7,115 results match your criteria Mastocytosis


Acute myeloid leukemia with t(8;21)(q22;q22.1)/RUNX1-RUNX1T1 and KIT Exon 8 mutation is associated with characteristic mastocytosis and dismal outcomes.

Exp Mol Pathol 2019 Apr 17. Epub 2019 Apr 17.

Departments of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America. Electronic address:

KIT mutations are observed in about 20-40% of acute myeloid leukemia with t(8;21)(q22;q22.1)/RUNX1-RUNX1T1 [abbreviated AML t(8;21) here] with mutations involving exon 17 being the most common. Despite high frequencies of KIT mutations in both AML t(8;21) and systemic mastocytosis (SM), AML t(8;21) associated with SM is uncommon, and restricted to KIT exon 17 mutated cases. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00144800193021
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http://dx.doi.org/10.1016/j.yexmp.2019.04.009DOI Listing
April 2019
4 Reads

1-CD30 expression in cutaneous lesions of systemic mastocytosis: clinical, biological and histopathological analysis of 27 patients.

J Eur Acad Dermatol Venereol 2019 Apr 16. Epub 2019 Apr 16.

Sorbonne Université, UPMC Université Paris 06, Unité Fonctionnelle de Dermatologie, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, Paris, France.

Mastocytosis is a composite group of heterogeneous diseases defined by the accumulation of abnormal mast cells (MCs) in various tissues. Cutaneous and bone marrow tissues are the most frequently infiltrated in adult patients with systemic mastocytosis (SM) (1) The CD30 (KI-1 antigen) receptor, belongings to the tumor necrosis factor receptor family, may have a role in mastocytosis. CD30 expression in MCs cytoplasm was reported in 2011 by Sotlar K et al. Read More

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http://dx.doi.org/10.1111/jdv.15633DOI Listing
April 2019
1 Read

Protective Effect of Cashew Gum (Anacardium occidentale L.) on 5-Fluorouracil-Induced Intestinal Mucositis.

Pharmaceuticals (Basel) 2019 Apr 3;12(2). Epub 2019 Apr 3.

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

Intestinal mucositis is a common complication associated with 5-fluorouracil (5-FU), a chemotherapeutic agent used for cancer treatment. Cashew gum (CG) has been reported as a potent anti-inflammatory agent. In the present study, we aimed to evaluate the effect of CG extracted from the exudate of L. Read More

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http://dx.doi.org/10.3390/ph12020051DOI Listing

Relevant updates in systemic mastocytosis.

Leuk Res 2019 Apr 4;81:10-18. Epub 2019 Apr 4.

Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY, United States. Electronic address:

Systemic Mastocytosis (SM) is a rare myeloproliferative neoplasm (MPN) that is characterized by a clonal proliferation of mast cells (MCs). The symptoms and clinical presentation of SM are the result of both MC proliferation as well as activation and degranulation, causing hyperactive and over-exaggerated hypersensitivity responses, as well as organ infiltration by pathogenic MCs. The clinical presentation and course of SM is varied and organ involvement can lead to significant morbidity and mortality in some cases. Read More

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http://dx.doi.org/10.1016/j.leukres.2019.04.001DOI Listing
April 2019
1 Read

Single-cell analysis reveals the KIT D816V mutation in haematopoietic stem and progenitor cells in systemic mastocytosis.

EBioMedicine 2019 Apr 8. Epub 2019 Apr 8.

Department of Medicine Solna, Karolinska Institutet, and Karolinska University Hospital, Stockholm 17164, Sweden. Electronic address:

Background: Systemic mastocytosis (SM) is a haematological disease characterised by organ infiltration by neoplastic mast cells. Almost all SM patients have a mutation in the gene encoding the tyrosine kinase receptor KIT causing a D816V substitution and autoactivation of the receptor. Mast cells and CD34 haematopoietic progenitors can carry the mutation; however, in which progenitor cell subset the mutation arises is unknown. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S23523964193023
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http://dx.doi.org/10.1016/j.ebiom.2019.03.089DOI Listing
April 2019
6 Reads

Telangiectasia macularis eruptiva perstans: a neglected type of mastocytosis with exclusively cutaneous involvement? A case series.

