrome.

Leuk Res 2020 Jun 11;95:106404. Epub 2020 Jun 11.

Division of Hematology, Oncology and Transplantation, Department of Medicine, University of Minnesota, Minneapolis, MN, United States; Masonic Cancer Center, University of Minnesota, Minneapolis, MN, United States. Electronic address:

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http://dx.doi.org/10.1016/j.leukres.2020.106404DOI Listing

A New Digital Method for Counting Mast Cells in Cutaneous Specific Lesions of Mastocytosis: A Series of Adult Cases of Mastocytosis With Clinical-Pathological Correlations.

Am J Dermatopathol 2020 Jun 17. Epub 2020 Jun 17.

Service d'Anatomie et Cytologie Pathologiques, Hôpital Necker-Enfants Malades, APHP, Paris, France.

Cutaneous mastocytosis is characterized by the abnormal accumulation of mast cells in the skin. However, mast cell counting is not always easy and reproducible with classical methods. This work aims to demonstrate the reliability, usability, and virtues of a new software used on digital tablets for counting mast cells in cutaneous specific lesions of mastocytosis, to assess differences in mast cell counts between clinical subtypes of mastocytosis in the skin, and to consider the feasibility of applying a diagnostic mast cell count cutoff to urticaria pigmentosa, which is the most frequent form of cutaneous mastocytosis. Read More

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http://dx.doi.org/10.1097/DAD.0000000000001705DOI Listing

Risk and Management of Patients with Mastocytosis and MCAS in the SARS-CoV-2 (COVID-19) Pandemic: Expert Opinions.

J Allergy Clin Immunol 2020 Jun 16. Epub 2020 Jun 16.

Division of Allergy, Department of Dermatology,and Department of Biomedicine, University of Basel, Basel Switzerland.

The COVID-19 (SARS-CoV-2) pandemic has massively distorted our health care systems and caused catastrophic consequences in our affected communities. The number of victims continues to increase and patients at risk can only be protected to a degree, since the virulent state may be asymptomatic. Risk factors concerning COVID-19-induced morbidity and mortality include advanced age, an impaired immune system, cardiovascular or pulmonary diseases, obesity, diabetes mellitus, and cancer treated with chemotherapy. Read More

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http://dx.doi.org/10.1016/j.jaci.2020.06.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7297685PMC

Role of Rutin in 5-Fluorouracil-Induced Intestinal Mucositis: Prevention of Histological Damage and Reduction of Inflammation and Oxidative Stress.

Molecules 2020 Jun 17;25(12). Epub 2020 Jun 17.

Department of Morphology, Faculty of Medicine, Federal University of Ceará, s/n Delmiro of Farias Street, Porangabuçu Campus, Fortaleza 60416-030, Brazil.

Intestinal mucositis, characterized by inflammatory and/or ulcerative processes in the gastrointestinal tract, occurs due to cellular and tissue damage following treatment with 5-fluorouracil (5-FU). Rutin (RUT), a natural flavonoid extracted from , exhibits antioxidant, anti-inflammatory, cytoprotective, and gastroprotective properties. However, the effect of RUT on inflammatory processes in the intestine, especially on mucositis promoted by antineoplastic agents, has not yet been reported. Read More

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http://dx.doi.org/10.3390/molecules25122786DOI Listing

Prevalence and causes of elevated bone mass.

Bone 2020 Jun 11;138:115476. Epub 2020 Jun 11.

Univ. Lille, CHU Lille, MABLab ULR 4490, Department of Rheumatology, 59000 Lille, France. Electronic address:

Introduction: Reports of elevated bone mass (EBM) on routine DXA scanning are not infrequent. However, epidemiological studies of EBM are few in number and definition thresholds variable. The purpose of this study was to assess the prevalence and causes of EBM in the general population referred to a single university hospital - catering for a population of 4 million inhabitants - for DXA scanning. Read More

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http://dx.doi.org/10.1016/j.bone.2020.115476DOI Listing

Low frequency of IgE-mediated food hypersensitivity in mastocytosis.

