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    1 OF 134

    Patterns of anaphylaxis after diagnostic work-up: a follow-up study of 226 patients with suspected anaphylaxis.
    Allergy 2017 May 19. Epub 2017 May 19.
    Department of Dermatology and Allergy Center, Odense Research Center for Anaphylaxis (ORCA), Odense University Hospital, Odense, Denmark.
    Background: Most published studies on anaphylaxis are retrospective or register based. Data on subsequent diagnostic work-up are sparse. We aimed to characterize patients seen with suspected anaphylaxis at the emergency care setting (ECS), after subsequent diagnostic work-up at our Allergy Center (AC). Read More

    R634W KIT Mutation in an Adult With Systemic Mastocytosis.
    Lab Med 2017 May 18. Epub 2017 May 18.
    Departments of Pathology and Laboratory Medicine, Yale University School of Medicine, Pathology and Laboratory Medicine, VA Connecticut Healthcare System, West Haven, CT.
    Mastocytosis is a clonal neoplasm with the potential to affect various organs within the body. It can range in clinical severity from benign to extremely aggressive. Mastocytosis can be separated into cutaneous, systemic, and leukemic forms, as well as mast-cell sarcoma and extracutaneous mastocytoma. Read More

    Morphologic Confounders and CD19 Negativity in a Case of Hairy Cell Leukemia.
    Mediterr J Hematol Infect Dis 2017 1;9(1):e2017033. Epub 2017 May 1.
    Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh - 160012, India.
    Objectives: We report a case of hairy cell leukemia (HCL) initially misdiagnosed as plasma cell dyscrasia due to various clinical, morphological and immunophenotypic confounders.

    Methods And Results: In a patient diagnosed of marrow plasmacytosis and serum monoclonal protein elsewhere and referred to our hospital, morphological evaluation of bone marrow aspirate smears and trephine biopsy, immunophenotyping, and molecular testing (BRAFV600E mutation) were done. Clinically, the patient was asymptomatic; bone marrow revealed plasmacytosis, mastocytosis, and lymphocytosis with a few "hairy" cells. Read More

    Deficiency of Interleukin-1 Receptor Antagonist (DIRA): Report of the First Indian Patient and a Novel Deletion Affecting IL1RN.
    J Clin Immunol 2017 May 15. Epub 2017 May 15.
    Translational Autoinflammatory Diseases Studies, NIAID, NIH, Bethesda, MD, USA.
    Purpose: Deficiency of interleukin-1 receptor antagonist (DIRA) is a rare life-threatening autoinflammatory disease caused by autosomal recessive mutations in IL1RN. DIRA presents clinically with early onset generalized pustulosis, multifocal osteomyelitis, and elevation of acute phase reactants. We evaluated and treated an antibiotic-unresponsive patient with presumed DIRA with recombinant IL-1Ra (anakinra). Read More

    Differences in the imaging features and distribution of non-indolent and indolent mastocytosis: a single institution experience of 29 patients.
    Clin Imaging 2017 May 6;44:111-116. Epub 2017 May 6.
    Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115, United States; Department of Imaging, Dana Farber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, Boston, MA 02215, United States.
    Purpose: Compare imaging features of indolent and non-indolent mastocytosis.

    Methods: For 29 patients, imaging features, imaging indications, and distribution of indolent and non-indolent mastocytosis subtypes were analyzed.

    Results: 16/29 (55%) patients had three distinct patterns of osseous abnormality, not significantly differing between cohorts. Read More

    Fatal Anaphylaxis to Yellow Jacket Stings in Mastocytosis: Options for Identification and Treatment of At-Risk Patients.
    J Allergy Clin Immunol Pract 2017 May 10. Epub 2017 May 10.
    Department of Allergology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands; GRIAC Research Institute, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
    Background: Patients with indolent systemic mastocytosis (ISM) are at risk for severe anaphylactic reactions to yellow jacket (YJ) stings while demonstration of sensitization can be challenging because specific IgE (sIgE) levels are regularly below 0.35 kUA/L. The implication of missing YJ allergy is illustrated by a case of fatal anaphylaxis. Read More

    Routine abdominal ultrasonography has limited value in the care for patients with indolent systemic mastocytosis.
    Hematology 2017 May 10:1-4. Epub 2017 May 10.
    a Department of Internal Medicine, Section of Clinical Immunology , Erasmus University Medical Centre , Rotterdam , Netherlands.
    Objectives: Systemic mastocytosis (SM) is a myeloproliferative disease characterized by the accumulation of aberrant mast cells. Since advanced subtypes of SM can lead to organ dysfunction and shortened survival, timely recognition of progressive disease is important for the adequate treatment of SM patients.

