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Pediatric mastocytosis

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https://www.readbyqxmd.com/read/27738395/current-approach-to-cutaneous-mastocytosis-in-childhood
#1
REVIEW
Zeynep Tamay, Deniz Özçeker
Mastocytosis is a heterogeneous disorder characterized by clonal proliferation and accumulation of mast cells in one of more organs which may lead to different clinical pictures. Pathological increase and activation of mast cells in various tissues can cause different clinical pictures. Cutaneous mastocytosis limited to the skin is the most typical clinical picture observed in children and systemic mastocytosis is very rare in the pediatric age group. The diagnosis of cutaneous mastocytosis is based on clinical findings, but is often delayed due to lack of clinical awareness of the disease and lack of its consideration in the differential diagnosis...
September 2016: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/27510692/-childhood-systemic-mastocytosis-associated-with-t-8-21-q22-q22-acute-myeloid-leukemia
#2
Nikhil Rabade, Prashant Tembhare, Nikhil Patkar, Pratibha Amare, Brijesh Arora, P G Subramanian, Sumeet Gujral
Systemic mastocytosis (SM) with associated clonal nonmast cell lineage disease is seen in up to 20% cases of SM. SM is uncommon in the pediatric population. T (8; 21) (q22; q22) is a good prognostic factor in acute myeloid leukemia (AML). However, the presence of SM confers poor prognosis in t (8; 21) (q22; q22) associated AML. We report the case of a child with t (8; 21) (q22; q22) associated AML with SM and her minimal residual disease status over the course of her treatment. In our case, the abnormal mast cells, showing co-expression of CD25 and CD2, persisted even after the marrow showed no evidence of residual AML...
July 2016: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/26476479/cutaneous-manifestations-in-patients-with-mastocytosis-consensus-report-of-the-european-competence-network-on-mastocytosis-the-american-academy-of-allergy-asthma-immunology-and-the-european-academy-of-allergology-and-clinical-immunology
#3
REVIEW
Karin Hartmann, Luis Escribano, Clive Grattan, Knut Brockow, Melody C Carter, Ivan Alvarez-Twose, Almudena Matito, Sigurd Broesby-Olsen, Frank Siebenhaar, Magdalena Lange, Marek Niedoszytko, Mariana Castells, Joanna N G Oude Elberink, Patrizia Bonadonna, Roberta Zanotti, Jason L Hornick, Antonio Torrelo, Jürgen Grabbe, Anja Rabenhorst, Boguslaw Nedoszytko, Joseph H Butterfield, Jason Gotlib, Andreas Reiter, Deepti Radia, Olivier Hermine, Karl Sotlar, Tracy I George, Thomas K Kristensen, Hanneke C Kluin-Nelemans, Selim Yavuz, Hans Hägglund, Wolfgang R Sperr, Lawrence B Schwartz, Massimo Triggiani, Marcus Maurer, Gunnar Nilsson, Hans-Peter Horny, Michel Arock, Alberto Orfao, Dean D Metcalfe, Cem Akin, Peter Valent
Cutaneous lesions in patients with mastocytosis are highly heterogeneous and encompass localized and disseminated forms. Although a classification and criteria for cutaneous mastocytosis (CM) have been proposed, there remains a need to better define subforms of cutaneous manifestations in patients with mastocytosis. To address this unmet need, an international task force involving experts from different organizations (including the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology) met several times between 2010 and 2014 to discuss the classification and criteria for diagnosis of cutaneous manifestations in patients with mastocytosis...
January 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/26160029/management-of-anesthesia-in-adult-and-pediatric-mastocytosis-a-study-of-the-spanish-network-on-mastocytosis-rema-based-on-726-anesthetic-procedures
#4
Almudena Matito, José Mario Morgado, Paula Sánchez-López, Iván Álvarez-Twose, Laura Sánchez-Muñoz, Alberto Orfao, Luis Escribano
BACKGROUND: The role of anesthesia as an elicitor of mast cell (MC) mediator release symptoms in mastocytosis is poorly investigated. OBJECTIVE: To determine the frequency and type of MC mediator release symptoms during anesthetic procedures in mastocytosis patients. METHODS: Medical records were reviewed regarding the anesthetic techniques for 501 mastocytosis patients (459 adults and 42 children; 95 and 5% with systemic involvement, respectively) who were subjected to 676 and 50 anesthetic techniques, respectively...