Eur J Dermatol 2019 Apr 10. Epub 2019 Apr 10.

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

Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis (CM). Although TMEP has been traditionally thought to be restricted to the skin, a recent retrospective multicentric study established a diagnosis with systemic involvement of mastocytosis in 47% patients affected by TMEP and aggressive systemic mastocytosis in 9%. To evaluate systemic involvement in patients affected by TMEP. Read More

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http://dx.doi.org/10.1684/ejd.2019.3532DOI Listing
April 2019
1 Read

Successful mast-cell-targeted treatment of chronic dyspareunia, vaginitis, and dysfunctional uterine bleeding.

J Obstet Gynaecol 2019 Apr 9:1-6. Epub 2019 Apr 9.

c HealthPartners North Suburban Family Physicians , Roseville , MN , USA.

Dyspareunia, vaginitis and dysfunctional uterine bleeding (DUB) are common problems which, despite their polygenicity, commonly appear idiopathic and treatment-refractory. Mast cell (MC) activation syndrome (MCAS) is a newly-recognised, prevalent, chronic multisystem polymorbidity of general themes of inflammation ± allergic-type phenomena ± aberrant growth/development in assorted tissues. MCs produce significant quantities of heparin, too. Read More

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http://dx.doi.org/10.1080/01443615.2018.1550475DOI Listing
April 2019
2 Reads

Blisters, Vaccines, and Mast Cells: A Difficult Case of Diffuse Cutaneous Mastocytosis.

J Allergy Clin Immunol Pract 2019 Apr;7(4):1370-1372

Division of Rheumatology, Immunology, and Allergy, Department of Medicine, Brigham, and Women's Hospital, Harvard Medical School, Boston, Mass.

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http://dx.doi.org/10.1016/j.jaip.2018.11.046DOI Listing

Insect Sting Anaphylaxis-Or Mastocytosis-Or Something Else?

J Allergy Clin Immunol Pract 2019 Apr;7(4):1117-1123

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

Insect sting anaphylaxis and mast cell disorders are intertwined in a specific and unusual way. There may be specific subsets of clonal mast cell disorders that are predisposed to sting anaphylaxis. The clinical characteristics of the sting reactions should raise suspicion of underlying mastocytosis (eg, hypotension without hives especially in a male). Read More

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http://dx.doi.org/10.1016/j.jaip.2019.01.014DOI Listing
April 2019
2 Reads

Doctor, I Think I Am Suffering from MCAS: Differential Diagnosis and Separating Facts from Fiction.

J Allergy Clin Immunol Pract 2019 Apr;7(4):1109-1114

Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, Mich.

Mast cell activation syndrome (MCAS) is a rare condition defined by a severe systemic reaction to mast cell (MC)-derived mediators. Most cases present with clinical signs of anaphylaxis, and some have an underlying IgE-dependent allergy. A primary MC disease (mastocytosis) may also be detected. Read More

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http://dx.doi.org/10.1016/j.jaip.2018.11.045DOI Listing
April 2019
1 Read

Mast Cell Activation Syndrome and Mastocytosis: Initial Treatment Options and Long-Term Management.

J Allergy Clin Immunol Pract 2019 Apr;7(4):1097-1106

Mayo Clinic Program for Mast Cell and Eosinophil Disorders, Mayo Clinic, Rochester, Minn.

Patients with clonal mast cell activation syndromes (MCAS) including cutaneous and systemic mastocytosis (SM) may present with symptoms of mast cell activation, but in addition can have organ damage from the local effects of tissue infiltration by clonal mast cells. Patients with nonclonal MCAS may have chronic or episodic mast cell activation symptoms with an increase in serum tryptase and/or urinary metabolites of histamine, prostaglandin D2, and leukotrienes. Symptoms of MCAS and SM can be managed by blockade of mediator receptors (H1 and H2 antihistamines, leukotriene receptor blockade), inhibition of mediator synthesis (aspirin, zileuton), mediator release (sodium cromolyn), anti-IgE therapy, or a combination of these approaches. Read More

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http://dx.doi.org/10.1016/j.jaip.2019.02.002DOI Listing
April 2019
2 Reads

Usefulness of Omalizumab and Sting Challenge Test in Hymenoptera Venom Allergy and Mastocytosis.

