7,063 results match your criteria Mastocytosis


Pseudoxanthomatous or xanthelasmoid mastocytosis: Reporting a rare entity.

Indian J Dermatol Venereol Leprol 2019 Feb 15. Epub 2019 Feb 15.

Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India.

Mastocytosis is a disease characterized by abnormal and pathologic increase in mast cells in the cutaneous tissue and extracutaneous organs such as the bone marrow, liver, spleen, lymph node and gastrointestinal tract. Cutaneous mastocytosis comprises of four major clinical variants: solitary and multiple mastocytomas, urticaria pigmentosa, diffuse cutaneous mastocytosis and telangiectasia macularis eruptiva perstans. Cutaneous mastocytosis of the xanthelasmoid type is a rare variant of diffuse mastocytosis. Read More

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http://dx.doi.org/10.4103/ijdvl.IJDVL_633_17DOI Listing
February 2019

A case of childhood-onset cutaneous mastocytosis with loss of wild-type KIT allele.

J Eur Acad Dermatol Venereol 2019 Feb 17. Epub 2019 Feb 17.

Department of Dermatology, Sapporo Medical University School of Medicine, Sapporo, Japan.

Childhood-onset cutaneous mastocytosis (CM) have been regarded as a clonal disease similar to adult-onset systemic mastocytosis since the discovery of somatic KIT mutations in pediatric patients. To our knowledge, all mutations previously detected in CM have been heterozygous. Here we report a case of pediatric CM in which only a KIT 502_503dupAY mutation, but not the wild-type allele, was detected. Read More

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http://dx.doi.org/10.1111/jdv.15501DOI Listing
February 2019

Multiple vertebral fractures as the first manifestation of systemic mastocytosis.

Osteoporos Int 2019 Feb 15. Epub 2019 Feb 15.

Rheumatology, Hospital Universitario de Gran Canaria Doctor Negrín, Las Palmas, Spain.

Systemic mastocytosis is a clonal disease of the mast cell progenitors of the bone marrow. The clinical picture varies from asymptomatic (indolent) to highly aggressive (mast cell leukemia). Up to one-third of patients with SM have osteoporosis and fractures. Read More

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http://dx.doi.org/10.1007/s00198-019-04897-9DOI Listing
February 2019
1 Read

Vomiting and Dehydration in a 2-Year-Old.

Pediatrics 2019 Feb 12. Epub 2019 Feb 12.

Divisions of Emergency Medicine and.

A 2-year-old girl with a past medical history of cutaneous mastocytosis and eczema presented with 1 day of yellow-green, nonbloody vomiting, bradycardia, and listlessness. She was evaluated by her pediatrician and sent to the emergency department because of concern for dehydration. In the emergency department, she improved with fluid rehydration but still had decreased energy and bradycardia. Read More

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http://dx.doi.org/10.1542/peds.2018-0504DOI Listing
February 2019
1 Read

Midostaurin for the management of FLT3-mutated acute myeloid leukemia and advanced systemic mastocytosis.

Am J Health Syst Pharm 2019 Feb;76(5):268-274

Department of Pharmaceutical Care, University of Iowa Hospitals & Clinics, Iowa City, IA.

Purpose: This article reviews the pharmacology, efficacy, safety, cost, and future directions of midostaurin for the treatment of acute myeloid leukemia (AML), aggressive systemic mastocytosis, systemic mastocytosis with associated hematological neoplasm, and mast cell leukemia, collectively known as advanced systemic mastocytosis (SM).

Summary: Midostaurin was approved by the U.S. Read More

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http://dx.doi.org/10.1093/ajhp/zxy050DOI Listing
February 2019
1 Read

Validation of the Mayo alliance prognostic system for mastocytosis.

Blood Cancer J 2019 Feb 11;9(2):18. Epub 2019 Feb 11.

CRIMM, Centro di Ricerca e Innovazione per le Malattie Mieloproliferative, Azienda Ospedaliera Universitaria Careggi, Dipartimento di Medicina Sperimentale e Clinica, Università degli Studi, Firenze, Italy.

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http://dx.doi.org/10.1038/s41408-019-0179-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6370807PMC
February 2019

Palmoplantar maculopapular cutaneous mastocytosis.

Int J Dermatol 2019 Feb 11. Epub 2019 Feb 11.