J Allergy Clin Immunol Pract 2020 Jun 10. Epub 2020 Jun 10.

Department of Respiratory Medicine and Allergy, Karolinska University Hospital Huddinge, Stockholm, Sweden;; Clinical Immunology and Allergy Unit, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden;; Mastocytosis Center Karolinska, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden;. Electronic address:

Background: Patients with mastocytosis have an increased risk for severe anaphylaxis, particularly to hymenoptera venoms. These patients may also develop more often systemic hypersensitivity reactions to certain foods. However, this issue has not been systematically investigated. Read More

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http://dx.doi.org/10.1016/j.jaip.2020.05.044DOI Listing

Solitary juvenile xanthogranuloma of the hypopharynx. Clinico-pathologic study in a child with β-Thalassemia Major and Cutaneous Mastocytosis.

Int J Pediatr Otorhinolaryngol 2020 Aug 5;135:110088. Epub 2020 May 5.

Department of Molecular Medicine, Sapienza University of Rome, Italy. Electronic address:

Juvenile Xanthogranuloma (JXG), the most common pediatric non-Langerhans cell histiocytosis, may rarely occur in association with Neurofibromatosis (types 1 and 2), Juvenile Myelomonocytic Leukemia and Cutaneous Mastocytosis (CM) and, morphologically, mimics Erdheim-Chester Disease tissue lesions and ALK-positive histiocytosis. We describe a 4-year-old girl with Beta-Thalassemia Major who developed an hypopharyngeal BRAFV600E- and ALK-negative JXG and CM. JXG has been rarely reported in the aerodigestive tract and in association with CM. Read More

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http://dx.doi.org/10.1016/j.ijporl.2020.110088DOI Listing
August 2020
1.319 Impact Factor

Recent Advances in the Molecular Biology of Systemic Mastocytosis: Implications for Diagnosis, Prognosis, and Therapy.

Int J Mol Sci 2020 Jun 2;21(11). Epub 2020 Jun 2.

Department of Experimental, Diagnostic and Specialty Medicine, Hematology/Oncology "L. e A. Seràgnoli", University of Bologna, 40138 Bologna, Italy.

In recent years, molecular characterization and management of patients with systemic mastocytosis (SM) have greatly benefited from the application of advanced technologies. Highly sensitive and accurate assays for D816V mutation detection and quantification have allowed the switch to non-invasive peripheral blood testing for patient screening; allele burden has prognostic implications and may be used to monitor therapeutic efficacy. Progress in genetic profiling of , together with the use of next-generation sequencing panels for the characterization of associated gene mutations, have allowed the stratification of patients into three subgroups differing in terms of pathogenesis and prognosis: i) patients with mast cell-restricted D816V; ii) patients with multilineage D816V-involvement; iii) patients with "multi-mutated disease". Read More

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http://dx.doi.org/10.3390/ijms21113987DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7312790PMC

Red Meat Allergies after Lone Star Tick () Bites.

Authors:
James H Diaz

South Med J 2020 Jun;113(6):267-274

From the School of Public Health, Environmental, and Occupational Health Sciences, Louisiana State University Health Sciences Center, New Orleans.

Red meat allergies have followed tick bites on every continent except Antarctica. The sensitizing antigen is galactose-α-1,3-galactose (α-gal), an oligosaccharide constituent of nonprimate blood and meat, acquired by ticks during animal bloodfeeding. Because red meat allergy after tick bites is a worldwide phenomenon, the objectives of this review were to describe the global epidemiology of red meat allergy after tick bites and its immunological mechanisms; to identify the human risk factors for red meat allergy after tick bites; to identify the most common tick vectors of red meat allergy worldwide; to describe the clinical manifestations, diagnostic confirmation, and management of patients with red meat allergy after tick bites; and to recommend strategies for the prevention of tick bites. Read More

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http://dx.doi.org/10.14423/SMJ.0000000000001102DOI Listing

Treatment of Systemic Mastocytosis With the Pulsed Dye Laser.

