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    Crenolanib is a type I tyrosine kinase inhibitor that inhibits mutant KIT D816 isoforms prevalent in systemic mastocytosis and core binding factor leukemia.
    Oncotarget 2017 Oct 7;8(47):82897-82909. Epub 2017 Aug 7.
    University Hospital Tübingen, Department of Oncology, Hematology, Rheumatology, Clinical Immunology and Pulmology, Tübingen, Germany.
    Activating D816 mutations of the class III receptor tyrosine kinase KIT are associated with the majority of patients with systemic mastocytosis (SM), but also core binding factor (CBF) AML, making KIT mutations attractive therapeutic targets for the treatment of these cancers. Crenolanib is a potent and selective inhibitor of wild-type as well as mutant isoforms of the class III receptor tyrosine kinases FLT3 and PDGFRα/β. Notably, crenolanib inhibits constitutively active mutant-FLT3 isoforms resulting from amino acid substitutions of aspartic acid at codon 835, which is homologous to codon 816 in the KIT gene - suggesting sensitivity against mutant-KIT D816 isoforms as well. Read More

    Histamine-releasing factor enhances food allergy.
    J Clin Invest 2017 Nov 13. Epub 2017 Nov 13.
    Division of Cell Biology, La Jolla Institute for Allergy and Immunology, La Jolla, California, USA.
    Food allergy occurs due to IgE- and mast cell-dependent intestinal inflammation. Previously, we showed that histamine-releasing factor (HRF), a multifunctional protein secreted during allergy, interacts with a subset of IgE molecules and that the HRF dimer activates mast cells in an HRF-reactive IgE-dependent manner. In this study, we investigated whether HRF plays any role in food allergy. Read More

    Mast Cell Disease Assessment by Flow Cytometric Analysis.
    Clin Lab Med 2017 Dec 10;37(4):869-878. Epub 2017 Oct 10.
    Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA. Electronic address:
    Mast cells are present at a low frequency in bone marrow, rendering high-sensitivity multiparametric flow cytometric analysis an ideal method to assess antigen expression on mast cells. This article discusses the normal antigen expression profile of mast cells, established criteria to identify neoplastic mast cells, and new immunophenotypic markers and approaches to identify the presence of neoplastic mast cells in cases of mastocytosis. Read More

    Simultaneous physiologically-based pharmacokinetic (PBPK) modeling of parent and active metabolites to investigate complex CYP3A4 drug-drug interaction potential: a case example of midostaurin.
    Drug Metab Dispos 2017 Nov 8. Epub 2017 Nov 8.
    Novartis Institutes for Biomedical Research.
    Midostaurin (PKC412) is being investigated for the treatment of acute myeloid leukemia (AML) and advanced systemic mastocytosis (advSM). It is extensively metabolized by cytochrome P450 (CYP) 3A4 to form 2 major active metabolites, CGP52421 and CGP62221. In vitro and clinical drug-drug interaction (DDI) studies indicated that midostaurin and its metabolites are substrates, reversible and time-dependent inhibitors, and inducers of CYP3A4. Read More

    FGFR1 translocation with concurrent myeloproliferative neoplasm, systemic mastocytosis, and lymphoblastic lymphoma: a case report.
    Hum Pathol 2017 Oct 28. Epub 2017 Oct 28.
    Department of Pathology, National Taiwan University Hospital, National Taiwan University College of Medicine, No.7, South Chung-Shan Rd., Taipei, Taiwan, 100; Graduate Institute of Pathology, National Taiwan University College of Medicine, No.1, Section 1, Jen Ai Road, Taipei, Taiwan,100. Electronic address:
    FGFR1 translocation may cause myeloid or lymphoid neoplasm but rarely systemic mastocytosis (SM). Conversely, SM is associated with myeloproliferative neoplasm (MPN), but rarely lymphoblastic lymphoma (LBL) or FGFR1 translocation. We report the first case of FGFR1 translocation in a patient with concurrent LBL, MPN, and SM. Read More