J Investig Allergol Clin Immunol 2019 Apr 8. Epub 2019 Apr 8.

Allergy Department, Hospital Universitario Ramón y Cajal, Madrid, Spain.

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http://dx.doi.org/10.18176/jiaci.0395DOI Listing
April 2019
1 Read

Omalizumab therapy for mast cell-mediator symptoms in patients with ISM, CM, MMAS and MCAS.

J Allergy Clin Immunol Pract 2019 Apr 4. Epub 2019 Apr 4.

Imagine Institute, INSERM U1163 and CNRS ERL 8654, Paris Descartes University, Sorbonne Paris Cité, Paris, France; Department of Hematology, Necker Children's Hospital, APHP, Paris, France; French Reference Center for Mastocytosis (CEREMAST), Necker Children's Hospital, APHP, Paris, France.

Background: Patients with mast cell diseases may suffer from various distressing symptoms, which can be insufficiently controlled with available therapies, severely affecting their quality of life. There is a need for new and safe treatment options for these patients.

Objectives: We aimed to evaluate safety and efficacy of omalizumab administration in patients with a symptomatic mast cell disorder. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22132198193032
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http://dx.doi.org/10.1016/j.jaip.2019.03.039DOI Listing
April 2019
8 Reads

Oncogenic D816V-KIT signaling in mast cells causes persistent IL-6 production.

Haematologica 2019 Apr 4. Epub 2019 Apr 4.

Mast Cell Biology Section, Laboratory of Allergic Diseases, NIAID, NIH;

Persistent dysregulation of IL-6 production and signaling have been implicated in the pathology of various cancers. In systemic mastocytosis, increased serum levels of IL-6 associate with disease severity and progression, although the mechanisms involved are not well understood. Since systemic mastocytosis often associates with the presence in hematopoietic cells of a somatic gain-of-function variant in KIT, D816V-KIT, we examined its potential role in IL-6 upregulation. Read More

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.212126DOI Listing
April 2019
5 Reads

Plasma histamine elevation in a large cohort of sickle cell disease patients.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Department of Internal Medicine, Tenon Hospital, AP-HP, Paris VI University, Paris, France.

The role of mast cells has been questioned in sickle cell disease (SCD). We performed a prospective study evaluating plasma histamine and tryptase levels in a cohort of paediatric and adult patients, in steady state (n = 132) and during vaso-occlusive crisis (VOC) (n = 121). Histamine level was elevated in 18% of patients in steady state and in 61% during VOC. Read More

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http://dx.doi.org/10.1111/bjh.15900DOI Listing
March 2019
2 Reads

Anaesthetic management of patients with pre-existing allergic conditions: a narrative review.

Br J Anaesth 2019 Mar 12. Epub 2019 Mar 12.

Department of Anaesthesia and Peri-operative Medicine, Auckland City Hospital, Auckland, New Zealand.

This narrative review seeks to distinguish the clinical patterns of pre-existing allergic conditions from other confounding non-allergic clinical entities, and to identify the potential related risks and facilitate their perioperative management. Follow-up investigation should be performed after a perioperative immediate hypersensitivity to establish a diagnosis and provide advice for subsequent anaesthetics, the main risk factor for perioperative immunoglobulin E (IgE)-mediated anaphylaxis being a previous uninvestigated perioperative immediate hypersensitivity reaction. The concept of cross-reactivity between drugs used in the perioperative setting and food is often quoted, but usually not supported by evidence. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00070912193006
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http://dx.doi.org/10.1016/j.bja.2019.01.020DOI Listing
March 2019
7 Reads

Inhibitory effects of midostaurin and avapritinib on myeloid progenitors derived from patients with KIT D816V positive advanced systemic mastocytosis.