2015: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/26152315/large-maculopapular-cutaneous-lesions-are-associated-with-favorable-outcome-in-childhood-onset-mastocytosis
#5
Tim Wiechers, Anja Rabenhorst, Tina Schick, Liane M Preussner, Anja Förster, Peter Valent, Hans-Peter Horny, Karl Sotlar, Karin Hartmann
BACKGROUND: Mastocytosis, characterized by pathologic accumulation of mast cells, can manifest itself in adulthood or childhood. Pediatric patients usually have cutaneous mastocytosis (CM) with mast cell infiltrates limited to the skin and spontaneous improvement of skin lesions after several years. However, there are some patients with persistent disease resembling adulthood-onset mastocytosis. OBJECTIVE: The current classification of CM differentiates between 3 subforms...
December 2015: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/26139333/cutaneous-and-systemic-mastocytosis-in-children-a-risk-factor-for-anaphylaxis
#6
REVIEW
A Matito, M Carter
Childhood mastocytosis is usually a clonal mast cell disease related to activating mutations in KIT. The symptoms in childhood mastocytosis are typically cutaneous in nature although systemic symptoms including anaphylaxis due to the release of mast cells (MC) mediators can also manifest. The prevalence of anaphylaxis reported in childhood mastocytosis is higher than the rates reported in the pediatric general population, but lower than the prevalence of anaphylaxis described in adult mastocytosis. An extensive cutaneous involvement was reported as a risk factor for anaphylaxis, and patients with diffuse cutaneous mastocytosis have been documented to have more severe anaphylaxis symptoms...
May 2015: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/26044856/assessment-of-clinical-findings-tryptase-levels-and-bone-marrow-histopathology-in-the-management-of-pediatric-mastocytosis
#7
Melody C Carter, Sarah T Clayton, Hirsh D Komarow, Erica H Brittain, Linda M Scott, Daly Cantave, Donna M Gaskins, Irina Maric, Dean D Metcalfe
BACKGROUND: The management of children with pediatric mastocytosis poses a challenge. This is because there is limited information as to the application of clinical and laboratory findings and bone marrow histopathology as they relate to medical intervention and communication. OBJECTIVE: We sought to examine clinical aspects of pediatric mastocytosis in relationship to serum tryptase levels and bone marrow pathology to provide practical guidance for management. METHODS: Between 1986 and 2012, 105 children were evaluated at the National Institutes of Health...
December 2015: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/25952500/transglutaminase-2-expressed-in-mast-cells-recruited-into-skin-or-bone-marrow-induces-the-development-of-pediatric-mastocytosis
#8
Young Min Ahn, Gwan Ui Hong, Sang Hoon Kim, Ho Jung Lee, Hey Sung Baek, Myung Nam Kim, Kui Young Park, Jai Youl Ro
BACKGROUND: Mastocytosis is characterized by a pathological increase in mast cells in organs such as skin and bone marrow. Transglutaminase 2 (TG2) expressed in mast cells contributes to allergic diseases, but its role in mastocytosis has not been investigated. This study aimed to investigate whether TG2 contributes to pediatric mastocytosis. METHODS: Serum, various skin tissues or bone marrow (BM) biopsy and aspirates were obtained from pediatric normal control or patients with indolent systemic mastocytosis (SM), mastocytoma, and urticaria pigmentosa (UP)...
August 2015: Pediatric Allergy and Immunology
https://www.readbyqxmd.com/read/25779849/suplatast-tosilate-for-treating-cutaneous-mastocytosis
#9
Hiroki Yasudo, Tomoaki Ando, Mitsuteru Hiwatari, Akira Oka
Pediatric cutaneous mastocytosis is a rare disease caused by mast cell hyperplasia. We report the case of an infant diagnosed as cutaneous mastocytosis and seasonal allergies. The wheals, flushing, and pruritus of the mastocytosis were unresponsive to combination therapy with an antihistamine, a mast cell stabilizer (sodium cromoglycate), and a leukotriene antagonist. Addition of suplatast tosilate as a treatment for the seasonal allergy also dramatically improved his cutaneous symptoms and signs. Further trials of suplatast tosilate in selected cases of cutaneous mastocytosis are warranted...