Department of Dermatology, Sir Paul Boffa Hospital, Floriana, Malta.

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http://dx.doi.org/10.1111/ijd.14385DOI Listing
February 2019

Proposed Diagnostic Algorithm for Patients With Suspected Mast Cell Activation Syndrome.

J Allergy Clin Immunol Pract 2019 Feb 5. Epub 2019 Feb 5.

Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md.

Mast cell activation (MCA) accompanies diverse physiologic and pathologic processes and is one of the more frequently encountered conditions in medicine. MCA-related symptoms are usually mild and often transient. In such cases, histamine receptor blockers and other mediator-targeting drugs can usually control MCA. Read More

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http://dx.doi.org/10.1016/j.jaip.2019.01.006DOI Listing
February 2019
7 Reads

The "cholesterol paradox" in patients with mastocytosis.

Atherosclerosis 2019 Jan 26. Epub 2019 Jan 26.

Divisione Immunologia, Euro Medical Center Srl, Firenze, Italy.

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http://dx.doi.org/10.1016/j.atherosclerosis.2019.01.019DOI Listing
January 2019

The efficacy of omalizumab in Cutaneous Mastocytosis: A case series.

Dermatol Ther 2019 Jan 29:e12848. Epub 2019 Jan 29.

Dermatology Department, Center for Clinical Studies, Houston, Texas.

Background: Mastocytosis describes a heterogeneous group of disorders arising from a clonal proliferation of mast cells. Given the lack of curative treatments for the cutaneous form, there is a significant need for superior therapies. Omalizumab is a recombinant DNA-derived humanized IgG monoclonal antibody that selectively binds to human immunoglobulin E (IgE). Read More

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http://doi.wiley.com/10.1111/dth.12848
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http://dx.doi.org/10.1111/dth.12848DOI Listing
January 2019
2 Reads

Bone Marrow Mast Cell Antibody-Targetable Cell Surface Protein Expression Profiles in Systemic Mastocytosis.

Int J Mol Sci 2019 Jan 28;20(3). Epub 2019 Jan 28.

Cancer Research Centre (IBMCC, USAL-CSIC), Cytometry Service (NUCLEUS) and Department of Medicine, University of Salamanca, 37007 Salamanca, Spain.

Despite recent therapeutic advances, systemic mastocytosis (SM) remains an incurable disease due to limited complete remission (CR) rates even after novel therapies. To date, no study has evaluated the expression on SM bone marrow mast cells (BMMC) of large panel of cell surface suitable for antibody-targeted therapy. In this study, we analyzed the expression profile of six cell-surface proteins for which antibody-based therapies are available, on BMMC from 166 SM patients vs. Read More

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http://dx.doi.org/10.3390/ijms20030552DOI Listing
January 2019
6 Reads

Novel Approaches for Systemic Mastocytosis.

Curr Opin Hematol 2019 Mar;26(2):112-118

Knight Cancer Institute.

Purpose Of Review: The purpose of this review is to summarize the pathophysiology of systemic mastocytosis, review the most recent clinical trials and drug development in systemic mastocytosis, with a specific focus on the advanced systemic mastocytosis subtypes.

Recent Findings: Systemic mastocytosis is a clonal neoplasm of mast cells that has had a number of successful therapeutic options being developed in the past few years. The first therapeutic agent to be Food and Drug Administration (FDA) approved in decades was midostaurin in 2017 with a 60% response rate % with improvement in both end-organ damage and symptoms. Read More

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http://dx.doi.org/10.1097/MOH.0000000000000486DOI Listing
March 2019
5 Reads

Urticaria pigmentosa-like skin disease in a domestic shorthair cat.

JFMS Open Rep 2019 Jan-Jun;5(1):2055116918821197. Epub 2019 Jan 7.

Department of Clinical Medicine, University of Illinois Veterinary Teaching Hospital, Urbana, IL, USA.

Case Summary: A 14-month-old castrated male domestic shorthair cat presented with an 8 month history of severe pruritus, alopecia, papules and excoriations. Initial evaluation and treatment prior to referral included skin scrape, cytology, two strict food trials, dermatophyte culture, and bacterial culture and sensitivity, as well as antibiotic therapy, empiric treatment for mites, steroids and ciclosporin A (Atopica; Elanco). The cat was referred to the Dermatology and Otology Clinic at the University of Illinois Veterinary Teaching Hospital for further diagnostics and treatment. Read More

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http://dx.doi.org/10.1177/2055116918821197DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6327337PMC
January 2019
1 Read

A registry-based analysis of survival outcomes in mast cell leukemia.