    A precision therapy against cancers driven by KIT/PDGFRA mutations.
    Sci Transl Med 2017 Nov;9(414)
    Blueprint Medicines, Cambridge, MA 02139, USA.
    Targeting oncogenic kinase drivers with small-molecule inhibitors can have marked therapeutic benefit, especially when administered to an appropriate genomically defined patient population. Cancer genomics and mechanistic studies have revealed that heterogeneous mutations within a single kinase can result in various mechanisms of kinase activation. Therapeutic benefit to patients can best be optimized through an in-depth understanding of the disease-driving mutations combined with the ability to match these insights to tailored highly selective drugs. Read More

    Activation of TRKA receptor elicits mastocytosis in mice and is involved in the development of resistance to KIT-targeted therapy.
    Oncotarget 2017 Sep 19;8(43):73871-73883. Epub 2017 May 19.
    Department of Hematology, Hemostasis, Oncology, and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany.
    The neurotrophins (NTs) play a key role in neuronal survival and maintenance. The TRK (tropomyosin-related kinase) tyrosine kinase receptors (TRKA, TRKB, TRKC) are high affinity receptors for NTs. There is increasing data demonstrating an important role of the TRK family in cancer initiation and progression. Read More

    Digital quantitative analysis of mast cell infiltration in interstitial cystitis.
    Neurourol Urodyn 2017 Oct 24. Epub 2017 Oct 24.
    Department of Urology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
    Aims: To evaluate the significance of mast cell infiltration in interstitial cystitis (IC) by comparison with equally inflamed controls using a digital quantification technique.

    Methods: Bladder biopsy specimens from 31 patients with Hunner type IC and 38 patients with non-Hunner type IC were analyzed. Bladder biopsy specimens from 37 patients without IC, including 19 non-specific chronic cystitis ("non-IC cystitis") specimens and 18 non-inflamed bladder ("normal bladder") specimens, were used as controls. Read More

    Midostaurin: a novel therapeutic agent for patients with FLT3-mutated acute myeloid leukemia and systemic mastocytosis.
    Ther Adv Hematol 2017 Sep 19;8(9):245-261. Epub 2017 Aug 19.
    Department of Medicine, University Hospitals Cleveland Medical Center, 11100 Euclid Avenue, Cleveland, OH 44106, USA.
    The development of FLT3-targeted inhibitors represents an important paradigm shift in the management of patients with highly aggressive fms-like tyrosine kinase 3-mutated (FLT3-mut) acute myeloid leukemia (AML). Midostaurin is an orally administered type III tyrosine kinase inhibitor which in addition to FLT3 inhibits c-kit, platelet-derived growth factor receptors, src, and vascular endothelial growth factor receptor. Midostaurin is the first FLT3 inhibitor that has been shown to significantly improve survival in younger patients with FLT3-mut AML when given in combination with standard cytotoxic chemotherapy based on the recently completed RATIFY study. Read More

    Hepatic Mastocytosis in Japanese Black Cattle.
    Vet Pathol 2017 Jan 1:300985817736115. Epub 2017 Jan 1.
    1 Department of Histopathology, Diagnostic Animal Pathology Office, Hokkaido, Japan.
    In 5 Japanese Black steers (2-2.4 years old) that originated from 5 different feedlots, the livers were found at slaughter to have multiple nodular or cordlike lesions (5 steers) and an extensive fibrotic area (1 steer). Microscopic changes included extensive fibroplasia in the portal tracts and chronic proliferative endophlebitis-like lesions confined to the portal vein branches. Read More