May 2015: Pediatric Dermatology
https://www.readbyqxmd.com/read/25037281/association-of-mastocytosis-with-inflammatory-joint-diseases-a-series-of-31-patients
#10
MULTICENTER STUDY
Brigitte Bader-Meunier, Cristina Bulai Livideanu, Claire Larroche, Isabelle Durieu, Laure Artru, Anne Beucher, Grégoire Cormier, Divi Cornec, Michel Delarco, Jean-Jacques Dubost, Christophe Fontaine, Marie-Pierre Gourin, Rose-Marie Javier, Jean-Pierre de Jauréguiberry, Hervé Maisonneuve, Eric Toussirot, Valérie Ugo, Stéphane Echaubard, Nizar Mahlaoui, Achille Aouba, Christine Bodemer, Karine Briot, Laurent Frenzel, Olivier Lortholary, Marie-Olivia Chandesris, Olivier Hermine
OBJECTIVES: We studied the clinical phenotypes and tolerance to treatments in a series of patients affected by both inflammatory joint diseases and mastocytosis. METHODS: This retrospective multicenter study was conducted on behalf of 3 networks focused on mastocytosis, pediatric, and adults' inflammatory joint diseases. Patients who displayed both mastocytosis and inflammatory joint diseases were included. RESULTS: A total of 31 patients were included...
December 2014: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/25015329/identification-of-kit-m541l-somatic-mutation-in-chronic-eosinophilic-leukemia-not-otherwise-specified-and-its-implication-in-low-dose-imatinib-response
#11
Alessandra Iurlo, Umberto Gianelli, Alessandro Beghini, Orietta Spinelli, Nicola Orofino, Francesca Lazzaroni, Stefano Cambiaghi, Tamara Intermesoli, Alessandro Rambaldi, Agostino Cortelezzi
Activating mutations of KIT receptor tyrosine kinase have been reported in different neoplasms. The M541L KIT substitution (KIT(M541L)) has been described to be associated with pediatric mastocytosis, to enhance growth rate of the affected cells and to confer higher sensitivity to imatinib therapy. We investigated the presence of KIT(M541L) in five males with chronic eosinophilic leukemia, not otherwise specified (CEL, NOS), all negative for Platelet-derived growth factor-alpha (PDGFR) or PDGFRbeta abnormalities, which responded to imatinib therapy...
July 15, 2014: Oncotarget
https://www.readbyqxmd.com/read/24612340/severity-of-cutaneous-findings-predict-the-presence-of-systemic-symptoms-in-pediatric-maculopapular-cutaneous-mastocytosis
#12
Meredith Barnes, Livia Van, Laura DeLong, Leslie P Lawley
Although the prognosis of maculopapular cutaneous mastocytosis (MPCM), also referred to as urticaria pigmentosa, is often benign, clinicians lack evidence to reliably predict those at risk of associated systemic manifestations. We sought to elucidate clinical markers of disease severity to provide better treatment and prognostic information for individuals with MPCM. A retrospective chart review querying characteristics of children diagnosed with MPCM in the Emory Dermatology Clinic was performed. Follow-up was obtained through a clinical encounter or telephone interview...
May 2014: Pediatric Dermatology
https://www.readbyqxmd.com/read/24380017/pediatric-mastocytosis-a-review-of-the-literature
#13
Marianne Frieri, Mahvish Quershi
Mastocytosis has a bimodal distribution often presenting in children from birth to 2 years of age and in those over the age of 15. Pediatric mastocytosis is due to the effects of mast-cell degranulation enzymes such as histamine and tryptase causing the presentation of pruritis, flushing, vesicles, abdominal and bone pain, or headache. Three different forms of mastocytosis can occur: urticaria pigmentosa, diffuse cutaneous, and solitary mastocytoma. Systemic symptoms are typically a result of mast-cell mediator release but do not prove systemic mast-cell hyperplasia...
December 1, 2013: Pediatric Allergy, Immunology, and Pulmonology
https://www.readbyqxmd.com/read/24262698/mastocytosis
#14
REVIEW
Melody C Carter, Dean D Metcalfe, Hirsh D Komarow
Mastocytosis is a disorder of abnormal mast cell proliferation, with clinical features that include flushing, pruritus, abdominal pain, diarrhea, hypotension, syncope, and musculoskeletal pain. These features are the result of mast cell mediator release and infiltration into target organs. Patients of all ages may be affected, although in children, manifestations primarily involve the skin. Most patients with systemic disease have a somatically acquired activating mutation in the KIT oncogene. This article discusses the causes and pathogenesis of mastocytosis, with an overview of the clinical features and the approach to diagnosis, evaluation, and therapy in adults and pediatric patients...
February 2014: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/24150753/primary-mast-cell-disorders-in-children
#15
REVIEW
Ari J Fried, Cem Akin
Mastocytosis arises from clonal mast cell expansion and the resultant accumulation of mast cells in cutaneous and sometimes extracutaneous tissues. Recent studies have demonstrated that c-kit mutations seem to be more prevalent in pediatric mastocytosis than previously assumed, but what determines disease evolution and severity in the individual patient remains elusive. For the large majority of children, mastocytosis is a self-limited cutaneous disease that spontaneously regresses before they reach adult age...