Dermatol Surg 2020 May 29. Epub 2020 May 29.

Department of Dermatology, Duke University Medical Center, Durham, North Carolina.

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http://dx.doi.org/10.1097/DSS.0000000000002430DOI Listing

Rare causes of emesis.

Med Pharm Rep 2020 Apr 22;93(2):127-132. Epub 2020 Apr 22.

2 Medical Department, Iuliu Hatieganu University of Medicine and Pharmacy Cluj-Napoca, Romania.

Prompt diagnosis in the emergency department in the case of a patient with emesis may be difficult due to the increasing prevalence of diseases which manifest with emesis. Furthermore, in the case of chronic symptomatology, management and therapy are even more complicated. One episode of emesis rarely causes complications, but severe or repetitive episodes of emesis can cause life-threatening complications. Read More

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http://dx.doi.org/10.15386/mpr-1509DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7243886PMC

Osteoblastic lesions in systemic mastocytosis associated with hematological malignancy.

Med Clin (Barc) 2020 May 24. Epub 2020 May 24.

Servicio de Anatomía Patológica, Hospital Germans Trias i Pujol, Universitat Autònoma de Barcelona, Badalona, España.

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http://dx.doi.org/10.1016/j.medcli.2020.01.042DOI Listing

Development and characterization of a fully human antibody targeting SCF/c-kit signaling.

Int J Biol Macromol 2020 May 11;159:66-78. Epub 2020 May 11.

College of Pharmacy and Research Institute of Pharmaceutical Science and Technology (RIPST), Ajou University, 206 World Cup-ro, Yeongtong-gu, Suwon-si, Gyeonggi-do 16499, Republic of Korea; Novelty Nobility, 227 Unjung-ro, Seongnam-si, Gyeonggi-do 13477, Republic of Korea. Electronic address:

CD117/c-kit, a tyrosine kinase receptor, plays a critical role in hematopoiesis, pigmentation, and fertility. The overexpression and activation of c-kit are thought to promote tumor growth and have been reported in various cancers, including leukemia, glioblastoma and mastocytosis. To disrupt the SCF/c-kit signaling axis in cancer, we generated a c-kit antagonist human antibody (NN2101) that binds to domain 2/3 of c-kit. Read More

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http://dx.doi.org/10.1016/j.ijbiomac.2020.05.045DOI Listing

Midostaurin improves quality of life and mediator-related symptoms in advanced systemic mastocytosis.

J Allergy Clin Immunol 2020 May 11. Epub 2020 May 11.

University Medical Center Groningen, Department of Hematology, University of Groningen, Groningen, The Netherlands.

Background: Advanced systemic mastocytosis (advSM) is characterized by presence of the KIT D816V mutation and pathologic accumulation of neoplastic mast cells (MCs) in various tissues, leading to severe symptoms and organ damage (eg, cytopenias, liver dysfunction, portal hypertension, malabsorption, and weight loss). Treatment with midostaurin, an orally active multikinase/KIT inhibitor now approved for advSM in the United States and the European Union, resulted in a high rate of response accompanied by reduced MC infiltration of the bone marrow and lowered serum tryptase level.

Objective: We aimed to determine whether midostaurin improves health-related quality of life (QOL) and MC mediator-related symptoms in patients with advSM. Read More

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http://dx.doi.org/10.1016/j.jaci.2020.03.044DOI Listing

Hematological Diseases and Osteoporosis.

Int J Mol Sci 2020 May 16;21(10). Epub 2020 May 16.

Department of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy.

Secondary osteoporosis is a common clinical problem faced by bone specialists, with a higher frequency in men than in women. One of several causes of secondary osteoporosis is hematological disease. There are numerous hematological diseases that can have a deleterious impact on bone health. Read More

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http://dx.doi.org/10.3390/ijms21103538DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7279036PMC

EAACI Task force Clinical epidemiology of anaphylaxis: experts' perspective on the use of adrenaline autoinjectors in Europe.