Leuk Res 2019 Mar 16;78:24-28. Epub 2019 Jan 16.

University of Rochester, Department of Radiation Oncology, 601 Elmwood Avenue, Rochester, NY, 14642, United States. Electronic address:

Introduction: Mast cell leukemia (MCL) is rare and carries a poor prognosis. No standard-of-care has been established. No USA registry-based analyses have examined clinical correlates of overall survival (OS) in MCL patients, thus we aimed to do this using the Surveillance, Epidemiology, and End Results (SEER) database, and the National Cancer Database (NCDB). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01452126193000
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http://dx.doi.org/10.1016/j.leukres.2019.01.005DOI Listing
March 2019
5 Reads

Early diagnosis of cutaneous mastocytosis in an infant: the importance of a clinical sign.

BMJ Case Rep 2019 Jan 18;12(1). Epub 2019 Jan 18.

Dermatology Department, Hospital Distrital da Figueira da Foz, Figueira da Foz, Portugal.

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http://dx.doi.org/10.1136/bcr-2018-228835DOI Listing
January 2019
2 Reads

Mast cells in mastocytosis and allergy - Important player in metabolic and immunological homeostasis.

Adv Med Sci 2019 Jan 11;64(1):124-130. Epub 2019 Jan 11.

Department of General and Medical Biochemistry, University of Gdańsk, Gdańsk, Poland.

The role of mast cell (MC) activity in pathophysiology is complex and challenging and its clinical effects are difficult to predict. Apart from the known role of MCs in basic immunological processes and allergy, underlined is their importance in bone mineralization and in regulation of autoimmune reactions. Mast cell mediators, especially those released from mast cells in degranulation, but also those released constitutively, are important both in metabolic and immunological processes. Read More

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http://dx.doi.org/10.1016/j.advms.2018.08.013DOI Listing
January 2019
2 Reads

Luke Fletcher, md1,2 and uma borate, md1,2.

Curr Opin Hematol 2019 Jan 11. Epub 2019 Jan 11.

Knight Cancer Institute.

Purpose Of Review: The purpose of this review is to summarize the pathophysiology of systemic mastocytosis, review the most recent clinical trials and drug development in systemic mastocytosis, with a specific focus on the advanced systemic mastocytosis subtypes.

Recent Findings: Systemic mastocytosis is a clonal neoplasm of mast cells that has had a number of successful therapeutic options being developed in the past few years. The first therapeutic agent to be Food and Drug Administration (FDA) approved in decades was midostaurin in 2017 with a 60% response rate % with improvement in both end-organ damage and symptoms. Read More

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http://dx.doi.org/10.1097/MOH.0000000000000486DOI Listing
January 2019
2 Reads

KIT D816 mutated/CBF-negative acute myeloid leukemia: a poor-risk subtype associated with systemic mastocytosis.

Leukemia 2019 Jan 11. Epub 2019 Jan 11.

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

KIT D816 mutations (KIT D816) are strongly associated with systemic mastocytosis (SM) but are also detectable in acute myeloid leukemia (AML), where they represent an adverse prognostic factor in combination with core binding factor (CBF) fusion genes. Here, we evaluated the clinical and molecular features of KIT D816/CBF-negative (CBF) AML, a previously uncharacterized combination. All KIT D816/CBF cases (n = 40) had histologically proven SM with associated AML (SM-AML). Read More

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http://www.nature.com/articles/s41375-018-0346-z
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http://dx.doi.org/10.1038/s41375-018-0346-zDOI Listing
January 2019
9 Reads

Mastocytosis and the Fig Wasp (Blastophaga psenes).

Int Arch Allergy Immunol 2019 Jan 9:1-4. Epub 2019 Jan 9.

Sección de Alergia, Hospital Río Carrión, Palencia, Spain.

Background: Mastocytosis involves the abnormal proliferation of mast cells and clinical variability. In the case of anaphylaxis, the triggering antigen, often associated with Hymenoptera allergens, must be identified. The common fig (Ficus carica) requires the fig wasp (Blastophaga psenes) for pollination. Read More

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https://www.karger.com/Article/FullText/495303
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http://dx.doi.org/10.1159/000495303DOI Listing
January 2019
10 Reads

Vertebral Fractures of Unknown Origin: Role of Computed Tomography-Guided Biopsy.