    Methods: Here, we report the results of our cohort study on the value of routine abdominal ultrasonography for the detection of progression of indolent systemic mastocytosis (ISM). Read More

    Tolerability and benefit of a tetramethoxyluteolin-containing skin lotion.
    Int J Immunopathol Pharmacol 2017 May 1:394632017707610. Epub 2017 May 1.
    1 Molecular Immunopharmacology and Drug Discovery Laboratory, Department of Integrative Physiology and Pathobiology, Tufts University School of Medicine, Boston, MA, USA.
    As many as 40% of people have sensitive skin and at least half of them suffer from pruritus associated with allergies, atopic dermatitis (AD), chronic urticaria (CU), cutaneous mastocytosis (CM), and psoriasis. Unfortunately, the available topical formulations contain antihistamines that are often not as effective as those containing corticosteroids. Certain natural flavonoids have anti-inflammatory actions. Read More

    [Uncommon dermatologic disorders triggered by radiation therapy of breast cancer: A case-series].
    Cancer Radiother 2017 Apr 28. Epub 2017 Apr 28.
    Institut universitaire du cancer Toulouse Oncopole, 1, avenue Irène-Joliot-Curie, 31059 Toulouse cedex 9, France; Oncodermatologie, institut Claudius-Regaud, 1, avenue Irène-Joliot-Curie, 31059 Toulouse cedex 9, France. Electronic address:
    Radiotherapy's main skin toxicities are now well-separated, acute (acute radiation dermatitis) or chronic complications (chronic radiation dermatitis, induced cutaneous carcinoma, aesthetic sequelae). Exceptionally, radiotherapy may induce, by isomorphic reaction or Koebner's phenomenon, some specific dermatosis. In this article, we report five new observations of these unusual complications of radiation therapy, occurring in very variable time after breast irradiation and remaining strictly localized in the irradiated field (cutaneous mastocytosis, Sweet syndrome, lichen planus, vitiligo). Read More

    Killer cell immunoglobulin-like receptor 2DL4 is expressed in and suppresses the cell growth of Langerhans cell histiocytosis.
    Oncotarget 2017 Apr 7. Epub 2017 Apr 7.
    Department of Diagnostic Pathology, Kyoto University Hospital, Kyoto, Japan.
    Killer cell immunoglobulin-like receptor (KIR) 2DL4 (CD158d) is a receptor for human leukocyte antigen-G. The function of KIR2DL4 has been reported in human natural killer cell lymphoma and mastocytosis, but not in Langerhans cell histiocytosis (LCH). Herein, we examined the expression and function of KIR2DL4 in LCHs. Read More

    Systemic mastocytosis with KIT V560G mutation presenting as recurrent episodes of vascular collapse: response to disodium cromoglycate and disease outcome.
    Allergy Asthma Clin Immunol 2017 24;13:21. Epub 2017 Apr 24.
    Consulta Multidisciplinar de Linfomas Cutâneos e Mastocitoses (CMLC), Hospital de Santo António (HSA), Centro Hospitalar do Porto (CHP), Porto, Portugal.
    Background: Mastocytosis are rare diseases characterized by an accumulation of clonal mast cells (MCs) in one or multiple organs or tissues. Patients with systemic mastocytosis (SM), whose MCs frequently arbor the activating D816V KIT mutation, may have indolent to aggressive diseases, and they may experience MC mediator related symptoms. Indolent SM with recurrent anaphylaxis or vascular collapse in the absence of skin lesions, ISMs(-), is a specific subtype indolent SM (ISM), and this clonal MC activation disorder represents a significant fraction of all MC activation syndromes. Read More

    Continuous diphenhydramine infusion and imatinib for KIT-D816V-negative mast cell activation syndrome: a case report.
    J Med Case Rep 2017 Apr 24;11(1):119. Epub 2017 Apr 24.
    Rosenfeld Cancer Center, Abington Jefferson Health, 1200 Old York Road, Abington, PA, 19001, USA.
    Background: We present the first full case report of the treatment of mast cell activation syndrome with continuous diphenhydramine infusion, which resulted in the improvement of anaphylactic reactions and a decrease in hospital readmission. Furthermore, the patient received imatinib in the absence of the KIT-D816V mutation, which led to further improvement of quality of life. Currently, we are trying to wean this patient off diphenhydramine; if successful, this attempt will represent the first reported case. Read More