    How we diagnose and treat systemic mastocytosis in adults.
    Br J Haematol 2017 Oct 19. Epub 2017 Oct 19.
    Department of Hematology and Oncology, Oregon Health and Science University, Portland, OR, USA.
    Rapid advances in the understanding of the molecular biology, data from translational and clinical trials, and retrospective analyses has influenced the diagnosis and treatment of systemic mastocytosis (SM). Many options have existed for the symptomatic management of SM patients, but recent evolution in regards to the molecular underpinnings of this disease and our ability to distinguish clonal mastocytosis from mast cell activation syndrome has changed our treatment paradigm and opened new opportunities for understanding genetic risk, transformation to mast cell leukaemia, and treatment choices. Key to this change has been the discovery of the KIT mutation and the use of next generation sequencing to evaluate for co-existing molecular mutations that may define the disease course. Read More

    From the observation of atypical cells on blood smear to the diagnosis of mast cell leukemia: a case report in a 79 year old woman consulting for anemia.
    Ann Biol Clin (Paris) 2017 Oct 18. Epub 2017 Oct 18.
    Laboratoire d'hématologie, Hôpital de Mercy, CHR Metz-Thionville, France.
    Mast cell leukemia is an extremely rare disease, which belongs to the systemic mastocytosis group (WHO 2016). We are reporting the case of a 79-year-old woman, without any hematological particular history consulting for hyperthermia, repeated malaise and subacute anemia. Her clinical examination was normal. Read More

    A novel TRIP11-FLT3 fusion in a patient with a myeloid/lymphoid neoplasm with eosinophilia.
    Cancer Genet 2017 Oct 10;216-217:10-15. Epub 2017 May 10.
    Department of Pathology, Stanford University School of Medicine, Stanford, CA 94305, USA. Electronic address:
    FLT3 fusions are associated with myeloid and lymphoid neoplasms with eosinophilia. We describe a patient presenting with clinicopathologic features of both chronic eosinophilic leukemia, not otherwise specified (CEL, NOS) and systemic mastocytosis (SM). The bone marrow demonstrated a myeloproliferative neoplasm with eosinophilia and aggregates of atypical mast cells. Read More

    Mastocytosis in Children.
    Curr Allergy Asthma Rep 2017 Oct 7;17(11):80. Epub 2017 Oct 7.
    Department of Pediatrics, Division of Allergy and Immunology, Virginia Commonwealth University Health Systems, 1000 E Broad Street, Richmond, VA, 23219, USA.
    Purpose Of Review: In this review, we examine the current understanding of the pathogenesis, clinical presentations, diagnostic tools, and treatment options of pediatric mastocytosis as well as the natural history of the disease.

    Recent Findings: We discuss the emerging concept of mast cell activation syndrome. Mastocytosis in children presents most commonly as isolated cutaneous lesions and is a relatively rare occurrence with excellent prognosis and spontaneous regression often occurring by adolescence. Read More

    MANAGEMENT OF ENDOCRINE DISEASE: Flushing: current concepts.
    Eur J Endocrinol 2017 Nov;177(5):R219-R229
    Department of EndocrinologyOxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, and NET Centre of Excellence, Royal Free London, UK.
    Objective: Flushing can be defined as a sensation of warmth accompanied by erythema that most commonly is seen on the face and which occurs in episodic attacks. Such a problem can be clinically problematic, since many conditions and drugs can be related to flushing, and while often there appears to be no underlying organic disease, it is important to exclude disorders since they may be life-threatening conditions.

    Design And Methods: We performed a search in MEDLINE using the terms 'flushing' in combination with 'carcinoid syndrome', 'pheochromocytoma', 'mastocytosis', 'menopausal hot flush' and 'treatment'. Read More

    General Anesthesia for Electroconvulsive Therapy in a Patient With Systemic Mastocytosis.
    J ECT 2017 Oct 3. Epub 2017 Oct 3.
    Departments of Anesthesiology and Psychiatry, The Icahn School of Medicine at Mount Sinai, New York, NY Departments of Psychiatry and Neurology, The Icahn School of Medicine at Mount Sinai, New York, NY Department of Psychiatry, The Icahn School of Medicine at Mount Sinai, New York, NY.