Int J Spine Surg 2018 Dec 21;12(6):673-679. Epub 2018 Dec 21.

Diagnostic and Interventional Radiology, IRCCS Instituto Ortopedico Rizzoli, Bologna, Italy.

Background: We performed a retrospective evaluation of histological and imaging results of patients submitted to computed tomography (CT)-guided biopsy for vertebral fractures (VFs) of unknown etiology to evaluate the pathological causes of fractures and also to observe the diagnostic results of imaging studies available.

Methods: We retrospectively reviewed all the CT-guided vertebral biopsies performed in our institution in the last 2 years, selecting patients with VF of unknown etiology. We reviewed clinical records, imaging studies, and histological examination results. Read More

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http://ijssurgery.com/lookup/doi/10.14444/5084
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http://dx.doi.org/10.14444/5084DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314342PMC
December 2018
4 Reads

Cutaneous mastocytosis treatment: strategies, limitations and perspectives.

Postepy Dermatol Alergol 2018 Dec 13;35(6):541-545. Epub 2018 Aug 13.

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

Mastocytosis is a rare myeloproliferative disease, characterized by excessive proliferation and accumulation of mast cells in the tissues. In cutaneous mastocytosis (CM), mast cells infiltration is limited to the skin, whereas in systemic mastocytosis (SM) internal organs are involved. The first-line treatment in CM is antimediator therapy (mainly H1 and H2 antihistamines) and short-term topical corticosteroids. Read More

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http://dx.doi.org/10.5114/ada.2018.77605DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320483PMC
December 2018
1 Read

Molecular response to imatinib in KIT F522C-mutated systemic mastocytosis.

Leuk Res 2019 Feb 24;77:28-29. Epub 2018 Dec 24.

Department of Haematology, St. James's Hospital, Dublin, Ireland.

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http://dx.doi.org/10.1016/j.leukres.2018.12.010DOI Listing
February 2019
1 Read

Toward a Unified Database Registry in Mastocytosis.

J Allergy Clin Immunol Pract 2019 Jan;7(1):88

Department of Internal Medicine, Division of Allergy, Mayo Clinic, Rochester, Minn.

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https://linkinghub.elsevier.com/retrieve/pii/S22132198183071
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http://dx.doi.org/10.1016/j.jaip.2018.11.001DOI Listing
January 2019
3 Reads

Advances in diagnosis of mastocytosis and hypereosinophilic syndrome.

Semin Hematol 2019 Jan 28;56(1):22-29. Epub 2018 May 28.

Hematology Section, Department of Laboratory Medicine, Clinical Center, National Institutes of Health, Bethesda, MD 20892-1508.

Mastocytosis and hypereosinophilic syndrome is very rare neoplastic hematopoietic diseases. Mastocytosis is characterized by expansion and accumulation of clonal tissue mast cells in skin and/or various internal organs, while hypereosinophilic syndrome manifests with an increased number of eosinophils in the peripheral blood and tissue damage. These diseases represent a diagnostic challenge, since they can have overlapping clinical and pathologic features. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00371963183003
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http://dx.doi.org/10.1053/j.seminhematol.2018.05.005DOI Listing
January 2019
6 Reads

Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology.

J Natl Compr Canc Netw 2018 Dec;16(12):1500-1537

Mastocytosis is a group of heterogeneous disorders resulting from the clonal proliferation of abnormal mast cells and their accumulation in the skin and/or in various extracutaneous organs. Systemic mastocytosis is the most common form of mastocytosis diagnosed in adults, characterized by mast cell infiltration of one or more extracutaneous organs (with or without skin involvement). The identification of KIT D816V mutation and the emergence of novel targeted therapies have significantly improved the diagnosis and treatment of systemic mastocytosis. Read More

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http://www.jnccn.org/lookup/doi/10.6004/jnccn.2018.0088
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http://dx.doi.org/10.6004/jnccn.2018.0088DOI Listing
December 2018
22 Reads

Systemic mastocytosis with an associated hematological neoplasm masquerading as overt primary myelofibrosis.

Blood 2018 Dec;132(24):2613

Royal Jubilee Hospital.

