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Mastocytosis

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https://www.readbyqxmd.com/read/28332308/ceacam1-long-isoform-has-opposite-effects-on-the-growth-of-human-mastocytosis-and-medullary-thyroid-carcinoma-cells
#1
Chiyuki Ueshima, Tatsuki R Kataoka, Yusuke Takei, Masahiro Hirata, Akihiko Sugimoto, Mitsuyoshi Hirokawa, Yoshimichi Okayama, Richard S Blumberg, Hironori Haga
Carcinoembryonic antigen-related cell adhesion molecule 1 (CEACAM1) is expressed in a number of tumor cell types. The immunoreceptor tyrosine-based inhibitory motif (ITIM)-containing isoforms of this molecule which possess a long cytoplasmic tail (CEACAM1-L) generally play inhibitory roles in cell function by interacting with Src homology 2 domain-containing tyrosine phosphatase (SHP)-1 and/or SHP-2. Src family kinases (SFKs) are also known to bind to and phosphorylate CEACAM1-L isoforms. Here, we report that CEACAM1 was uniquely expressed at high levels in both human neoplastic mast cells (mastocytosis) and medullary thyroid carcinoma cell (MTC) lines, when compared with their expression in nonneoplastic mast cells or nonneoplastic C cells...
March 23, 2017: Cancer Medicine
https://www.readbyqxmd.com/read/28328617/cutaneous-mastocytosis-with-atypical-mast-cells-in-a-7-year-old-girl
#2
Gabriel Marrero Alemán, Constantin El Habr, Diana Islas Norris, Társila Montenegro Dámaso, Leopoldo Borrego, Omar P Sangueza
Cutaneous mastocytosis is defined by the presence of mast cells within the skin in the absence of other criteria for the diagnosis of systemic mastocytosis. Mast cells are characterized by an abundant granular cytoplasm and a round to oval or spindle-shaped nuclei. The presence of mast cells with bilobed and multilobed nuclei in cutaneous mastocytosis is a rare phenomenon and has been rarely reported in the literature. To our knowledge, there are only 4 reported cases of cutaneous mastocytosis with atypical mast cells...
April 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28286795/systemic-mastocytosis-complicated-by-non-cirrhotic-portal-hypertension-and-variceal-bleeding
#3
Thomas R McCarty, Adelina Hung, Arpan Mohanty, John I Allen
Systemic mastocytosis is a myeloproliferative disorder characterized by extracutaneous involvement of at least one organ. Although rare, infiltration of inflammatory mast cells within the portal vein may lead to obstruction of the sinusoids resulting in non-cirrhotic portal hypertension. We present a patient with known history of systemic mastocytosis with bone marrow involvement presenting with new-onset esophageal variceal bleeding. Although systemic mastocytosis is uncommon, the subsequent development of hepatic involvement and non-cirrhotic portal hypertension are discussed...
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28262030/highly-sensitive-assays-are-mandatory-for-the-differential-diagnosis-of-patients-presenting-with-symptoms-of-mast-cell-activation-diagnostic-work-up-of-38-patients
#4
Bea Van den Poel, Anne-Marie Kochuyt, Elke Del Biondo, Barbara Dewaele, Els Lierman, Thomas Tousseyn, Gert de Hertogh, Peter Vandenberghe, Nancy Boeckx
Mastocytosis is a heterogeneous disease caused by excessive mast cell (MC) proliferation. Diagnosis of systemic mastocytosis (SM) is based on the presence of major and minor criteria defined by the World Health Organization. Symptoms of MC activation can also occur in patients without SM or without allergic or inflammatory disease. These MC activation syndromes (MCAS) can be divided into primary (monoclonal) MCAS (MMAS) vs. secondary and idiopathic MCAS. In this single center study, the diagnostic work-up of 38 patients with a clinical suspicion of SM and/or with elevated basic tryptase levels is presented...
