2,366 results match your criteria Mastocytosis Systemic


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

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

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

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

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

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

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

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

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

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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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

Age Distribution of Multiple Functionally Relevant Subsets of CD4+ T Cells in Human Blood Using a Standardized and Validated 14-Color EuroFlow Immune Monitoring Tube.

Front Immunol 2020 27;11:166. Epub 2020 Feb 27.

Translational and Clinical Research Program, Centro de Investigación del Cáncer (CIC) and Instituto de Biología Molecular y Celular del Cancer (IBMCC), CSIC-University of Salamanca (USAL), Salamanca, Spain.

CD4+ T cells comprise multiple functionally distinct cell populations that play a key role in immunity. Despite blood monitoring of CD4+ T-cell subsets is of potential clinical utility, no standardized and validated approaches have been proposed so far. The aim of this study was to design and validate a single 14-color antibody combination for sensitive and reproducible flow cytometry monitoring of CD4+ T-cell populations in human blood to establish normal age-related reference values and evaluate the presence of potentially altered profiles in three distinct disease models-monoclonal B-cell lymphocytosis (MBL), systemic mastocytosis (SM), and common variable immunodeficiency (CVID). Read More

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http://dx.doi.org/10.3389/fimmu.2020.00166DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7056740PMC
February 2020

Educational Case: Systemic Mastocytosis with an Associated Hematological Neoplasm.

Acad Pathol 2020 Jan-Dec;7:2374289520906526. Epub 2020 Feb 27.

Department of Pathology and Laboratory Medicine, The University of Texas Health Center at Houston, TX 77030, USA.

http://journals.sagepub.com/doi/10. Read More

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http://dx.doi.org/10.1177/2374289520906526DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7047421PMC
February 2020

The Utility of Measuring Urinary Metabolites of Mast Cell Mediators in Systemic Mastocytosis and Mast Cell Activation Syndrome.

J Allergy Clin Immunol Pract 2020 Mar 3. Epub 2020 Mar 3.

Division of Allergic Diseases, Mayo Clinic, Rochester, Minn.

Mast cells (MCs) leave evidence of their presence and activation. Aside from increased numbers of MCs in tissues, this evidence includes detecting elevated serum levels of tryptase and discovering increased excretion of urinary metabolites of prostaglandin (PG) D, leukotriene (LT) C, and/or histamine. The importance of measuring these nontryptase mediator metabolites has largely gone unnoticed. Read More

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http://dx.doi.org/10.1016/j.jaip.2020.02.021DOI Listing

Bone lesions in systemic mastocytosis: Bone histomorphometry and histopathological mechanisms.

Morphologie 2020 May 1;104(345):97-108. Epub 2020 Mar 1.

Groupe études remodelage osseux et biomatériaux (GEROM), EA-4658, SFR-4208, IRIS-Institut de Biologie en Santé (IBS), université d'Angers, CHU d'Angers, 49933 Angers, France. Electronic address:

Osteoporosis is considered the most frequent skeletal manifestation of systemic mastocytosis (SM). We performed a retrospective analysis of sixty patients (37 males and 23 females) who underwent a bone biopsy in the assessment of SM or in the assessment of unexplained bone fragility. Thirty-three had simultaneously a bone marrow biopsy with a Jamshidi's needle; this sample was used for immunohistochemical analysis (tryptase, c-KIT. Read More

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http://dx.doi.org/10.1016/j.morpho.2020.01.004DOI Listing

Elevated Serum Tryptase in Non-Anaphylaxis Cases: A Concise Review.

Authors:
Adrian Y S Lee

Int Arch Allergy Immunol 2020 3;181(5):357-364. Epub 2020 Mar 3.

Department of Allergy/Clinical Immunology and SA Pathology, Flinders Medical Centre, Bedford Park, South Australia, Australia,

One of the most important blood tests in the field of allergy, mast cell tryptase has numerous diagnostic uses, particularly for anaphylactic reactions and for the diagnosis of mastocytosis. However, there are numerous other non-anaphylactic conditions where clinicians may see elevated serum tryptase (hypertryptasemia) and the practicing clinician ought to be aware of these important differential diagnoses. Such conditions include systemic mastocytosis, hematological malignancies, and chronic kidney disease. Read More

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http://dx.doi.org/10.1159/000506199DOI Listing

Siglec-7, a target for novel therapeutical approaches of Systemic Mastocytosis.