Clin Transl Allergy 2020 11;10:12. Epub 2020 May 11.

1Division of Allergy and Immunology, Dpt. of Dermatology, Venerology and Allergology, Charité - Universitätsmedizin Berlin Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany.

Background: Worldwide, guidelines recommend the use of adrenaline autoinjectors (AAIs) for self-medication in patients who experience severe allergic reaction. The European Medical Agency recommends the prescription of two AAIs, which should be carried by patients at all times. The European Academy of Allergy and Clinical Immunology guidelines propose to prescribe a second AAI under some defined conditions. Read More

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http://dx.doi.org/10.1186/s13601-020-00317-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7216364PMC

Systemic Mastocytosis with Associated Primary Myelofibrosis.

Indian J Hematol Blood Transfus 2020 Apr 28;36(2):442-443. Epub 2019 Oct 28.

2Dipartimento di Scienze Mediche e Sanità Pubblica, SC Ematologia e CTMO, Ospedale Businco, P.O. Businco, Università di Cagliari, Azienda Ospedaliera Brotzu, Via Jenner, sn, 09124 Cagliari, Italy.

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http://dx.doi.org/10.1007/s12288-019-01225-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7229127PMC

Paratrabecular myelofibrosis and occult mastocytosis are strong morphological clues to suspect translocation in hypereosinophilia.

Indian J Hematol Blood Transfus 2020 Apr 4;36(2):384-389. Epub 2019 Dec 4.

1Department of Hematology, Post Graduate Institute of Medical Education and Research, 5th floor, Research block A, Chandigarh, 160012 India.

To study the clinico-haematological and histopathological characteristics of rearranged hypereosinophilia/hypereosinophilic syndrome (F/P+ve HE/HES), a retrospective analysis of patients with F/P+ve HE diagnosed over a period of 43 months was performed. Peripheral blood smears, bone marrow aspirate (BMA) and biopsies (BMB) were reviewed in each case and; reticulin stain and immunohistochemistry for mast cell tryptase (MCT) and CD117 was performed. F/P+ve HE was diagnosed in a total of ten patients during study period. Read More

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http://dx.doi.org/10.1007/s12288-019-01236-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7229073PMC
April 2020
0.234 Impact Factor

Importance of Adequate Diagnostic Workup for Correct Diagnosis of Advanced Systemic Mastocytosis.

J Allergy Clin Immunol Pract 2020 May 15. Epub 2020 May 15.

Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany. Electronic address:

Background: Little is known about the epidemiology of advanced systemic mastocytosis (advSM).

Objectives: To investigate epidemiologic features and diagnostic pitfalls of advSM in Germany.

Methods: Therefore, 140 patients from a single German reference center of the European Competence Network on Mastocytosis between 2003 and 2018 were analyzed. Read More

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http://dx.doi.org/10.1016/j.jaip.2020.05.005DOI Listing

Trichinella spiralis-induced mastocytosis and erythropoiesis are simultaneously supported by a bipotent mast cell/erythrocyte precursor cell.

PLoS Pathog 2020 May 18;16(5):e1008579. Epub 2020 May 18.

Center for Immunity and Inflammation, New Jersey Medical School, Rutgers-The State University of New Jersey, Newark, New Jersey, United States of America.

Anti-helminth responses require robust type 2 cytokine production that simultaneously promotes worm expulsion and initiates the resolution of helminth-induced wounds and hemorrhaging. However, how infection-induced changes in hematopoiesis contribute to these seemingly distinct processes remains unknown. Recent studies have suggested the existence of a hematopoietic progenitor with dual mast cell-erythrocyte potential. Read More

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http://dx.doi.org/10.1371/journal.ppat.1008579DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7259795PMC

New Approach to Paediatric Mastocytosis: Implications of KIT D816V Mutation Detection in Peripheral Blood.

Acta Derm Venereol 2020 May 28;100(10):adv00149. Epub 2020 May 28.

Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland.

is missing (Short communication). Read More

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http://dx.doi.org/10.2340/00015555-3504DOI Listing

A Rare Case of Systemic Mastocytosis with Associated Hematologic Neoplasm (SM-AHN) Involving Chronic Myeloid Leukemia: A Case Report and Literature Review.

Am J Case Rep 2020 May 13;21:e923354. Epub 2020 May 13.

Department of Medical Oncology, Hematology Section, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.

BACKGROUND Single or multiple cell line dysplasia is a characteristic feature of myelodysplastic syndrome. However, significant dysgranulopoiesis is not a feature of chronic myeloid leukemia (CML). Systemic mastocytosis (SM) with an associated hematologic neoplasm (SM-AHN) comprises 5% to 40% of cases of SM. Read More

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http://dx.doi.org/10.12659/AJCR.923354DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7252834PMC

[Systemic mastocytosis involving intestinal tract: report of a case].

Zhonghua Bing Li Xue Za Zhi 2020 May;49(5):499-501

Department of Digestion, the Third Hospital, Peking University Health Science Center, Beijing 100191, China.

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http://dx.doi.org/10.3760/cma.j.cn112151-20190808-00442DOI Listing

European Medicines Agency review of midostaurin (Rydapt) for the treatment of adult patients with acute myeloid leukaemia and systemic mastocytosis.

ESMO Open 2019 11;4(6)

European Medicines Agency, Amsterdam, The Netherlands.

On 18 September 2017, a marketing authorisation valid through the European Union (EU) was issued for midostaurin in combination with standard daunorubicin and cytarabine induction and high-dose cytarabine consolidation chemotherapy and for patients in complete response followed by midostaurin single agent maintenance therapy, for adult patients with newly diagnosed acute myeloid leukaemia (AML) who are Fms-like tyrosine kinase 3 mutation positive and as monotherapy for the treatment of adult patients with aggressive systemic mastocytosis (ASM), systemic mastocytosis with associated haematological neoplasm (SM-AHN) or mast cell leukaemia (MCL). The recommended dose of midostaurin is 50 mg orally twice daily for AML and 100 mg orally twice daily for ASM, SM-AHN and MCL. Midostaurin was evaluated in two pivotal studies. Read More

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http://dx.doi.org/10.1136/esmoopen-2019-000606DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7001097PMC
November 2019

Simultaneous occurrence of cutaneous mastocytosis and juvenile xanthogranuloma in a child: Random or true association?

Pediatr Dermatol 2020 May 5. Epub 2020 May 5.

Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast), Spanish Network on Mastocytosis (REMA), Toledo, Spain.

Juvenile xanthogranuloma (JXG) and cutaneous mastocytosis (CM) are two distinct conditions that have rarely been reported in association. We report a child with CM and disseminated JXG, who showed a significant decrease in serum tryptase levels and regression of JXG lesions over time. Due to the paucity of reports, a true association between these two conditions has not been validated, although a potential induction of histiocytic lesions by mast cell degranulation has been proposed. Read More

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http://dx.doi.org/10.1111/pde.14185DOI Listing

Serum tryptase levels in pediatric mastocytosis and association with systemic symptoms.

Ann Allergy Asthma Immunol 2020 May 1. Epub 2020 May 1.

Section of Allergy and Immunology, Department of Pediatrics Baylor College of Medicine, Houston, Texas; Texas Children's Hospital, Houston and The Woodlands, Texas. Electronic address:

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http://dx.doi.org/10.1016/j.anai.2020.04.021DOI Listing

Histone Methyltransferase Inhibition Has a Cytotoxic Impact on Transformed Mast Cells: Implications for Mastocytosis.

Anticancer Res 2020 May;40(5):2525-2536

Uppsala University, Department of Medical Biochemistry and Microbiology, Uppsala, Sweden

Background/aim: Mast cell transformation, as manifested in mastocytosis, can be a serious condition for which there are limited therapeutic options. Mastocytosis cells can be sensitive to histone deacetylase (HDAC) inhibitors, but their sensitivity to other histone-modifying enzymes has not been assessed. Here we addressed this issue. Read More

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http://dx.doi.org/10.21873/anticanres.14223DOI Listing

Microarray-Based Detection of Allergen-Reactive IgE in Patients with Mastocytosis.