Leukemia 2019 Mar 25. Epub 2019 Mar 25.

Department of Hematology and Oncology, University Medical Centre Mannheim, Mannheim, Germany.

Advanced systemic mastocytosis (advSM) is characterized by the presence of an acquired KIT D816V mutation in >90% of patients. In the majority of patients, KIT D816V is not only detected in mast cells but also in other hematopoietic lineages. We sought to investigate the effects of the KIT-inhibitors midostaurin and avapritinib on single-cell-derived myeloid progenitor cells using granulocyte-macrophage colony-forming-units of patients with KIT D816V positive advSM. Read More

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http://www.nature.com/articles/s41375-019-0450-8
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http://dx.doi.org/10.1038/s41375-019-0450-8DOI Listing
March 2019
3 Reads

Recent advances in our understanding of mast cell activation - or should it be mast cell mediator disorders?

Expert Rev Clin Immunol 2019 Mar 18. Epub 2019 Mar 18.

e Department of Otolaryngology , Beijing Electric Power Hospital , Beijing , China .

Introduction: An increasing number of patients present with multiple symptoms affecting many organs including the brain due to multiple mediators released by mast cells. These unique tissue immune cells are critical for allergic reactions triggered by IgE, but are also stimulated (not activated) by immune, drug, environmental, food, infectious, and stress triggers, leading to secretion of multiple mediators often without histamine and tryptase. The presentation, diagnosis and management of the spectrum of mast cell disorders is very confusing. Read More

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http://dx.doi.org/10.1080/1744666X.2019.1596800DOI Listing

[Surgical interventions in patients with systemic mast cell activation disease : Recommendations for perioperative management].

Chirurg 2019 Mar 14. Epub 2019 Mar 14.

Institut für Humangenetik, Universitätsklinikum Bonn, Sigmund-Freud-Str. 25, 53127, Bonn, Deutschland.

Background: Systemic mast cell activation disease (MCAD, prevalence 5-10%) is a multifactorial, polygenic disease with multisystemic symptoms that is characterized by an unregulated increased release of mast cell mediators and an accumulation of activated mast cells potentially in all organs and tissues. Due to the high prevalence of the disease, physicians involved in surgical, anesthesiological and interventional procedures are often unknowingly faced with MCAD patients experiencing unexpected preoperative, intraoperative and postoperative complications, if no mast cell-specific treatment regimens have been applied.

Objective: The findings from a literature search, consensus recommendations of the various international expert groups and extensive own experience in the treatment of MCAD patients enable an empirical and evidence-based care of MCAD patients in association with invasive procedures. Read More

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http://dx.doi.org/10.1007/s00104-019-0935-zDOI Listing
March 2019
3 Reads

Indolent systemic mastocytosis mimicking Crohn's disease.

Ann Gastroenterol 2019 Mar-Apr;32(2):208-210. Epub 2019 Jan 2.

School of Medicine, European University Cyprus (Alexandros Hatjivasilis, Kalliopi J. Ioakim, Anastasia Neocleous, Karolos Demetriou, Soni Panjiyar, George Potamitis), Cyprus.

Mastocytosis is a rare and heterogeneous group of diseases whose common element is the presence of dense mast-cell infiltrates in various tissues. The gastrointestinal (GI) tract is frequently affected with vague and subtle manifestations, making the diagnosis of GI mastocytosis rather formidable and challenging. The diagnosis of the disease requires a high level of clinical suspicion and an index of familiarity. Read More

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http://dx.doi.org/10.20524/aog.2018.0342DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6394271PMC
January 2019
1 Read

Reply to: "The "cholesterol paradox" in patients with mastocytosis".

Atherosclerosis 2019 Mar 2. Epub 2019 Mar 2.

Department of Internal Medicine, Laboratory Vascular Medicine, Erasmus MC, Rotterdam, the Netherlands. Electronic address:

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http://dx.doi.org/10.1016/j.atherosclerosis.2019.02.018DOI Listing
March 2019
1 Read

Perioperative anaphylaxis in a patient with a solitary mastocytoma.