    Prospective evaluation of the diagnostic value of sensitive KIT D816V mutation analysis of blood in adults with suspected systemic mastocytosis.
    Allergy 2017 Apr 22. Epub 2017 Apr 22.
    Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.
    Background: Sensitive KIT D816V mutation analysis of blood has been proposed to guide bone marrow (BM) investigation in suspected systemic mastocytosis (SM). The aim of this prospective study was for the first time to compare the D816V status of the "screening blood sample" used to guide BM biopsy in suspected SM to the outcome of the subsequent BM investigation.

    Methods: Fifty-eight adult patients with suspected SM were included. Read More

    Response and progression on midostaurin in advanced systemic mastocytosis: KIT D816V and other molecular markers.
    Blood 2017 Apr 19. Epub 2017 Apr 19.
    Department of Hematology and Oncology, University Medical Centre Mannheim, Germany;
    In advanced systemic mastocytosis (advSM), disease evolution is often triggered by activating KIT mutations (D816V in >80% of cases) and by additional mutations, e.g. in SRSF2, ASXL1 and/or RUNX1 (S/A/R(pos), >60% of cases). Read More

    A retrospective epidemiological study of skin diseases among pediatric population attending a tertiary dermatology referral center in Northern Greece.
    Clin Cosmet Investig Dermatol 2017 3;10:99-104. Epub 2017 Apr 3.
    First Department of Dermatology, Aristotle University Medical School, Thessaloniki, Greece.
    Background: The incidence of skin diseases in children is influenced by hereditary, social, and environmental factors. The objective of this study was to determine the incidence of pediatric dermatoses at a University Hospital in Northern Greece.

    Patients And Methods: We reviewed epidemiologic data of 940 patients, aged 0-18 years, who were referred to the outpatient clinic of a University Hospital between January 2013 and December 2015. Read More

    The IL-31/IL-31 receptor axis: general features and role in tumor microenvironment.
    J Leukoc Biol 2017 Apr 13. Epub 2017 Apr 13.
    Immunology Area, Ospedale Pediatrico Bambino Gesù, Roma, Italy
    IL-31 is a recently identified cytokine with a well-defined role in the pathogenesis of pruritus. IL-31, whose production is induced by IL-4 and IL-33, binds a heterodimeric receptor (R) composed of the exclusive IL-31RA chain and the shared oncostatin M R. Signaling through the IL-31R involves the MAPK, PI3K/AKT and Jak/STAT pathways. Read More

    Mutational profiling in the peripheral blood leukocytes of patients with systemic mast cell activation syndrome using next-generation sequencing.
    Immunogenetics 2017 Jun 6;69(6):359-369. Epub 2017 Apr 6.
    Institute of Human Genetics, University Hospital of Bonn, D-53127, Bonn, Germany.
    Mast cell activation syndrome (MCAS) and systemic mastocytosis (SM) are two clinical systemic mast cell activation disease variants. Few studies to date have investigated the genetic basis of MCAS. The present study had two aims. Read More

    Constitutively active ABL family kinases, TEL/ABL and TEL/ARG, harbor distinct leukemogenic activities in vivo.
    Leukemia 2017 May 5. Epub 2017 May 5.
    Department of Molecular Diagnostics and Therapeutics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan.
    ABL (ABL1) and ARG (ABL2) are highly homologous to each other in overall domain structure and amino-acid sequence, with the exception of their C termini. As with ABL, translocations that fuse ARG to ETV6/TEL have been identified in patients with leukemia. To assess the in vivo leukemogenic activity of constitutively active ABL and ARG, we generated a bone marrow (BM) transplantation model using the chimeric forms TEL/ABL and TEL/ARG, which have comparable kinase activities. Read More