J Allergy Clin Immunol Pract 2020 Apr 26. Epub 2020 Apr 26.

Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria. Electronic address:

Background: Because of a high risk to develop fatal anaphylaxis, early detection of immunoglobulin E (IgE)-dependent allergy is of particular importance in patients with mastocytosis.

Objective: We examined whether microarray-based screening for allergen-reactive IgE (allergen-chip) is a sensitive and robust approach to detect specific IgE in patients with mastocytosis.

Methods: Sera for 42 patients were analyzed, including 4 with cutaneous mastocytosis, 2 with mastocytosis in the skin, and 36 with systemic mastocytosis. Read More

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http://dx.doi.org/10.1016/j.jaip.2020.04.030DOI Listing

Systemic Mastocytosis: Following the Tyrosine Kinase Inhibition Roadmap.

Front Pharmacol 2020 14;11:443. Epub 2020 Apr 14.

Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast) and CIBERONC, Hospital Virgen del Valle, Toledo, Spain.

Systemic mastocytosis is a rare and heterogeneous disease characterized by mast cell proliferation and activation. KIT is a transmembrane tyrosine kinase which plays a key role in mast cell growth, differentiation and survival. After interaction with its ligand, the stem cell factor, KIT dimerizes activating downstream pathways involving multiple tyrosine kinases (PI3K, JAK/STAT, RAS/ERK). Read More

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http://dx.doi.org/10.3389/fphar.2020.00443DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7171446PMC

Non-hematologic diagnosis of systemic mastocytosis: Collaboration of radiology and pathology.

Blood Rev 2020 Apr 8:100693. Epub 2020 Apr 8.

Division of Hematology, Oncology and Cellular Therapy, Department of Medicine, Rush University, Chicago, IL, USA. Electronic address:

Systemic mastocytosis (SM) is a hematologic disease with a wide range of clinical courses ranging from an indolent condition with normal life expectancy to exceedingly aggressive disorder with a poor prognosis. The symptoms and signs of SM result from the release of mast cell mediators with heterogeneous functions, and/or organ damage from neoplastic mast cell infiltration, or both. Diagnostic criteria for SM are well-defined by the World Health Organization (WHO). Read More

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http://dx.doi.org/10.1016/j.blre.2020.100693DOI Listing

Diagnosis of mast cell activation syndrome: a global "consensus-2".

Diagnosis (Berl) 2020 Apr 22. Epub 2020 Apr 22.

Institute of Human Genetics, University Hospital of Bonn, Bonn, Germany.

The concept that disease rooted principally in chronic aberrant constitutive and reactive activation of mast cells (MCs), without the gross MC neoplasia in mastocytosis, first emerged in the 1980s, but only in the last decade has recognition of "mast cell activation syndrome" (MCAS) grown significantly. Two principal proposals for diagnostic criteria have emerged. One, originally published in 2012, is labeled by its authors as a "consensus" (re-termed here as "consensus-1"). Read More

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http://dx.doi.org/10.1515/dx-2020-0005DOI Listing

Systemic mastocytosis associated with Hodgkin's lymphoma in a 4-year-old child.

Pediatr Dermatol 2020 Apr 22. Epub 2020 Apr 22.

Department of Pediatric Dermatology, Institute of Child Health, Kolkata, India.

Systemic mastocytosis with associated clonal hematologic non-mast cell lineage disease (SM-AHN) represents a specific subtype of mastocytosis and is extremely rare in children. We describe a 4-year-old child with systemic mastocytosis associated with Hodgkin's lymphoma. The child had cutaneous mastocytosis and lymphadenopathy without other clinical features of SM, which was diagnosed only by bone marrow examination. Read More

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http://dx.doi.org/10.1111/pde.14183DOI Listing

[HEREDITARY ALPHA TRYPTASEMIA - NEW DIAGNOSIS, FAMILIAR SYMPTOMS].