Pharmacol Res 2020 Aug 29;158:104731. Epub 2020 Feb 29.

Department of Immunology, IRCCS Bambino Gesù Children's Hospital, 00146, Roma, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.phrs.2020.104731DOI Listing

Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification.

Allergy 2020 Feb 28. Epub 2020 Feb 28.

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

Background: In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM. Read More

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http://dx.doi.org/10.1111/all.14248DOI Listing
February 2020

Omalizumab in the treatment of adult patients with mastocytosis: A systematic review.

Clin Exp Allergy 2020 Jun 25;50(6):654-661. Epub 2020 Mar 25.

Department of Dermatology, Mastocytosis National Reference Center (CEREMAST), Toulouse University Hospital, Toulouse, France.

 BACKGROUND: Mastocytosis is associated with mast cell (MC) mediator-related symptoms for which limited therapies are available. OBJECTIVE: Our aim was to assess the efficacy and safety of omalizumab in the treatment of MC mediator-related symptoms in adult patients with mastocytosis. RESULTS: We identified one multi-centre retrospective cohort study (39 patients), one retrospective cohort study (13 patients), 4 case series and 10 case reports. Read More

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http://dx.doi.org/10.1111/cea.13592DOI Listing

New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis.

Blood 2020 Apr;135(16):1365-1376

Division of Hematology, Stanford University School of Medicine/Stanford Cancer Institute, Stanford, CA.

Systemic mastocytosis (SM) has greatly benefited from the broad application of precision medicine techniques to hematolymphoid neoplasms. Sensitive detection of the recurrent KIT D816V mutation and use of next-generation sequencing (NGS) panels to profile the genetic landscape of SM variants have been critical adjuncts to the diagnosis and subclassification of SM, and development of clinical-molecular prognostic scoring systems. Multilineage KIT involvement and multimutated clones are characteristic of advanced SM (advSM), especially SM with an associated hematologic neoplasm (AHN). Read More

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http://dx.doi.org/10.1182/blood.2019000932DOI Listing

[Mast cell activation syndrome. About a clinical case].

Rev Alerg Mex 2019 Oct-Dec;66(4):504-509

Universidad de Antioquia, Grupo de Alergología Clínica y Experimental, Medellín, Colombia.

Background: Monoclonal mast cell activation syndrome is included in mast cell activation disorders in which, after a diagnostic process, it is not possible to meet the required criteria for a diagnosis of systemic mastocytosis.

Clinical Case: A 73-year-old woman who presented two events of anaphylaxis 15 minutes after the intake of yucca; with a positive skin test, elevated tryptase, and mast cells with abnormal phenotype in the bone marrow biopsy, and without criteria for systemic mastocytosis.

Conclusion: The diagnosis of monoclonal mast cell activation syndrome requires high clinical suspicion for patients with recurrent anaphylaxis and elevated tryptase, for whom joint management with hematology is essential. Read More

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http://dx.doi.org/10.29262/ram.v66i4.587DOI Listing
February 2020

Avapritinib: First Approval.

Authors:
Sohita Dhillon

Drugs 2020 Mar;80(4):433-439

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

Avapritinib (AYVAKIT™) is a potent and selective tyrosine kinase inhibitor of platelet-derived growth factor receptor alpha (PDGFRA) and KIT activation loop mutants. It is being developed by Blueprint Medicines for the treatment of gastrointestinal stromal tumours (GIST), solid tumours and systemic mastocytosis. Avapritinib is approved in the USA for PDGFRA exon 18 (including D842V) mutant GIST and is undergoing regulatory assessment in the USA as a 4th-line treatment for GIST. Read More

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http://dx.doi.org/10.1007/s40265-020-01275-2DOI Listing

Presumed mast cell choroidal infiltrate in aggressive systemic mastocytosis.

Am J Ophthalmol Case Rep 2020 Jun 4;18:100614. Epub 2020 Feb 4.

Department of Ophthalmology and Visual Neuroscience, University of Minnesota, Minneapolis, MN, USA.

Purpose: To report a rare case of a unilateral choroidal mast cell infiltration in a patient with aggressive systemic mastocytosis (ASM).