    Successful targeted treatment of mast cell activation syndrome with tofacitinib.
    Eur J Haematol 2017 Apr 6. Epub 2017 Apr 6.
    Inflammatory Bowel and Celiac Disease Program, University of Florida, Gainesville, FL, USA.
    Mast cell (MC) activation syndrome (MCAS) is a collection of illnesses of inappropriate MC activation with little to no neoplastic MC proliferation, distinguishing it from mastocytosis. MCAS presents as chronic, generally inflammatory multisystem polymorbidity likely driven in most by heterogeneous patterns of constitutively activating mutations in MC regulatory elements, posing challenges for identifying optimal mutation-targeted treatment in individual patients. Targeting commonly affected downstream effectors may yield clinical benefit independent of upstream mutational profile. Read More

    In-Utero Presentation of Aggressive Systemic Mastocytosis in a Neonate.
    Br J Dermatol 2017 Mar 30. Epub 2017 Mar 30.
    Department of Dermatology, State University of New York Downstate Medical Center, Brooklyn, NY.
    Mastocytosis is a clinically heterogenous disease characterized by mast cell hyperplasia in skin, bone marrow, and/or visceral organs. Cutaneous mastocytosis (CM) is more frequently observed in children, while indolent systemic mastocytosis (ISM) is more commonly observed in adults. Aggressive systemic presentation, particularly, of the neonate, is exceptionally rare. Read More

    Future Prospects of Biologic Therapies for Immunologic Diseases.
    Immunol Allergy Clin North Am 2017 May;37(2):431-448
    Division of Allergy and Immunology, Children's Hospital of Richmond, Virginia Commonwealth University, CHoR Pavilion, 5th Floor, 1000 East Broad Street, Richmond, VA 23298-0225, USA.
    This article presents an overview of future uses for biologic therapies in the treatment of immunologic and allergic conditions. Discussion is centered on the use of existing therapies outside of their current indication or on new therapies that are close to approval. This information may help familiarize practicing allergists and immunologists with therapies they may soon encounter in their practice as well as help identify conditions and treatments that will require further study in the near future. Read More

    Risk Factor Analysis of Anaphylactic Reactions in Patients With Systemic Mastocytosis.
    J Allergy Clin Immunol Pract 2017 Mar 25. Epub 2017 Mar 25.
    Mastocytosis Centre at Brigham and Women's Hospital, Harvard Medical School, Boston, Mass.
    Background: Systemic mastocytosis (SM) is a rare disorder of abnormal mast cells in at least 1 extracutaneous organ/tissue. Anaphylaxis is an acute, severe systemic hypersensitivity reaction, and a strong association between SM and anaphylaxis has been shown. However, not all patients with SM experience anaphylaxis. Read More

    CEACAM1 long isoform has opposite effects on the growth of human mastocytosis and medullary thyroid carcinoma cells.
    Cancer Med 2017 Apr 23;6(4):845-856. Epub 2017 Mar 23.
    Department of Diagnostic Pathology, Kyoto University, Kyoto, Japan.
    Carcinoembryonic antigen-related cell adhesion molecule 1 (CEACAM1) is expressed in a number of tumor cell types. The immunoreceptor tyrosine-based inhibitory motif (ITIM)-containing isoforms of this molecule which possess a long cytoplasmic tail (CEACAM1-L) generally play inhibitory roles in cell function by interacting with Src homology 2 domain-containing tyrosine phosphatase (SHP)-1 and/or SHP-2. Src family kinases (SFKs) are also known to bind to and phosphorylate CEACAM1-L isoforms. Read More

    Cutaneous Mastocytosis With Atypical Mast Cells in a 7-Year-Old Girl.
    Am J Dermatopathol 2017 Apr;39(4):310-312
    *Department of Dermatology, Complejo Hospitalario Universitario Insular Materno-Infantil, Universidad de Las Palmas de Gran Canaria, Las Palmas de Gran Canaria, Spain;†Department of Pathology and Dermatology, Wake Forest Baptist Health, Winston-Salem, NC; and‡Department of Pathology, Complejo Hospitalario Universitario Insular Materno-Infantil, Las Palmas de Gran Canaria, Spain.
    Cutaneous mastocytosis is defined by the presence of mast cells within the skin in the absence of other criteria for the diagnosis of systemic mastocytosis. Mast cells are characterized by an abundant granular cytoplasm and a round to oval or spindle-shaped nuclei. The presence of mast cells with bilobed and multilobed nuclei in cutaneous mastocytosis is a rare phenomenon and has been rarely reported in the literature. Read More