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http://dx.doi.org/10.1182/blood-2018-09-875914DOI Listing
December 2018
10.452 Impact Factor

Sialic acid-binding immunoglobulin-like lectin (Siglec) 8 in patients with eosinophilic disorders: Receptor expression and targeting using chimeric antibodies.

J Allergy Clin Immunol 2018 Dec 10. Epub 2018 Dec 10.

Human Eosinophil Section, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, Md. Electronic address:

Background: Sialic acid-binding immunoglobulin-like lectin (Siglec) 8 is selectively expressed on eosinophils, mast cells, and basophils and, when engaged on eosinophils, can cause cell death.

Objective: We sought to characterize surface and soluble Siglec-8 (sSiglec-8) levels in normal donors (NDs) and eosinophilic donors (EOs) and assess the efficacy of anti-Siglec-8 antibodies in inducing eosinophil cell death in vitro.

Methods: Eosinophil expression of Siglec-8 was assessed by using flow cytometry and quantitative PCR. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00916749183174
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http://dx.doi.org/10.1016/j.jaci.2018.10.066DOI Listing
December 2018
11 Reads
11.476 Impact Factor

Systemic mastocytosis in adults: 2019 update on diagnosis, risk stratification and management.

Am J Hematol 2019 Mar 2;94(3):363-377. Epub 2019 Jan 2.

Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.

Overview: Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MCs) in extra-cutaneous organs.

Diagnosis: The major criterion is presence of multifocal clusters of abnormal MC in the bone marrow. Minor diagnostic criteria include elevated serum tryptase level, abnormal MC CD25 expression, and presence of KITD816V mutation. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/ajh.25371
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http://dx.doi.org/10.1002/ajh.25371DOI Listing
March 2019
23 Reads

Mast Cells as Key Players in Allergy and Inflammation.

J Investig Allergol Clin Immunol 2018 Dec;28(6):365-378

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

Mast cells (MCs) are a key structural and functional component of both the innate and the adaptive immune systems. They are involved in many different processes, but play a major role in the response to infections and in inflammatory reactions. In addition, MCs are the main effector cells in allergy. Read More

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http://dx.doi.org/10.18176/jiaci.0327DOI Listing
December 2018
1 Read

Adverse reactions to drugs and biologics in patients with clonal mast cell disorders: A Work Group Report of the Mast Cells Disorder Committee, American Academy of Allergy, Asthma & Immunology.

J Allergy Clin Immunol 2018 Dec 6. Epub 2018 Dec 6.

Department of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany.

Providers caring for patients with mastocytosis are tasked with the decision to consider therapeutic options. This can come with some trepidation because information available in the public domain lists numerous mast cell (MC) activators based on data that do not discriminate between primates, rodents, and MC lines; do not consider dosage; and do not take into account previous exposure and resultant clinical findings. This being said, there is support in the literature for an enhanced MC response in some patients with mastocytosis and in cases in which there is a greater incidence of adverse reactions associated with certain antigens, such as venoms and drugs. Read More

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http://dx.doi.org/10.1016/j.jaci.2018.10.063DOI Listing
December 2018
4 Reads

Chondroitin sulfate inhibits secretion of TNF and CXCL8 from human mast cells stimulated by IL-33.

Biofactors 2019 Jan 6;45(1):49-61. Epub 2018 Dec 6.

Laboratory of Molecular Immunopharmacology and Drug Discovery, Department of Immunology, Tufts University School of Medicine, Boston, MA, USA.

Glycosaminoglycans (GAGs) are linear, highly negatively charged carbohydrate chains present in connective tissues. Chondroitin sulfate (CS) and heparin (Hep) are also found in the numerous secretory granules of mast cells (MC), tissue immune cells involved in allergic and inflammatory reactions. CS and Hep may inhibit secretion of histamine from rat connective tissue MC, but their effect on human MC remains unknown. Read More

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http://dx.doi.org/10.1002/biof.1464DOI Listing
January 2019
3 Reads

The new tool "" in advanced systemic mastocytosis.

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):127-136

Divisions of Hematology and.