Observations: The patient is a man in his fifties with a diagnosis of ASM. He developed visual complaints in the right eye associated with an area of subretinal fluid on fundus examination. Read More

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http://dx.doi.org/10.1016/j.ajoc.2020.100614DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7021538PMC

Primary cicatricial alopecia associated with systemic indolent mastocytosis.

JAAD Case Rep 2020 Feb 30;6(2):146-148. Epub 2020 Jan 30.

Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts.

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http://dx.doi.org/10.1016/j.jdcr.2019.11.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7000442PMC
February 2020

Activation of Siglec-7 results in inhibition of in vitro and in vivo growth of human mast cell leukemia cells.

Pharmacol Res 2020 Aug 5;158:104682. Epub 2020 Feb 5.

Pharmacology & Experimental Therapeutics Unit, Institute for Drug Research, School of Pharmacy, Faculty of Medicine, The Hebrew University of Jerusalem, Israel. Electronic address:

Advanced systemic mastocytosis is a rare and still untreatable disease. Blocking antibodies against inhibitory receptors, also known as "immune checkpoints", have revolutionized anti-cancer treatment. Inhibitory receptors are expressed not only on normal immune cells, including mast cells but also on neoplastic cells. Read More

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http://dx.doi.org/10.1016/j.phrs.2020.104682DOI Listing

Risk of comorbidities in patients diagnosed with chronic urticaria: A nationwide registry-study.

World Allergy Organ J 2020 Jan 25;13(1):100097. Epub 2020 Jan 25.

Department of Dermatology, Aarhus University Hospital, Aarhus, Denmark.

Background: The autoimmune profile of Chronic Urticaria (CU) patients is an increasing topic of interest. Associated diseases suggest shared pathogenic pathways, and they may provide important knowledge on specific targets for future treatment models. In this study we examined the prevalence and risk of comorbidities in CU. Read More

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http://dx.doi.org/10.1016/j.waojou.2019.100097DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6994395PMC
January 2020

Aurora kinase inhibitor tozasertib suppresses mast cell activation in vitro and in vivo.

Br J Pharmacol 2020 Jun 6;177(12):2848-2859. Epub 2020 Apr 6.

Department of Biochemistry and Molecular Biology, School of Medicine, Shenzhen University, Shenzhen, China.

Background And Purpose: Mast cells are important in allergic reactions. Here, we assessed the anti-allergic effects of the anti-cancer drug tozasertib specifically regarding regulatory effects on mast cell activation.

Experimental Approach: Tozasertib effects on mast cell degranulation were determined by measuring β-hexosaminidase and histamine release and by assessing morphological changes in RBL-2H3 and mouse bone marrow-derived mast cells (BMMCs) stimulated with mouse anti-dinitrophenyl (DNP)-IgE/DNP-human serum albumin or human LAD2 cells activated with phorbol-12-myristate 13-acetate plus calcium ionophore (PMACI). Read More

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http://dx.doi.org/10.1111/bph.15012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7236079PMC

Can Diagnostic Low-dose Whole-body CT Reflect Bone Marrow Findings in Systemic Mastocytosis?

Anticancer Res 2020 Feb;40(2):1015-1022

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

Background/aim: Systemic mastocytosis (SM) is a heterogeneous hematological entity, characterized by the proliferation of mast cells, commonly involving the skeleton. The present study sought to elucidate whether the computed tomographic (CT) number as Hounsfield units (HU) derived from whole-body CT is associated with bone marrow findings in SM.

Patients And Methods: Patient records of the local Oncology and Hematology Department from 2007 to 2018 were screened for patients with SM. Read More

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http://dx.doi.org/10.21873/anticanres.14036DOI Listing
February 2020

The clinical and pathological panoply of systemic mastocytosis.

Br J Haematol 2020 Mar 27;188(5):623-640. Epub 2020 Jan 27.

Department of Cellular Pathology, Guys and St Thomas' NHS Hospitals, London, UK.

Mastocytosis is a rare disease with varied presentation, myriad symptomatology and variable prognosis. Most patients present with cutaneous disease and mediator-related symptomatology with a small subset having systemic disease (systemic mastocytosis, SM). A subset of the latter develops synchronous or metachronous haematologic neoplasms (SM-AHN), most commonly chronic myelomonocytic leukaemia (CMML). Read More

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http://dx.doi.org/10.1111/bjh.16288DOI Listing