December 2013: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/24035776/food-allergy-testing-in-eosinophilic-esophagitis-what-the-gastroenterologist-needs-to-know
#16
Seema S Aceves
Eosinophilic esophagitis (EoE) is a clinicopathologic disease of increasing prevalence in children and adults. The triggering antigen in EoE is often a food that initiates a cascade of Th2-associated interleukins such as interleukin-5 and interleukin-13 and chemokines such as eotaxin-3 as well as esophageal eosinophilia and mastocytosis. Amino acid-based formulas have high efficacy rates in EoE and constitute the first evidence for food-triggered esophageal eosinophilia. Animal models have demonstrated the sufficiency of food antigens in triggering both the inflammatory and remodeling complications of EoE...
August 2014: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/23659595/role-of-imatinib-in-the-treatment-of-pediatric-onset-indolent-systemic-mastocytosis-a-case-report
#17
Manoj Kumar Agarwala, Renu George, Vikram Mathews, Poonkuzhali Balasubramanian, Meera Thomas, Sheila Nair
BACKGROUND: Mastocytosis is a sporadic disease characterized by an abnormal accumulation of mast cells (MCs) in single or multiple organs. It has a heterogeneous clinical picture which is a reflection of underlying tissue MC burden, mediators released and the organs involved. Here, the authors report significant symptomatic, cutaneous and systemic response to imatinib in a case of childhood onset indolent D816V KIT unmutated systemic mastocytosis (SM). CASE REPORT: A 19-year-old female presented with a history of itchy skin lesions over the face, trunk and extremities since 6 months of age associated with recurrent bouts of angioedema...
December 2013: Journal of Dermatological Treatment
https://www.readbyqxmd.com/read/23623266/anti-il-5-therapy-reduces-mast-cell-and-il-9-cell-numbers-in-pediatric-patients-with-eosinophilic-esophagitis
#18
Iris M Otani, Arjun A Anilkumar, Robert O Newbury, Monica Bhagat, Lisa Y Beppu, Ranjan Dohil, David H Broide, Seema S Aceves
BACKGROUND: Eosinophilic esophagitis (EoE) is a clinicopathologic entity of increasing worldwide prevalence. IL-5 is essential for eosinophil trafficking, and anti-IL-5 therapy decreases esophageal eosinophilia. EoE is associated with prominent mast cell infiltration. OBJECTIVE: We investigated whether anti-IL-5 (mepolizumab) treatment reduced esophageal mast cell accumulation in biopsy specimens from pediatric patients with EoE from a previous randomized anti-IL-5 trial...
June 2013: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/23459373/extracellular-domain-c-kit-mutation-with-duplication-of-ser501ala502-found-in-gastrointestinal-stromal-tumors-is-more-imatinib-and-nilotinib-sensitive-than-that-with-duplication-of-ala502tyr503
#19
Ning-Ning Liu, Mizuka Ohkouchi, Yuka Hashikura, Noriko Kajimoto, Ikuo Matsuda, Koji Isozaki, Yasushi Toh, Tsuyoshi Takahashi, Toshirou Nishida, Seiichi Hirota
The great majority of gastrointestinal stromal tumors (GISTs) have gain-of-function mutations of the c-kit gene, which encodes KIT receptor tyrosine kinase. Most of the mutations are located at exon 11, but some are at exon 9 or at other exons. Mutation types at exon 11 vary, while most mutations at exon 9 are a particular duplication of Ala502Tyr503 (KIT-Dup-Ala502Tyr503). Recently a duplication of Ser501Ala502 (KIT-Dup-Ser501Ala502) at exon 9 has been reported in two cases of pediatric mastocytosis and one case of adult mast cell leukemia...
May 2013: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/23408045/anesthetic-considerations-in-pediatric-mastocytosis-a-review
#20
REVIEW
Norma J Klein, Shad Misseldine
Mastocytosis is an orphan disease rarely encountered by practicing anesthesiologists. Children with mastocytosis often present for procedures or surgery requiring anesthesia. Because many of the medications commonly used in pediatric anesthesia have been reported to initiate mast cell activation, parents are often very anxious about their child's perioperative experience. Laboratory investigations of serum histamine assays associated with different anesthetic drugs have not been shown to predict mast cell degranulation in these patients...
August 2013: Journal of Anesthesia
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