    Systemic Mastocytosis Complicated by Non-Cirrhotic Portal Hypertension and Variceal Bleeding.
    ACG Case Rep J 2017 1;4:e30. Epub 2017 Mar 1.
    Section of Digestive Diseases, Yale University School of Medicine, New Haven, CT.
    Systemic mastocytosis is a myeloproliferative disorder characterized by extracutaneous involvement of at least one organ. Although rare, infiltration of inflammatory mast cells within the portal vein may lead to obstruction of the sinusoids resulting in non-cirrhotic portal hypertension. We present a patient with known history of systemic mastocytosis with bone marrow involvement presenting with new-onset esophageal variceal bleeding. Read More

    Characterization of Mast Cell Activation Syndrome.
    Am J Med Sci 2017 Mar 16;353(3):207-215. Epub 2016 Dec 16.
    Department of Pathology and Laboratory Medicine, Medical University of South Carolina, Charleston, South Carolina.
    Background: Mast cell activation syndrome (MCAS), a recently recognized nonneoplastic mast cell disease driving chronic multisystem inflammation and allergy, appears prevalent and thus important. We report the first systematic characterization of a large MCAS population.

    Method: Demographics, comorbidities, symptoms, family histories, physical examination and laboratory findings were reviewed in 298 retrospective and 115 prospective patients with MCAS. Read More

    Highly sensitive assays are mandatory for the differential diagnosis of patients presenting with symptoms of mast cell activation: diagnostic work-up of 38 patients.
    Acta Clin Belg 2017 Apr 6;72(2):123-129. Epub 2017 Mar 6.
    a Department of Laboratory Medicine , University Hospitals Leuven , Leuven , Belgium.
    Mastocytosis is a heterogeneous disease caused by excessive mast cell (MC) proliferation. Diagnosis of systemic mastocytosis (SM) is based on the presence of major and minor criteria defined by the World Health Organization. Symptoms of MC activation can also occur in patients without SM or without allergic or inflammatory disease. Read More

    Assessment of in vivo mast cell reactivity in patients with systemic mastocytosis.
    Clin Exp Allergy 2017 Mar 4. Epub 2017 Mar 4.
    Mastocytosis Centre Karolinska, Karolinska University Hospital and Karolinska Institutet, Stockholm, Sweden.
    Background: Patients with systemic mastocytosis (SM) have clinical signs of mast cell (MC) activation and increased levels of MC mediators. It is unclear whether the increased mediator levels are caused by increased numbers of tissue MCs, or whether these cells in affected individuals have a hyperactive phenotype.

    Objective: To determine reactivity of the skin and the airways to directly acting mediators and indirectly acting mast cell secretagogues in subjects with SM. Read More

    The clinical and molecular diversity of mast cell leukemia with or without associated hematologic neoplasm.
    Haematologica 2017 Mar 2. Epub 2017 Mar 2.
    Medical Faculty Mannheim, Heidelberg University, Germany;
    Mast cell leukemia is a rare variant of advanced systemic mastocytosis characterized by ≥20% mast cells in a bone marrow smear. We evaluated clinical and molecular characteristics of 28 patients with (n=20, 71%) or without an associated hematologic neoplasm. De novo mast cell leukemia was diagnosed in 16/28 (57%) patients and secondary mast cell leukemia evolving from other advanced systemic mastocytosis subtypes in 12/28 (43%) patients, of which 7 patients progressed while on cytoreductive treatment. Read More

    Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future.
    Cancer Res 2017 Mar 2;77(6):1261-1270. Epub 2017 Mar 2.
    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, Maryland.
    Mastocytosis is a term used to denote a heterogeneous group of conditions defined by the expansion and accumulation of clonal (neoplastic) tissue mast cells in various organs. The classification of the World Health Organization (WHO) divides the disease into cutaneous mastocytosis, systemic mastocytosis, and localized mast cell tumors. On the basis of histomorphologic criteria, clinical parameters, and organ involvement, systemic mastocytosis is further divided into indolent systemic mastocytosis and advanced systemic mastocytosis variants, including aggressive systemic mastocytosis and mast cell leukemia. Read More