Mastocytosis is a rare disease characterized by KIT-driven expansion and accumulation of neoplastic mast cells in various tissues. Although mediator symptoms related to mast cell activation can impose a symptom burden in cutaneous disease and across the spectrum of systemic mastocytosis subtypes, the presence of an associated hematologic neoplasm and/or organ damage denotes advanced disease and the potential for increased morbidity and mortality. In addition to the revised 2016 World Health Organization classification of mastocytosis, a new diagnostic and treatment toolkit, tethered to enhanced molecular characterization and monitoring, is poised to transform the management of patients with advanced systemic mastocytosis (advSM). Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.127DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245986PMC
November 2018
8 Reads

Detection of KIT D816V in peripheral blood of children with manifestations of cutaneous mastocytosis suggests systemic disease.

Br J Haematol 2018 Dec;183(5):775-782

Mast Cell Biology Section, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.

The use of allele-specific quantitative polymerase chain reaction to identify KIT D816V in the peripheral blood of adults with mastocytosis has been reported to have value in the diagnosis, assessment of disease burden and management of this disease. To examine the value of this assay in children with cutaneous manifestations of mastocytosis, we assessed data on 65 patients with all variants of paediatric-onset mastocytosis, including those known to have systemic disease, to correlate KIT mutation status with clinical findings, serum tryptase levels and bone marrow histopathology. We found that KIT D816V was not identified in the peripheral blood of children known to have only cutaneous disease (specificity 100%) but was found in those known to have both cutaneous and systemic/probable systemic disease (sensitivity of 85·2%). Read More

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http://dx.doi.org/10.1111/bjh.15624DOI Listing
December 2018
13 Reads

Radiotherapy for breast cancer associated with a cutaneous presentation of systemic mastocytosis-a case report and literature review.

J Surg Case Rep 2018 Nov 23;2018(11):rjy317. Epub 2018 Nov 23.

Department of Breast & Endocrine Surgery, Nepean Hospital, Penrith, NSW 2747, Australia.

Ionizing radiation therapy is a common adjuvant therapy for individuals undergoing surgery for breast cancer. There are many well-recognized acute and chronic cutaneous reactions that can vary in severity, course and duration. We present a rare cutaneous manifestation of systemic mastocystosis, in a 59-year-old female who underwent adjuvant radiotherapy following local excision of ductal carcinoma in situ. Read More

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http://dx.doi.org/10.1093/jscr/rjy317DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6250909PMC
November 2018
1 Read

Dermoscopic findings of pseudoxanthomatous mastocytosis localized on vulva.

An Bras Dermatol 2018 Nov/Dec;93(6):940-941

Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, China.

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http://dx.doi.org/10.1590/abd1806-4841.20188039DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6256220PMC
February 2019
2 Reads

Lokivetmab therapy for pruritus in a dog with cutaneous mastocytosis.

Vet Dermatol 2019 Feb 26;30(1):73-e22. Epub 2018 Nov 26.

Department of Small Animal Medicine and Surgery, College of Veterinary Medicine, University of Georgia, 2200 College Station Road, Athens, GA, 30602, USA.

Background: Cutaneous mastocytosis (CM) is a rare disease of dogs characterized by rash, pruritus and proliferation of mast cells in the skin. Oral H1 antihistamines are recommended as the treatment to control pruritus.

Hypothesis/objective: To describe the effective treatment of pruritus associated with CM with lokivetmab in one dog. Read More

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http://doi.wiley.com/10.1111/vde.12702
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http://dx.doi.org/10.1111/vde.12702DOI Listing
February 2019
13 Reads

Oral suplatast tosilate for the treatment of urticaria pigmentosa, skin lesion in mastocytosis.

Int J Dermatol 2019 Feb 19;58(2):e38-e39. Epub 2018 Nov 19.

Department of Dermatology, Sakai City Medical Center, Osaka, Japan.

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http://dx.doi.org/10.1111/ijd.14302DOI Listing
February 2019
3 Reads

Massive release of the histamine-degrading enzyme diamine oxidase during severe anaphylaxis in mastocytosis patients.

Allergy 2018 Nov 12. Epub 2018 Nov 12.

Department of Clinical Pharmacology, Medical University of Vienna, Vienna, Austria.

Background: Histaminolytic activity mediated by diamine oxidase (DAO) is present in plasma after induction of severe anaphylaxis in rats, guinea pigs, and rabbits. Heparin released during mast cell degranulation in the gastrointestinal tract might liberate DAO from heparin-sensitive storage sites. DAO release during anaphylaxis has not been demonstrated in humans. Read More

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http://doi.wiley.com/10.1111/all.13663
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http://dx.doi.org/10.1111/all.13663DOI Listing
November 2018
7 Reads

The Data Registry of the European Competence Network on Mastocytosis (ECNM): Set Up, Projects, and Perspectives.