Pediatr Dermatol 2019 Mar 10. Epub 2019 Mar 10.

Department of Dermatology, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.

Children with more extensive cutaneous mastocytosis have a higher risk for symptoms secondary to release of mast cell mediators. However, the remote possibility of anaphylaxis in patients with a solitary lesion suggests the need for cautious use of general anesthesia in these children. We describe an unusual case of a patient with a solitary mastocytoma who experienced an anaphylactic reaction during a surgical procedure and make recommendations to reduce the risk of intraoperative anaphylaxis in mast cell disease. Read More

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http://doi.wiley.com/10.1111/pde.13777
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http://dx.doi.org/10.1111/pde.13777DOI Listing
March 2019
5 Reads

Full-term newborn infant with blistering skin lesions-Caution regarding use of pain medications.

Pediatr Dermatol 2019 Mar 10. Epub 2019 Mar 10.

Department of Pediatrics, Division of Neonatology, University of Connecticut, Farmington, Connecticut.

Multiple opioids are known to trigger mast cell degranulation. We report the case of a neonate with blistering skin lesions at birth who died of multi-organ failure after administration of morphine. Given the excessive histamine release and potential complications associated with morphine administration, alternative opioids and adjuvants should be considered in infants presenting with evidence of bullous or infiltrative skin lesions until mastocytosis is ruled out. Read More

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http://dx.doi.org/10.1111/pde.13794DOI Listing
March 2019
1 Read

Anaphylactic risk due to systemic mastocytosis: Perioperative management in cardiac surgery.

Rev Esp Anestesiol Reanim 2019 Mar 5. Epub 2019 Mar 5.

Servicio de Anestesiología y Reanimación, Hospital Universitario de La Princesa, Madrid, España.

Mastocytosis is a disease characterised by an abnormal proliferation of mast cells. The degranulation of mast cells can be triggered by chemical, physical, and psychological factors, and in severe cases may be accompanied by cardiovascular alterations and shock. Tryptase concentrations greater than 20ug/L may be associated with an increased risk of mastocyte degranulation. Read More

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http://dx.doi.org/10.1016/j.redar.2019.01.008DOI Listing
March 2019
7 Reads

A case report of systemic mastocytosis associated with multiple hematologic non-mast cell lineage diseases.

Hematol Oncol 2019 Mar 8. Epub 2019 Mar 8.

Hematology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Systemic mastocytosis (SM) is a hematological malignancy characterized by extracutaneous infiltration by atypical mast cells. Together with indolent SM, aggressive SM, and mast cell leukemia, the World Health Organization (WHO) recognizes another major disease subgroup: SM with an associated hematological neoplasm, which is characterized by the presence of a concurrent neoplasm, more commonly, a chronic myelomonocytic leukemia. While KIT D816V is commonly regarded as the driver mutation, the clinical presentation of SM is extremely varied. Read More

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http://dx.doi.org/10.1002/hon.2605DOI Listing
March 2019
3 Reads

Higher prevalence of vertebral fractures in systemic mastocytosis, but not in cutaneous mastocytosis and idiopathic mast cell activation syndrome.

Osteoporos Int 2019 Mar 7. Epub 2019 Mar 7.

Rheumatology Centre, Pierre Paul Riquet Hospital, Toulouse University Hospital & Paul Sabatier University, 1 Place du Dr Baylac, 31059, Toulouse, France.

Little is known about osteoporosis in mast cell disorders (MCDs) not related to systemic mastocytosis. We described osteoporosis and fractures in MCDs and showed that systemic mastocytosis was the only studied MCDs associated with osteoporotic vertebral fractures.

Introduction: To describe osteoporosis (OP) and fragility fractures in mast cell disorders (MCDs). Read More

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http://dx.doi.org/10.1007/s00198-019-04918-7DOI Listing
March 2019
1 Read

World Health Organization class-independent risk categorization in mastocytosis.

Blood Cancer J 2019 Mar 4;9(3):29. Epub 2019 Mar 4.