    Systemic Mastocytosis in Association with Small Lymphocytic Lymphoma.
    Am J Case Rep 2017 Oct 3;18:1053-1057. Epub 2017 Oct 3.
    Division of Hematology/Oncology, Simmons Cancer Institute at Southern Illinois University (SIU), Springfield, IL, USA.
    BACKGROUND Systemic mastocytosis with an associated hematologic non-mast cell lineage disease is a rare entity, and the majority of systemic mastocytosis cases are associated with myeloid neoplasm. Lymphoproliferative disorders are less commonly associated with systemic mastocytosis and a few cases of systemic mastocytosis associated with chronic lymphocytic leukemia have been described in the literature. CASE REPORT We present a case of indolent systemic mastocytosis associated with small lymphocytic lymphoma. Read More

    Midostaurin treatment in FLT3-mutated acute myeloid leukemia and systemic mastocytosis.
    Expert Rev Clin Pharmacol 2017 Nov 10;10(11):1177-1189. Epub 2017 Oct 10.
    d National Center for Tumor Diseases , Heidelberg , Germany.
    Introduction: A number of tyrosine kinase inhibitors (TKIs) have been developed that inhibit the constitutively activated kinase activity caused by activating tyrosine kinase mutations, such as FLT3 or KIT, thus interrupting signaling pathways. Currently, midostaurin is the only approved TKI as monotherapy for aggressive systemic mastocytosis (SM), SM with associated hematological neoplasm, or mast cell leukemia displaying a KIT mutation as well as in combination with standard intensive chemotherapy for adult patients with newly diagnosed FLT3-mutated acute myeloid leukemia (AML). Areas covered: We provide a concise review of the pharmacology, tolerability and clinical efficacy of midostaurin and emerging new treatment options for ASM and FLT3-mutated AML. Read More

    The role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experience.
    Postepy Dermatol Alergol 2017 Aug 1;34(4):306-312. Epub 2017 Aug 1.
    Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Gdansk, Poland.
    Introduction: In children, cutaneous mastocytosis (CM) is considered to be a benign disease associated with mast cell mediator-related symptoms. However, systemic mastocytosis (SM) and anaphylaxis may also occur. Since the basal serum tryptase (bsT) level reflects mast cell burden, its determination is recommended in the diagnosis and follow-up. Read More

    Midostaurin: a magic bullet that blocks mast cell expansion and activation.
    Ann Oncol 2017 Oct;28(10):2367-2376
    Stanford University School of Medicine/Stanford Cancer Institute, Stanford, USA.
    Clinically relevant features in patients with systemic mastocytosis (SM) include the cosmetic burden of lesional skin, mediator-related symptoms, and organ damage resulting from mast cell (MC) infiltration in advanced forms of SM. Regardless of the SM variant, expansion of neoplastic MC in the skin and other organs is triggered by mutant forms of KIT, the most prevalent being D816V. Activation of MC with subsequent release of chemical mediators is often caused by IgE-dependent mechanisms in these patients. Read More

    A Practical Guide for Treatment of Pain in Patients with Systemic Mast Cell Activation Disease.
    Pain Physician 2017 Sep;20(6):E849-E861
    Institute of Human Genetics, University Hospital of Bonn, Germany.
    Systemic mast cell activation disease (MCAD, a subclass of mastocytosis), which has a prevalence of around 17% (at least in the German population), is characterized by accumulation of genetically altered dysfunctional mast cells with abnormal release of these cells' mediators. Since mast cells affect functions in potentially every organ system, often without causing abnormalities in routine laboratory or radiologic testing, this disease has to be considered routinely in the differential diagnosis of patients with chronic multisystem polymorbidity of a generally inflammatory and allergic theme. Pain in its different manifestations is a common symptom in MCAD found in more than three-quarters of the MCAD patients. Read More