J Allergy Clin Immunol Pract 2019 Jan 8;7(1):81-87. Epub 2018 Nov 8.

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

Mastocytosis is a unique hematologic neoplasm with complex biology and pathology and a variable clinical course. The disease can essentially be divided into cutaneous mastocytosis (CM) and systemic mastocytosis (SM). In adults, SM is diagnosed in most cases and manifests as either indolent or advanced disease. Read More

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http://dx.doi.org/10.1016/j.jaip.2018.09.024DOI Listing
January 2019
63 Reads

Mayo alliance prognostic system for mastocytosis: clinical and hybrid clinical-molecular models.

Blood Adv 2018 Nov;2(21):2964-2972

Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN.

Systemic mastocytosis (SM) is a clinically heterogeneous disease with prognosis chiefly assigned based on World Health Organization (WHO) morphologic subclassification. We assessed the feasibility of developing contemporary risk models for SM based on clinical and integrated clinical-genetics information. Diagnosis of SM was per WHO criteria, and karyotype and next-generation sequencing data were available in a subset of the total 580 patients (median age, 55 years; range, 18-88 years) seen at the Mayo Clinic between 1968 and 2015. Read More

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http://www.bloodadvances.org/lookup/doi/10.1182/bloodadvance
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http://dx.doi.org/10.1182/bloodadvances.2018026245DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6234360PMC
November 2018
7 Reads

Epidemiological assessment of the risk of canine mast cell tumours based on the Kiupel two-grade malignancy classification.

Acta Vet Scand 2018 Nov 3;60(1):70. Epub 2018 Nov 3.

Department and Clinic of Animal Reproduction, Faculty of Veterinary Medicine, University of Life Sciences, Akademicka 13 St., 20-950, Lublin, Poland.

Background: The degree of differentiation of mast cell tumours (MCTs) is the most important feature and reflects the morphological characteristics and metastatic potential of the tumour and its likely response to treatment and the prognosis. The aim of this study was to epidemiologically analyse the risk of MCT development in dogs according to breed, age, sex, size and anatomical location of the tumour using the Kiupel grading system. The analysis involved 492 dogs selected based on a histopathological assessment of 2763 canine skin tumours. Read More

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http://dx.doi.org/10.1186/s13028-018-0424-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6215678PMC
November 2018
5 Reads

Mast cell disorders: From infancy to maturity.

Allergy 2019 Jan 28;74(1):53-63. Epub 2018 Nov 28.

Lydia Becker Institute of Immunology and Inflammation, University of Manchester, Manchester, UK.

Mast cells are typically linked to immediate hypersensitivity and anaphylaxis. This review looks beyond this narrow role, focusing on how these cells have evolved and diversified via natural selection promoting serine protease gene duplication, augmenting their innate host defense function against helminths and snake envenomation. Plasticity of mast cell genes has come at a price. Read More

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http://doi.wiley.com/10.1111/all.13657
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http://dx.doi.org/10.1111/all.13657DOI Listing
January 2019
21 Reads

New role for the modern allergist in drug allergy: Assess, diagnose, and de-label.

Authors:
Mariana Castells

Ann Allergy Asthma Immunol 2018 Nov;121(5):515-516

Drug Hypersensitivity and Desensitization Center, Mastocytosis Center, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. Electronic address:

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http://dx.doi.org/10.1016/j.anai.2018.09.455DOI Listing
November 2018
3 Reads

Impact of somatic and germline mutations on the outcome of systemic mastocytosis.

Blood Adv 2018 Nov;2(21):2814-2828

Cancer Research Center (IBMCC, USAL-CSIC), Department of Medicine and Cytometry Service (NUCLEUS), CIBERONC, University of Salamanca, Salamanca, Spain.

Systemic mastocytosis (SM) is a highly heterogeneous disease with indolent and aggressive forms, with the mechanisms leading to malignant transformation still remaining to be elucidated. Here, we investigated the presence and frequency of genetic variants in 34 SM patients with multilineal D816V mutations. Initial screening was performed by targeted sequencing of 410 genes in DNA extracted from purified bone marrow cells and hair from 12 patients with nonadvanced SM and 8 patients with advanced SM, followed by whole-genome sequencing (WGS) in 4 cases. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018020628DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6234367PMC
November 2018
17 Reads

Two cases of mimics of bone metastasis in breast cancer.