Divisions of Hematology, Departments of Internal Medicine and Laboratory Medicine, Mayo Clinic, Rochester, MN, USA.

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http://dx.doi.org/10.1038/s41408-019-0189-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6399221PMC
March 2019
1 Read

Imported fire ant hypersensitivity and mastocytosis: A case series of successful venom immunotherapy.

Ann Allergy Asthma Immunol 2019 Mar 1. Epub 2019 Mar 1.

Allergy/Immunology, Becker Ear, Nose, and Throat Center, Robbinsville, New Jersey.

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http://dx.doi.org/10.1016/j.anai.2019.02.018DOI Listing

Management of a neonate with diffuse cutaneous mastocytosis: Case report and literature review.

Pediatr Dermatol 2019 Mar 3. Epub 2019 Mar 3.

Department of Internal Medicine (Dermatology), Dell Medical School at The University of Texas at Austin, Austin, Texas.

Mastocytosis is an accumulation of clonal mast cells within tissues, commonly caused by mutations in the KIT proto-oncogene. This report describes the management of a neonate with diffuse cutaneous mastocytosis (DCM) caused by a rare activating KIT mutation, specifically internal tandem duplication of the Ala502Tyr503 pair on exon 9, and reviews current data regarding work-up of DCM in pediatric patients. Read More

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http://dx.doi.org/10.1111/pde.13801DOI Listing

Mastocytosis with Bee Sting Anaphylaxis.

Authors:
Dennis K Ledford

J Allergy Clin Immunol Pract 2019 Apr 26;7(4):1374-1375. Epub 2019 Feb 26.

University of South Florida, Morsani College of Medicine, Tampa, Fla. Electronic address:

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http://dx.doi.org/10.1016/j.jaip.2019.01.047DOI Listing

Pediatric cutaneous mastocytosis and mutation screening.

Allergy Asthma Proc 2019 Mar;40(2):123-128

From the Pediatric Allergy and Clinical Immunology Department, Health Science University Dr. Sami Ulus Maternity and Children Training and Research Hospital, Ankara, Turkey.

Cutaneous mastocytosis (CM) is a heterogeneous disease that commonly presents with skin lesions in childhood. In this study, we aimed to evaluate the clinical and laboratory test results of our patients with CM to ascertain prognostic factors by using patients' long-term follow-up results and to determine c-KIT (receptor tyrosine kinase) mutation from peripheral blood samples, which might be responsible for the etiopathogenesis of pediatric mastocytosis. The clinical observation data of 32 children who had been diagnosed with CM were retrospectively researched. Read More

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http://dx.doi.org/10.2500/aap.2019.40.4201DOI Listing
March 2019
10 Reads

In vitro and in vivo efficacy of an anti-CD203c conjugated antibody (AGS-16C3F) in mouse models of advanced systemic mastocytosis.

Blood Adv 2019 Feb;3(4):633-643

INSERM, Unité Mixte de Recherche (UMR) 1170, Villejuif, France.

Antibody-drug conjugates (ADCs) are a new class of therapeutics that use antibodies to deliver potent cytotoxic drugs selectively to cancer cells. CD203c, an ecto-nucleotide pyrophosphatase-phosphodiesterase 3, is overexpressed on neoplastic mast cells (MCs) in systemic mastocytosis (SM), thus representing a promising target for antibody-mediated therapy. In this study, we have found that human neoplastic MC lines (ROSA and ROSA), which express high levels of CD203c, are highly and specifically sensitive to the antiproliferative effects of an ADC against CD203c (AGS-16C3F). Read More

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http://dx.doi.org/10.1182/bloodadvances.2018026179DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6391676PMC
February 2019
17 Reads

Acute Myeloid Leukemia with Occult Systemic Mastocytosis or Atypical Mast Cells Post-induction.

Cureus 2018 Dec 14;10(12):e3734. Epub 2018 Dec 14.

Oncology, Jinnah Postgraduate Medical Centre, Karachi, PAK.