    Prevalence and risk factors for fragility fracture in systemic mastocytosis.
    Bone 2017 Dec 14;105:219-225. Epub 2017 Sep 14.
    Department of Dermatology, Mastocytosis Expert Center of Midi-Pyrénées, Paul Sabatier University, Toulouse University Hospital, Toulouse, France. Electronic address:
    Objectives: Systemic mastocytosis (SM) is characterized by the accumulation of mast cells in tissues other than the skin. Bone involvement although frequent has not been thoroughly evaluated. Primary objective was to determine risk factors associated with fragility fractures (FF) in SM. Read More

    Siglec-8 as mast cell selective target: developing paradigms amidst inconvenient truths.
    Naunyn Schmiedebergs Arch Pharmacol 2017 Nov 14;390(11):1173-1175. Epub 2017 Sep 14.
    Institute of Human Genetics, University Hospital of Bonn, Sigmund-Freud-Strasse 25, 53127, Bonn, Germany.
    Due to the limited efficacy of current drugs in treating systemic mast cell activation disease, there is an urgent need for more effective drugs selectively acting at mast cells. In the past, a large number of compounds have been claimed to be effective and mast cell selective on the basis of cell culture experiments and studies on blood leukocytes which could not be verified in organ and animal studies. Nevertheless, over time in review papers about potential mast cell targets mast cell selectivity of these targets has been no longer challenged. Read More

    Fatal Anaphylaxis: Mortality Rate and Risk Factors.
    J Allergy Clin Immunol Pract 2017 Sep - Oct;5(5):1169-1178
    Department of Paediatric Allergy, Imperial College London, London, United Kingdom. Electronic address:
    Up to 5% of the US population has suffered anaphylaxis. Fatal outcome is rare, such that even for people with known venom or food allergy, fatal anaphylaxis constitutes less than 1% of total mortality risk. The incidence of fatal anaphylaxis has not increased in line with hospital admissions for anaphylaxis. Read More

    Characterization of midostaurin as a dual inhibitor of FLT3 and SYK and potentiation of FLT3 inhibition against FLT3-ITD-driven leukemia harboring activated SYK kinase.
    Oncotarget 2017 Aug 6;8(32):52026-52044. Epub 2017 Jul 6.
    Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
    Oncogenic FLT3 kinase is a clinically validated target in acute myeloid leukemia (AML), and both multi-targeted and selective FLT3 inhibitors have been developed. Spleen tyrosine kinase (SYK) has been shown to be activated and increased in FLT3-ITD-positive AML patients, and has further been shown to be critical for transformation and maintenance of the leukemic clone in these patients. Further, over-expression of constitutively activated SYK causes resistance to highly selective FLT3 tyrosine kinase inhibitors (TKI). Read More

    Mast Cell Disorders, Melanoma and Pancreatic Carcinoma: From a Clinical Observation to a Brief Review of the Literature.
    Acta Dermatovenerol Croat 2017 Jul;25(2):112-119
    Dario Didona, MD Istituto Dermopatico dell'Immacolata-IRCCS Via dei Monti di Creta 104, 00167 Rome Italy
    Mastocytosis can be associated with other clonal or non-clonal hematologic diseases as well as a variety of non-hematologic malignancies. A 75-year-old Caucasian male patient was referred to us with a 5-month history of neutrophilic leukocytosis and mild splenomegaly. He had developed a cutaneous melanoma sixteen years ago. Read More

    Management around invasive procedures in mastocytosis: An update.
    Ann Allergy Asthma Immunol 2017 Oct 31;119(4):304-309. Epub 2017 Aug 31.
    Department of Internal Medicine, Section of Allergy, Erasmus University Medical Center, Rotterdam, The Netherlands; Department of Internal Medicine, Section of Clinical Immunology, Erasmus University Medical Center, Rotterdam, The Netherlands.
    Objective: Mastocytosis is a chronic hematologic disorder that is characterized by the accumulation of aberrant mast cells and typically involves the skin and/or bone marrow. Patients with mastocytosis are at increased risk of anaphylaxis. Based on theoretical assumptions, medical procedures requiring general anesthesia or radiocontrast media are deemed hazardous for patients with mastocytosis. Read More