    Significance of cytological smear evaluation in diagnosis of splenic mast cell tumor-associated systemic mastocytosis in a cat (Felis catus).
    Can Vet J 2017 Mar;58(3):293-295
    University of Georgia, College of Veterinary Medicine, Veterinary Diagnostic and Investigational Laboratory, 43 Brighton Road, Tifton, Georgia 31793 (Woldemeskel, Merrill); Plantation Center Animal Hospital, 6411 Peake Road, Macon, Georgia 31210, USA (Brown).
    An 8-year-old cat was presented with vomiting and weight loss. Histopathology and cytology revealed systemic mastocytosis, a rare condition and a clinical challenge. This case emphasizes the significance of cytological evaluation of smears in diagnosis of mastocytosis and in confirmation in biopsy specimens. Read More

    Systemic Mastocytosis, Kounis Syndrome and Coronary Intervention: Case Report and Systematic Review.
    Heart Lung Circ 2017 Feb 15. Epub 2017 Feb 15.
    Department of Cardiology, St Vincent's Hospital Melbourne, Melbourne, Vic, Australia.
    A 72-year-old male reported a long-standing history of unexplained syncope. Stress echocardiography demonstrated inducible anterior hypokinesis, and he proceeded to percutaneous coronary intervention for an 80% stenosis of the left anterior descending artery. Thirty minutes post-procedure, he experienced a pulseless electrical activity (PEA) cardiac arrest. Read More

    American Academy of Asthma, Allergy & Immunology membership experience with venom immunotherapy in chronic medical conditions and pregnancy, and in young children.
    Allergy Asthma Proc 2017 Mar;38(2):121-129
    Background: Few data exist regarding the use of venom immunotherapy (VIT) in specific high-risk chronic medical conditions and pregnancy, and in young children.

    Methods: A Web-based survey was sent to American Academy of Asthma Allergy & Immunology members to explore their VIT experience in potential high-risk medical conditions and pregnancy, and in young children. Major problems were defined as "activation of underlying disease and/or VIT not well tolerated (systemic adverse events) and/or VIT discontinued for medical reasons. Read More

    Denosumab for the Treatment of Mastocytosis-Related Osteoporosis: A Case Series.
    Calcif Tissue Int 2017 Jun 22;100(6):595-598. Epub 2017 Feb 22.
    Rheumatology Section, Department of Medicine, University of Verona, Piazzale L. Scuro 10, 37134, Verona, Italy.
    The purpose of this study was to investigate the therapeutic effect of denosumab, an anti-RANKL monoclonal antibody for the treatment of bone loss in indolent systemic mastocytosis (ISM) patients intolerant to bisphosphonates. Four patients underwent upon informed consent a treatment with denosumab 60 mg administered subcutaneously every 6 months with the same regimen used for postmenopausal osteoporosis. Bone mineral density (BMD) was measured at lumbar and femoral sites at baseline and after 1 year. Read More

    Key Issues in Hymenoptera Venom Allergy: An Update.
    J Investig Allergol Clin Immunol 2017;27(1):19-31
    Hospital Universitario Clínico San Carlos, Madrid, Spain.
    In this review, the Hymenoptera Allergy Committee of the SEAIC analyzes the most recent scientific literature addressing problems related to the diagnosis of hymenoptera allergy and to management of venom immunotherapy. Molecular diagnosis and molecular risk profiles are the key areas addressed. The appearance of new species of hymenoptera that are potentially allergenic in Spain and the associated diagnostic and therapeutic problems are also described. Read More

    Mastering gut permeability: New roles for old friends.
    Eur J Immunol 2017 Feb;47(2):236-239
    Infection and Immunity Program, Department of Biochemistry and Molecular Biology, Monash Biomedicine Discovery Institute, Monash University, Clayton, VIC, Australia.
    Mast cells are innate immune cells that respond rapidly to infection in barrier tissues such as the skin and intestinal mucosa. Expulsion of parasitic worms in the gut involves a robust type 2 host response, and an acute mastocytosis is often generated at the site of infection. However, the role of mast cells in resistance to worm infections appears to be parasite specific. Read More

    Kounis Syndrome During Anesthesia: Presentation of Indolent Systemic Mastocytosis: A Case Report.
    A A Case Rep 2017 May;8(9):226-228
    From the Departments of *Anesthesiology, †Allergy, and §Cardiology, Hospital Universitario de Guadalajara, Castilla-La Mancha, Spain; ‡Department of Surgery, Hospital Universitario Quirónsalud Madrid, Madrid, Spain.
    Mastocytosis comprises a heterogeneous group of disorders characterized by mast cell accumulation and proliferation in distinct organs. Kounis syndrome is defined as the concurrence of acute coronary syndromes with mast cell activation in a setting of allergic or hypersensitivity reactions. This is the first reported case of an intraoperative Kounis syndrome as the onset of an indolent systemic mastocytosis probably triggered by succinylated gelatin infusion during general anesthesia. Read More