March 6, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28258965/assessment-of-in-vivo-mast-cell-reactivity-in-patients-with-systemic-mastocytosis
#5
T Gülen, C Möller Westerberg, K Lyberg, M Ekoff, J Kolmert, J Bood, J Öhd, A James, S-E Dahlén, G Nilsson, B Dahlén
BACKGROUND: Patients with systemic mastocytosis (SM) have clinical signs of mast cell (MC) activation and increased levels of MC mediators. It is unclear whether the increased mediator levels are caused by increased numbers of tissue MCs, or whether these cells in affected individuals have a hyperactive phenotype. OBJECTIVE: To determine reactivity of the skin and the airways to directly acting mediators and indirectly acting mast cell secretagogues in subjects with SM...
March 4, 2017: Clinical and Experimental Allergy: Journal of the British Society for Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28255023/the-clinical-and-molecular-diversity-of-mast-cell-leukemia-with-or-without-associated-hematologic-neoplasm
#6
Mohamad Jawhar, Juliana Schwaab, Manja Meggendorfer, Nicole Naumann, Hans-Peter Horny, Karl Sotlar, Torsten Haferlach, Karla Schmitt, Alice Fabarius, Peter Valent, Wolf-Karsten Hofmann, Nicholas C P Cross, Georgia Metzgeroth, Andreas Reiter
Mast cell leukemia is a rare variant of advanced systemic mastocytosis characterized by ≥20% mast cells in a bone marrow smear. We evaluated clinical and molecular characteristics of 28 patients with (n=20, 71%) or without an associated hematologic neoplasm. De novo mast cell leukemia was diagnosed in 16/28 (57%) patients and secondary mast cell leukemia evolving from other advanced systemic mastocytosis subtypes in 12/28 (43%) patients, of which 7 patients progressed while on cytoreductive treatment. Median bone marrow mast cell infiltration was 65% and median serum tryptase was 565 microg/L...
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28254862/advances-in-the-classification-and-treatment-of-mastocytosis-current-status-and-outlook-toward-the-future
#7
REVIEW
Peter Valent, Cem Akin, Karin Hartmann, Gunnar Nilsson, Andreas Reiter, Olivier Hermine, Karl Sotlar, Wolfgang R Sperr, Luis Escribano, Tracy I George, Hanneke C Kluin-Nelemans, Celalettin Ustun, Massimo Triggiani, Knut Brockow, Jason Gotlib, Alberto Orfao, Lawrence B Schwartz, Sigurd Broesby-Olsen, Carsten Bindslev-Jensen, Petri T Kovanen, Stephen J Galli, K Frank Austen, Daniel A Arber, Hans-Peter Horny, Michel Arock, Dean D Metcalfe
Mastocytosis is a term used to denote a heterogeneous group of conditions defined by the expansion and accumulation of clonal (neoplastic) tissue mast cells in various organs. The classification of the World Health Organization (WHO) divides the disease into cutaneous mastocytosis, systemic mastocytosis, and localized mast cell tumors. On the basis of histomorphologic criteria, clinical parameters, and organ involvement, systemic mastocytosis is further divided into indolent systemic mastocytosis and advanced systemic mastocytosis variants, including aggressive systemic mastocytosis and mast cell leukemia...
March 15, 2017: Cancer Research
https://www.readbyqxmd.com/read/28246420/significance-of-cytological-smear-evaluation-in-diagnosis-of-splenic-mast-cell-tumor-associated-systemic-mastocytosis-in-a-cat-felis-catus
#8
Moges Woldemeskel, Anita Merrill, Cindy Brown
An 8-year-old cat was presented with vomiting and weight loss. Histopathology and cytology revealed systemic mastocytosis, a rare condition and a clinical challenge. This case emphasizes the significance of cytological evaluation of smears in diagnosis of mastocytosis and in confirmation in biopsy specimens.
March 2017: Canadian Veterinary Journal. la Revue Vétérinaire Canadienne
https://www.readbyqxmd.com/read/28242292/systemic-mastocytosis-kounis-syndrome-and-coronary-intervention-case-report-and-systematic-review
#9
REVIEW
Elizabeth D Paratz, Nancy Khav, Andrew T Burns
A 72-year-old male reported a long-standing history of unexplained syncope. Stress echocardiography demonstrated inducible anterior hypokinesis, and he proceeded to percutaneous coronary intervention for an 80% stenosis of the left anterior descending artery. Thirty minutes post-procedure, he experienced a pulseless electrical activity (PEA) cardiac arrest. Urgent repeat angiography demonstrated profound coronary artery spasm consistent with Kounis syndrome. Three days later, a second PEA arrest occurred. Systemic mastocytosis was ultimately diagnosed as the cause of his recurrent syncopal episodes and cardiac arrests...