    Case Report: Unusual Manifestation of KIT Negative Systemic Mastocytosis.
    Am J Hematol Oncol 2016 Dec;12(12):24-27
    Department of Hematology-Oncology of the VA Caribbean Healthcare System, 10 Calle Casia, San Juan, Puerto Rico 00921.
    Aggressive systemic mastocytosis is a rare hematologic neoplastic disease that presents with a poor prognosis and low survival rate. It typically manifests with symptoms associated to mast cell release of bioactive substances, causing anaphylaxis, flushing, autonomic and hemodynamic instability, gastric distress and headache. Moreover, more than 95% of cases are related to a mutation in codon 816 of the KIT gene, located on human chromosome 4q12 which codes for a type III receptor tyrosine kinase. Read More

    Oesophageal mastocytosis: eosinophilic oesophagitis without eosinophils?
    BMJ Case Rep 2017 Aug 28;2017. Epub 2017 Aug 28.
    Division of Gastroenterology, Hepatology and Nutrition, College of Medicine, University of Florida, Gainesville, Florida, USA.
    A 59-year-old male with a history of lifelong asthma, allergic rhinitis and hypercholesterolaemia presented to the emergency department for management of severe substernal chest pain with radiating pain to his left arm, nausea and diaphoresis. Physical examination was unrevealing and a cardiac workup including cardiac enzymes, ECG, chest radiographs were negative for an underlying ischaemic event. A subsequent gastrointestinal workup including oesophageal manometry and oesophagogastroduodenoscopy revealed elevated lower oesophageal pressures and histopathology suggestive of mast cell proliferation, respectively. Read More

    Mast cell inhibition as a therapeutic approach in fibrodysplasia ossificans progressiva (FOP).
    Bone 2017 Aug 26. Epub 2017 Aug 26.
    Department of Medicine, Mayo Clinic School of Medicine, Mayo Clinic, Rochester, MN, United States. Electronic address:
    Background: Episodic flare-ups of fibrodysplasia ossificans progressiva (FOP) are characterized clinically by severe, often posttraumatic, connective tissue swelling and intramuscular edema, followed histologically by an intense and highly angiogenic fibroproliferative reaction. This early inflammatory and angiogenic fibroproliferative response is accompanied by the presence of abundant mast cells far in excess of other reported myopathies.

    Results: Using an injury-induced, constitutively-active transgenic mouse model of FOP we show that mast cell inhibition by cromolyn, but not aprepitant, results in a dramatic reduction of heterotopic ossification. Read More

    Beyond IgE-When Do IgE-Crosslinking and Effector Cell Activation Lead to Clinical Anaphylaxis?
    Front Immunol 2017 10;8:871. Epub 2017 Aug 10.
    Allergy Clinic, Copenhagen University Hospital at Gentofte, Hellerup, Denmark.
    Anaphylaxis in humans is inherently difficult to study due to the acuteness of symptoms and the lack of biomarkers serving as risk predictors. Most cases are related to IgE sensitizations to foods, insect venoms, and drugs with mastocytosis patients forming a smaller risk group. However, identifying the relatively small fraction of persons at risk has been exceedingly difficult. Read More

    Etiologies and management of cutaneous flushing: Malignant causes.
    J Am Acad Dermatol 2017 Sep;77(3):405-414
    Department of Dermatology, Tulane University, New Orleans, Louisiana.
    The second article in this 2-part continuing medical education series reviews the following malignant causes of flushing: mastocytosis, medullary thyroid carcinoma, pheochromocytoma, carcinoid tumors, gastroenteropancreatic neuroendocrine tumors, bronchogenic carcinoma, vasointestinal polypeptide secreting tumors, and renal cell carcinoma. The information provided will allow physicians to better distinguish patients who have worrisome presentations that require a more thorough investigation. Appropriate diagnostic workup and treatment options for these malignancies are reviewed. Read More