Harefuah 2020 Apr;159(4):253-255

Allergy and Clinical Immunology Unit, Meir General Hospital, Kfar-Saba, Israel.

Introduction: Mast cells are mostly known for their role in allergic reactions, as well as their involvement in diseases such as mast cell activation syndrome and systemic mastocytosis. One of the secreted enzymes during the allergic reaction and mast cell degranulation is tryptase. Recently, a new syndrome, namely - hereditary alpha tryptasemia, has been defined, originating from multiple copies of TPSAB1, the alpha-tryptase encoding gene. Read More

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A case of unexplained cutaneous lesions, cholestatic hepatitis and non-cirrhotic portal hypertension in a female patient.

Gastroenterology 2020 Apr 16. Epub 2020 Apr 16.

Gastroenterology department, Hospital Central do Funchal, Funchal, Portugal.

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http://dx.doi.org/10.1053/j.gastro.2020.03.076DOI Listing

Bone mineral density in patients with systemic mastocytosis: correlations with clinical and histopathological features.

Clin Exp Rheumatol 2020 Apr 17. Epub 2020 Apr 17.

Department of Diagnostic and Interventional Radiology, University of Leipzig, Germany.

Objectives: Systemic mastocytosis (SM) is a heterogeneous haematological entity characterised by proliferation of mast cells. Skeletal abnormalities of SM include osteolysis, osteopenia and osteoporosis but also osteosclerosis. A routinely used modality to assess bone density is dual-energy x-ray absorptiometry (DXA). Read More

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Idiopathic Anaphylaxis: A Form of Mast Cell Activation Syndrome.

J Allergy Clin Immunol Pract 2020 Apr;8(4):1196-1201

Brigham and Women's Hospital, Division of Allergy and Clinical Immunology, Boston, Mass; Harvard Medical School, Boston, Mass.

Idiopathic anaphylaxis is a condition caused by paroxysmal episodes of sudden-onset multiorgan involvement variably including laryngeal edema, urticaria, bronchoconstriction, dyspnea, hypoxia, abdominal pain, nausea, vomiting, diarrhea, and hypotension. Rarely, the episodes can lead to cardiovascular collapse and death in the absence of a clear trigger, especially in the presence of other cardiovascular comorbidities. Elevated mast cell mediators such as tryptase and histamine have been reported during episodes, and mast cells are considered the primary cells responsible for driving anaphylaxis in humans. Read More

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http://dx.doi.org/10.1016/j.jaip.2019.10.048DOI Listing

Changes in lung immune cells related to clinical outcome during treatment with infliximab for sarcoidosis.

Clin Exp Immunol 2020 Jul 22;201(1):85-93. Epub 2020 Apr 22.

Department of Respiratory Medicine, Theme Inflammation and Infection, Karolinska University Hospital, Stockholm, Sweden.

Pulmonary sarcoidosis is characterized by an exaggerated CD4 T cell response and formation of non-necrotizing granulomas. Tumour necrosis factor α (TNF-α) is regarded as crucial for granuloma formation and TNF-α inhibitors offer a third-line treatment option for patients not responding to conventional treatment. However, not all patients benefit from treatment, and an optimal dose and treatment duration have not been established. Read More

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http://dx.doi.org/10.1111/cei.13438DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7290087PMC

Mastocytosis with sudden recurrence of rash after a 13-year interval.

J Dermatol 2020 Jul 9;47(7):e271-e272. Epub 2020 Apr 9.

Department of Dermatology, Kansai Medical University, Hirakata, Japan.

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http://dx.doi.org/10.1111/1346-8138.15347DOI Listing

Bullae on the extremities of a newborn: A case of diffuse cutaneous mastocytosis mimicking epidermolysis bullosa.

J Eur Acad Dermatol Venereol 2020 Apr 8. Epub 2020 Apr 8.

Department of dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China, 100045.