February 15, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28229176/denosumab-for-the-treatment-of-mastocytosis-related-osteoporosis-a-case-series
#10
Giovanni Orsolini, Irene Gavioli, Gaia Tripi, Ombretta Viapiana, Davide Gatti, Luca Idolazzi, Roberta Zanotti, Maurizio Rossini
The purpose of this study was to investigate the therapeutic effect of denosumab, an anti-RANKL monoclonal antibody for the treatment of bone loss in indolent systemic mastocytosis (ISM) patients intolerant to bisphosphonates. Four patients underwent upon informed consent a treatment with denosumab 60 mg administered subcutaneously every 6 months with the same regimen used for postmenopausal osteoporosis. Bone mineral density (BMD) was measured at lumbar and femoral sites at baseline and after 1 year. C-terminal telopeptide of collagen type I (CTX), bone alkaline phosphatase (bALP) and tryptase serum level were determined at baseline and after 12 months with fasting blood samples withdrawals...
February 22, 2017: Calcified Tissue International
https://www.readbyqxmd.com/read/28229121/intertriginous-maculopapular-mastocytosis-in-a-patient-with-acute-myeloid-leukemia
#11
María Herrero-Moyano, Tania Marusia Capusan, Alejandra Pérez-Plaza, Almudena Godoy, Javier Sánchez-Perez
No abstract text is available yet for this article.
January 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28185248/mastering-gut-permeability-new-roles-for-old-friends
#12
Michael Bramhall, Colby Zaph
Mast cells are innate immune cells that respond rapidly to infection in barrier tissues such as the skin and intestinal mucosa. Expulsion of parasitic worms in the gut involves a robust type 2 host response, and an acute mastocytosis is often generated at the site of infection. However, the role of mast cells in resistance to worm infections appears to be parasite specific. Mast cells are also involved in tissue repair, but the long-term contribution of mast cell activation after worm expulsion has not been definitively studied...
February 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28181948/kounis-syndrome-during-anesthesia-presentation-of-indolent-systemic-mastocytosis-a-case-report
#13
Elena de la Fuente Tornero, Arantza Vega Castro, Pedro Álvarez de Sierra Hernández, Javier Balaguer Recena, Sofía Carmen Zaragoza Casares, Francisco Miguel Serrano Baylin, Paloma Gallardo Culebradas, Beatriz Amorós Alfonso, Jose Ramón Rodríguez Fraile
Mastocytosis comprises a heterogeneous group of disorders characterized by mast cell accumulation and proliferation in distinct organs. Kounis syndrome is defined as the concurrence of acute coronary syndromes with mast cell activation in a setting of allergic or hypersensitivity reactions. This is the first reported case of an intraoperative Kounis syndrome as the onset of an indolent systemic mastocytosis probably triggered by succinylated gelatin infusion during general anesthesia. The presentation of this case is intended to contribute to the knowledge of mastocytosis and Kounis syndrome at the time of diagnostic workup during intraoperative anaphylaxis or myocardial ischemia...
February 8, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28181942/successful-management-of-a-patient-with-possible-mast-cell-activation-syndrome-undergoing-pulmonary-embolectomy-a-case-report
#14
Ellen W Richter, Kai-Ling Hsu, Vanessa Moll
We report the successful perioperative management of a patient with presumed mastocytosis undergoing pulmonary embolectomy. Postoperatively the patient went into vasodilatory shock, which was partly attributed to mast cell mediator release. H1- and H2-antagonists, steroids, and a single dose of methylene blue were given with improvement of hemodynamics. The patient was weaned off vasoactive substances and extubated by postoperative day 2. We discuss the perioperative management of patients with mastocytosis, briefly review the literature concerning anesthetic management for cardiac surgery in patients with this disorder, and discuss our patient's alternative but related diagnosis of idiopathic mast cell activation syndrome...