Systemic mastocytosis (SM) is a state of disease that is related to the clonal, neoplastic proliferation of mast cells. Patients who present with SM-Acute Myeloid Leukemia (AML) often have the worst outcome. We present a case of an 18-year-old female who was diagnosed with AML (FLT3 (Fms like tyrosine kinase 3) and PML-RARA (promyelocytic leukemia-retinoic acid receptor alpha) translocation-negative) and after initial treatment with a standard induction regimen of cytarabine and daunorubicin (3+7 regimen), her bone marrow showed blast cells less than 5% and dense aggregates/sheets of atypical/immature mast cells with immunohistochemical stain CD117+ve and toluidine blue positive in mast cell aggregates. Read More

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http://dx.doi.org/10.7759/cureus.3734DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6384037PMC
December 2018
2 Reads

Arterio-venous fistula-expanding role for venous access in mastocytosis patients.

J Surg Case Rep 2019 Feb 19;2019(2):rjz053. Epub 2019 Feb 19.

Department of Vascular Surgery, Joondalup Health Campus, Joondalup, WA, Australia.

Long-term central venous access is increasingly common as there are growing number of patients suffering from conditions that require repeated infusions for various indications. However, central venous access has its downside where there is a risk of infection and thrombosis (F Pinelli, E Cecero, D Del'Innocenti, V Selmi, R Giua, G Villa , 'Infection of totally implantable venous access devices: a review of literature,' 2018;: 230-42.). Read More

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http://dx.doi.org/10.1093/jscr/rjz053DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6380077PMC
February 2019

Fatigue in Mastocytosis: A Case Series.

Clin Ther 2019 Feb 21. Epub 2019 Feb 21.

Clinical Immunology Unit, Department of Internal Medicine, Stavanger University Hospital, Stavanger, Norway; Department of Clinical Science, Faculty of Medicine, University of Bergen, Bergen, Norway. Electronic address:

Purpose: Accumulating evidence suggests that fatigue in chronic inflammatory diseases is generated in the brain by mechanisms involving proinflammatory cytokines. We recently reported a high prevalence of fatigue in patients with mastocytosis, a condition with a constant activation of mast cells and release of a variety of bioactive substances. This observation indicates that mast cells somehow could be involved in the biological mechanisms that generate fatigue. Read More

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http://dx.doi.org/10.1016/j.clinthera.2019.01.016DOI Listing
February 2019
1 Read

Anaphylaxis to quinolones in mastocytosis: Hypothesis on the mechanism.

J Allergy Clin Immunol Pract 2019 Feb 20. Epub 2019 Feb 20.

Division of Rheumatology, Immunology and Allergy, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Mass.

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http://dx.doi.org/10.1016/j.jaip.2019.01.059DOI Listing
February 2019

Desensitization therapy using 'Mariana Castells' protocol in a patient with multiple autoimmune disorders- does it work?

J Community Hosp Intern Med Perspect 2019 11;9(1):53-54. Epub 2019 Feb 11.

Department of Internal Medicine, North Florida Regional Medical Center, University of Central Florida (Gainesville), Gainesville, USA.

Clinical use of antibiotics is becoming more widespread with each passing day for various infectious diseases. This has caused an abrupt increase in hypersensitivity reactions linked to these drugs, sometimes preventing the use of first-line therapies. In these patients, clinical presentation may vary from mild skin infections to life-threatening anaphylactic reactions. Read More

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http://dx.doi.org/10.1080/20009666.2018.1528107DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6374938PMC
February 2019
3 Reads

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, South 1, West 16, Chuo-ku, Sapporo, 060-8543, Japan.

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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
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Vomiting and Dehydration in a 2-Year-Old.

Pediatrics 2019 Mar 12;143(3). 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
March 2019
7 Reads

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

Am J Health Syst Pharm 2019 02;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 Apr 11;58(4):E79-E80. 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
April 2019
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Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome.

J Allergy Clin Immunol Pract 2019 Apr 5;7(4):1125-1133.e1. 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
April 2019
31 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
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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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6387409PMC
January 2019
11 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
14 Reads