Mastocytosis is an uncommon disorder characterized by clonal proliferation of mast cells in one or more organs, including cutaneous mastocytosis limited to the skin and systemic mastocytosis involving the bone marrow, liver, spleen, or lymph nodes . Diffuse cutaneous mastocytosis (DCM) is a rare variant of cutaneous mastocytosis with an incidence of 3.57%-5. Read More

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http://dx.doi.org/10.1111/jdv.16438DOI Listing

[Cutaneous Manifestations in Mastocytosis: Update].

Acta Med Port 2020 Apr 1;33(4):275-281. Epub 2020 Apr 1.

Serviço de Dermatologia. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto. Unidade de Investigação em Dermatologia. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto. Portugal.

Introduction: Mastocytosis is characterized by the clonal expansion of morphological and immunophenotypically abnormal mast cells in different organs. The skin is the most frequently affected tissue. Virtually all children and more than 80% of adult patients with mastocytosis show cutaneous lesions. Read More

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http://dx.doi.org/10.20344/amp.12189DOI Listing

Acute Myocardial Infarction In Systemic Mastocytosis: Case Report With Literature Review Of The Role Of Inflammatory Process In Acute Coronary Syndrome.

Curr Cardiol Rev 2020 Mar 31. Epub 2020 Mar 31.

Department of Medicine, State University of New York, Downstate Medical Center. United States.

Systemic Mastocytosis (SM) is a disorder of excessive mast cell infiltration in multiple organ tissues. Atherosclerosis is a major risk factor for developing acute coronary syndrome [1]. In addition to lipid accumulation in the arterial wall, inflammation plays an important role in the pathogenesis of plaque rupture and activating the thrombosis cascade [2]. Read More

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http://dx.doi.org/10.2174/1573403X16666200331123242DOI Listing

Extensive Cutaneous Mastocytosis After Pregnancy.

Cureus 2020 Feb 20;12(2):e7057. Epub 2020 Feb 20.

Dermatology, St. Joseph Dermatopathology, Houston, USA.

Mastocytosis is an uncommon disease involving the proliferation of mast cells within at least one organ system, most commonly the skin. One rare variant is telangiectasia macularis eruptive perstans (TMEP). The telangiectatic tan-brown macules are highly characteristic, although a biopsy is indicated to confirm the diagnosis. Read More

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http://dx.doi.org/10.7759/cureus.7057DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7086116PMC
February 2020

Pharmacotherapy in Mast Cell Leukemia.

Expert Opin Pharmacother 2020 Jun 25;21(9):1059-1069. Epub 2020 Mar 25.

Department of Interventional Radiology and Integrated Medical Oncology, IRCCS Istituto Tumori "Giovanni Paolo II" , Bari, Italy.

Introduction: Mast cell leukemia (MCL) is one of the most aggressive forms of Systemic Mastocytosis (SM), a complex family of rare diseases, for which standard therapies are very few. MCL represents only <1% cases of SM and this is the reason why there are no specific clinical trials to better explore this disease. As a consequence, MCL is treated and grouped within other forms of SM, being all KIT-driven diseases; however, its KIT dysregulation leads to uncontrolled activation of mast cells (MCs), which correlates with forms of myeloid acute leukemia (AML). Read More

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http://dx.doi.org/10.1080/14656566.2020.1744566DOI Listing

Predictors of severe anaphylaxis in Hymenoptera venom allergy: The importance of absence of urticaria and angioedema.

Ann Allergy Asthma Immunol 2020 Jul 18;125(1):72-77. Epub 2020 Mar 18.

Dermatology Clinic, University Hospital Dresden, Dresden, Germany.

Background: Severe anaphylaxis (SA) in Hymenoptera venom allergy has been associated with a number of risk factors. However, the effect of several of those risk factors on the severity of anaphylaxis is poorly defined.

Objective: To evaluate risk factors for SA in Hymenoptera venom allergy. Read More

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http://dx.doi.org/10.1016/j.anai.2020.03.007DOI Listing