February 8, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28141734/anaesthesia-and-orphan-disease-rapid-sequence-induction-in-systemic-mastocytosis
#15
Christoph Unterbuchner, Marina Hierl, Timo Seyfried, Thomas Metterlein
No abstract text is available yet for this article.
March 2017: European Journal of Anaesthesiology
https://www.readbyqxmd.com/read/28135563/pathogenesis-and-pathology-of-mastocytosis
#16
Dean D Metcalfe, Yoseph A Mekori
Systemic mastocytosis is a clonal disorder of mast cells that may variably present with characteristic skin lesions, episodes of mast cell mediator release, and disturbances of hematopoiesis. No curative therapy presently exists. Conventional management has relied on agents that antagonize mediators released by mast cells, inhibit mediator secretion, or modulate mast cell proliferation. Recent advances in the molecular understanding of the pathophysiology of systemic mastocytosis have provided new therapeutic considerations, including new and novel tyrosine kinase inhibitors...
January 24, 2017: Annual Review of Pathology
https://www.readbyqxmd.com/read/28133781/longitudinal-study-of-pediatric-urticaria-pigmentosa
#17
Adam Heinze, Travis J Kuemmet, Yvonne E Chiu, Sheila S Galbraith
BACKGROUND/OBJECTIVES: Urticaria pigmentosa (UP) is the most common form of mastocytosis in children and is associated with systemic signs, symptoms, and triggers. To our knowledge, the effect of UP on children's quality of life has not been studied. The objective of the current study was to characterize the natural history, triggers, and complications of pediatric UP, identify prognostic indicators, and determine its effect on quality of life. METHODS: Between 2002 and 2007, children with three or more mastocytomas diagnosed by a pediatric dermatologist were recruited during visits at the Children's Hospital of Wisconsin Dermatology Clinic (Milwaukee, WI)...
March 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28112808/nerve-growth-factor-a-neuroimmune-crosstalk-mediator-for-all-seasons
#18
REVIEW
Stephen D Skaper
Neurotrophic factors comprise a broad family of biomolecules - most of which are peptides or small proteins - that support the growth, survival, and differentiation of both developing and mature neurons. The prototypical example and best-characterized neurotrophic factor is nerve growth factor (NGF) which is widely recognized as a target-derived factor responsible for the survival and maintenance of the phenotype of specific subsets of peripheral neurons and basal forebrain cholinergic nuclei during development and maturation...
January 23, 2017: Immunology
https://www.readbyqxmd.com/read/28095350/progressive-multifocal-leukoencephalopathy-in-a-patient-with-systemic-mastocytosis-treated-with-cladribine
#19
Karl B Alstadhaug, Randi Fykse Halstensen, Francis Odeh
BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic brain infection caused by the human polyomavirus JC (JCPyV). A particular problem with the drug cladribine seems to be prolonged suppression of the CD4+ T-cells, a well-known risk factor for PML. CASE DESCRIPTION: A 67-year-old male presented with a 3-weeks history of unsteady gait, dysarthria and a dysfunctional right arm. Seven years earlier, he had been diagnosed with urticaria pigmentosa, and 2 years later aggressive systemic mastocytosis...
March 2017: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/28074523/familial-gastrointestinal-stromal-tumors-lentigines-and-caf%C3%A3-au-lait-macules-associated-with-germline-c-kit-mutation-treated-with-imatinib
#20
Divya Gupta, Laxmisha Chandrashekar, Lidia Larizza, Elisa A Colombo, Laura Fontana, Cristina Gervasini, Devinder M Thappa, Medha Rajappa, Kalai Selvi Rajendiran, Gubbi Shamanna Sreenath, Vikram Kate
BACKGROUND: Familial lentiginosis syndromes are characterized by a wide array of manifestations resulting from activation of molecular pathways which control growth, proliferation, and differentiation of a broad range of tissues. Familial gastrointestinal stromal tumors (GISTs) are often accompanied by additional features like hyperpigmentation, mastocytosis, and dysphagia. They have been described with mutations in c-kit (most commonly), platelet-derived growth factor receptor A, neurofibromatosis-1, and succinate dehydrogenase genes...
February 2017: International Journal of Dermatology
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