    Successful Management of a Patient With Possible Mast Cell Activation Syndrome Undergoing Pulmonary Embolectomy: A Case Report.
    A A Case Rep 2017 May;8(9):232-234
    From the Departments of *Anesthesiology and †Critical Care, Emory University, Atlanta, Georgia.
    We report the successful perioperative management of a patient with presumed mastocytosis undergoing pulmonary embolectomy. Postoperatively the patient went into vasodilatory shock, which was partly attributed to mast cell mediator release. H1- and H2-antagonists, steroids, and a single dose of methylene blue were given with improvement of hemodynamics. Read More

    Treatment outcomes and prognostic factors of feline splenic mast cell tumors: A multi-institutional retrospective study of 64 cases.
    Vet Comp Oncol 2017 Feb 7. Epub 2017 Feb 7.
    School of Veterinary Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
    Background: Mast cell tumors (MCT) are common splenic tumors in cats, but there is limited information on treatment outcomes of cats with this disease.

    Materials And Methods: This retrospective study evaluated treatment outcomes in 64 cats with splenic MCT. Cats were categorized into the following treatment groups: splenectomy (A, n = 20); splenectomy with chemotherapy (B, n = 20); chemotherapy alone (C, n = 15); or supportive care (D, n = 9). Read More

    Pathogenesis and Pathology of Mastocytosis.
    Annu Rev Pathol 2017 Jan;12:487-514
    Tel Hai College, Upper Galilee, 1220800 Israel; email:
    Systemic mastocytosis is a clonal disorder of mast cells that may variably present with characteristic skin lesions, episodes of mast cell mediator release, and disturbances of hematopoiesis. No curative therapy presently exists. Conventional management has relied on agents that antagonize mediators released by mast cells, inhibit mediator secretion, or modulate mast cell proliferation. Read More

    Longitudinal Study of Pediatric Urticaria Pigmentosa.
    Pediatr Dermatol 2017 Mar 30;34(2):144-149. Epub 2017 Jan 30.
    Department of Dermatology, Mayo Clinic Health System, Eau Claire, Wisconsin.
    Background/objectives: Urticaria pigmentosa (UP) is the most common form of mastocytosis in children and is associated with systemic signs, symptoms, and triggers. To our knowledge, the effect of UP on children's quality of life has not been studied. The objective of the current study was to characterize the natural history, triggers, and complications of pediatric UP, identify prognostic indicators, and determine its effect on quality of life. Read More

    Nerve growth factor: a neuroimmune crosstalk mediator for all seasons.
    Immunology 2017 May 21;151(1):1-15. Epub 2017 Feb 21.
    Department of Pharmaceutical and Pharmacological Sciences, University of Padua, Padua, Italy.
    Neurotrophic factors comprise a broad family of biomolecules - most of which are peptides or small proteins - that support the growth, survival and differentiation of both developing and mature neurons. The prototypical example and best-characterized neurotrophic factor is nerve growth factor (NGF), which is widely recognized as a target-derived factor responsible for the survival and maintenance of the phenotype of specific subsets of peripheral neurons and basal forebrain cholinergic nuclei during development and maturation. In addition to being active in a wide array of non-nervous system cells, NGF is also synthesized by a range of cell types not considered as classical targets for innervation by NGF-dependent neurons; these include cells of the immune-haematopoietic lineage and populations in the brain involved in neuroendocrine functions. Read More

    IgE-Related Chronic Diseases and Anti-IgE-Based Treatments.
    J Immunol Res 2016 21;2016:8163803. Epub 2016 Dec 21.
    Biochemistry Unit, Biomedicine Department, Faculty of Medicine, University of Barcelona, Casanova 143, 08036 Barcelona, Spain.
    IgE is an immunoglobulin that plays a central role in acute allergic reactions and chronic inflammatory allergic diseases. The development of a drug able to neutralize this antibody represents a breakthrough in the treatment of inflammatory pathologies with a probable allergic basis. This review focuses on IgE-related chronic diseases, such as allergic asthma and chronic urticaria (CU), and on the role of the anti-IgE monoclonal antibody, omalizumab, in their treatment. Read More

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