BJR Case Rep 2018 16;4(2):20170091. Epub 2017 Dec 16.

Department of Radiology, James Cook University Hospital, Middlesbrough, UK.

In patients with breast cancer, the appearance of sclerotic bone lesions on imaging should raise the suspicion of skeletal metastases. However, before making the diagnosis it is important to consider the clinical context and remember that there are conditions that can mimic bone metastasis. We present two cases of mimics of bone metastasis: systemic mastocytosis and osteopoikilosis. Read More

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http://dx.doi.org/10.1259/bjrcr.20170091DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6159113PMC
December 2017
3 Reads

Plasma cytokeratin-18 concentrations as noninvasive biomarker of early gastrointestinal toxicosis in dogs receiving toceranib.

J Vet Intern Med 2018 Nov 24;32(6):2061-2068. Epub 2018 Oct 24.

Department of Veterinary Clinical Sciences, College of Veterinary Medicine, The Ohio State University, Columbus, Ohio.

Background: No biomarkers for the early detection of gastrointestinal (GI) toxicosis secondary to antineoplastic treatment are recognized in veterinary medicine. Toceranib causes GI toxicosis in dogs.

Hypothesis/objective: To assess if changes in plasma cytokeratin 18 (CK18) concentration, measured in dogs being treated with toceranib phosphate, can predict the onset of GI toxicosis. Read More

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http://dx.doi.org/10.1111/jvim.15326DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6271317PMC
November 2018

Mastocytosis-derived extracellular vesicles exhibit a mast cell signature, transfer KIT to stellate cells, and promote their activation.

Proc Natl Acad Sci U S A 2018 11 23;115(45):E10692-E10701. Epub 2018 Oct 23.

Mast Cell Biology Section, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892.

Extracellular vesicles (EVs) have been implicated in the development and progression of hematological malignancies. We thus examined serum samples from patients with systemic mastocytosis (SM) and found EVs with a mast cell signature including the presence of tryptase, FcεRI, MRGX2, and KIT. The concentration of these EVs correlated with parameters of disease including levels of serum tryptase, IL-6, and alkaline phosphatase and physical findings including hepatosplenomegaly. Read More

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http://www.pnas.org/lookup/doi/10.1073/pnas.1809938115
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http://dx.doi.org/10.1073/pnas.1809938115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6233074PMC
November 2018
23 Reads

[Cutaneous mastocytosis - update and clinical guidelines].

Lakartidningen 2018 10 22;115. Epub 2018 Oct 22.

Karolinska Institutet Department of Medicine Solna - Stockholm, Sweden Karolinska Institutet Department of Medicine Solna - Stockholm, Sweden.

Mastocytosis is a rare and multifaceted disease group characterized by mast cell accumulation in the skin and/or internal organs. In its most common form solitary or widespread, often itchy, red-brown skin lesions appear in childhood or during adulthood (cutaneous mastocytosis). The skin lesions are not always easy to recognize by medical professionals; hence, a correct diagnosis is often delayed. Read More

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October 2018
13 Reads

Fatigue is common and severe in patients with mastocytosis.

Int J Immunopathol Pharmacol 2018 Jan-Dec;32:2058738418803252

2 Department of Clinical Science, Faculty of Medicine, University of Bergen, Bergen, Norway.

Chronic fatigue is a common phenomenon in inflammatory and autoimmune conditions, in cancer, and in neurodegenerative diseases. Although pain and psychological factors influence fatigue, there is an increasing understanding that there is a genetic basis, and that activation of the innate immune system is an essential generator of fatigue. Mast cells are important actors in innate immunity and serve specialized defense responses against parasites and other pathogens. Read More

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http://dx.doi.org/10.1177/2058738418803252DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6201179PMC
December 2018
2 Reads

Image Gallery: An unusual presentation of pseudoxanthomatous mastocytosis.

Br J Dermatol 2018 Oct;179(4):e165

Department of Clinical-Surgical, Diagnostic and Pediatric Science, Institute of Dermatology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.

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http://dx.doi.org/10.1111/bjd.16985DOI Listing
October 2018
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