    Hymenoptera-induced anaphylaxis: is it a mast cell driven hematological disorder?
    Curr Opin Allergy Clin Immunol 2017 Oct;17(5):356-362
    aDepartment of Dermatology and Allergology, Technical University of Munich, Munich, Germany bDivision of Allergy and Clinical Immunology, Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA.
    Purpose Of Review: Hymenoptera-induced allergy (HVA) is a common cause of anaphylaxis and may be fatal. It is associated with systemic mastocytosis in about 7% of adult patients. Systemic mastocytosis is a proliferative disorder of hematopoietic mast cell progenitors. Read More

    Bidirectional Mast Cell-Eosinophil Interactions in Inflammatory Disorders and Cancer.
    Front Med (Lausanne) 2017 24;4:103. Epub 2017 Jul 24.
    Department of Translational Medical Sciences (DiSMeT), Center for Basic and Clinical Immunology Research (CISI), University of Naples Federico II, Naples, Italy.
    Human mast cells (MCs) and eosinophils were first described and named by Paul Ehrlich. These cells have distinct myeloid progenitors and differ morphologically, ultrastructurally, immunologically, biochemically, and pharmacologically. However, MCs and eosinophils play a pivotal role in several allergic disorders. Read More

    Neuroimaging evidence of brain abnormalities in mastocytosis.
    Transl Psychiatry 2017 Aug 8;7(8):e1197. Epub 2017 Aug 8.
    Centre de Référence des Mastocytoses, Université Paris Descartes, Sorbonne, Paris Cité, Hôpital Necker-Enfants Malades, Fondation Imagine, Paris, France.
    Mastocytosis is a rare disease in which chronic symptoms are related to mast cell accumulation and activation. Patients can display depression-anxiety-like symptoms and cognitive impairment. The pathophysiology of these symptoms may be associated with tissular mast cell infiltration, mast cell mediator release or both. Read More

    Mast cell activation syndromes.
    J Allergy Clin Immunol 2017 Aug;140(2):349-355
    Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, Mich. Electronic address:
    Mast cell activation is common and possibly necessary for maintenance of survival. Disordered mast cell activation occurs when mast cells are pathologically overproduced or if their activation is out of proportion to the perceived threat to homeostasis. Mast cell activation syndrome refers to a group of disorders with diverse causes presenting with episodic multisystem symptoms as the result of mast cell mediator release. Read More

    Neurobiological Basis of Hypersomnia.
    Sleep Med Clin 2017 Sep 7;12(3):265-277. Epub 2017 Jun 7.
    International Institute of Integrative Sleep Medicine, Tsukuba University, 1-1-1 Tennodai, Tsukuba, Ibaraki 305-8575, Japan. Electronic address:
    Narcolepsy is the most well-characterized hypersomnia in both clinical and basic research fields. Narcolepsy is caused by degeneration of hypocretin-producing neurons in the hypothalamus. Although hypocretin receptor antagonists have been developed as sleep-inducing drugs, a high dose of suvorexant, a hypocretin receptor antagonist, inhibits gene expression of prepro-hypocretin to induce narcoleptic attack in wild-type mice. Read More

    Targeted Treatment Options in Mastocytosis.
    Front Med (Lausanne) 2017 20;4:110. Epub 2017 Jul 20.
    French Reference Center for Mastocytosis (CEREMAST), Department of Hematology, Necker Children's Hospital, APHP, Paris, France.
    Mastocytosis refers to a heterogeneous group of disorders resulting from the clonal proliferation of abnormal mast cells and their accumulation in the skin (cutaneous mastocytosis when only in the skin, CM) or in various organs (systemic mastocytosis, SM). This leads to a wide variety of clinical manifestations resulting from excessive mediator release in CM and benign forms of SM (indolent SM, ISM) and from tissue mast cell infiltration causing multiorgan dysfunction and failure in more aggressive subtypes (aggressive SM, ASM, or mast cell leukemia). In addition, SM may be associated with hematological neoplasms (AHN). Read More

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