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Topics in Companion Animal Medicine Nov 2020Mast cell tumor (MCT) or mastocytoma is one of the most frequent malignant cutaneous tumors in the dog, and the second most frequent in the cat. Several mast cell tumor...
Mast cell tumor (MCT) or mastocytoma is one of the most frequent malignant cutaneous tumors in the dog, and the second most frequent in the cat. Several mast cell tumor therapeutic approaches have been proposed in the past years for dogs and cats, resulting in very distinct outcomes. The current comprehensive literature review presents a critical approach to the scientific information published about the MCTs treatments and the subsequent prognosis and survival times, in dogs and in cats diagnosed with MCTs. A systematic review of the literature following the Cochrane principles and methodology was performed. The authors resorted to MEDLINE, Scopus, Google Scholar and Web of Science databases to select the 133 publications with evidence-based treatments for MCTs in companion animals. Results of the review suggest that the recommended treatment, prognosis and survival times for dogs and cats with MCTs depends at all times on the clinical staging, histological grade and location of the tumor.
Topics: Animals; Cat Diseases; Cats; Dog Diseases; Dogs; Mastocytoma; Skin Neoplasms; Treatment Outcome
PubMed: 32891740
DOI: 10.1016/j.tcam.2020.100472 -
Postgraduate Medicine Nov 2017Mastocytosis, a heterogeneous group of disorders, is characterized by an abnormal increase in the number of mast cells that is limited to the skin (cutaneous... (Meta-Analysis)
Meta-Analysis Review
INTRODUCTION
Mastocytosis, a heterogeneous group of disorders, is characterized by an abnormal increase in the number of mast cells that is limited to the skin (cutaneous mastocytosis), involving extracutaneous tissues (systemic mastocytosis), or presenting as solid tumours (mastocytoma and mast cell sarcoma). Recent studies estimate that 1 in 10,000 people are diagnosed with mastocytosis. Although prompt diagnosis and appropriate management are crucial, little is known about the natural history and currently there are no established management guidelines. We have conducted a systematic review to assess the natural history and management of different mastocytosis subtypes.
METHODS
A systematic review and meta-analysis were conducted using the PubMed and Ovid database of studies published in English and French over the last fifteen years, from January 2001 to December 2016. Keywords 'Cutaneous mastocytosis', 'Systemic mastocytosis', 'pathophysiology', 'clinical course', 'prognosis', 'drug therapy', and 'therapy' were searched. Rate of complete resolution was subjected to pooled analysis for different mastocytosis subtypes. Meta-analysis was conducted using Stata version 12.0.
RESULTS
We reviewed 634 papers, of which 5 were included in the analysis of resolution, and 138 were included in the assessment of management. Pooled estimate for rate of complete resolution varied depending on the mastocytosis subtype. In cutaneous mastocytosis, the complete resolution rate for mastocytoma was 10% per year (95% CI: 4.8%, 15.1%) while the rate for urticaria pigmentosa was 1.9% per year (95% CI: -0.5%, 4.3%). Diffuse cutaneous mastocytosis and systemic mastocytosis subtypes did not show evidence of complete resolution in the studies reviewed. Treatment of cutaneous and systemic mastocytosis is purely symptomatic with topical corticosteroids, antihistamines, omalizumab and imatinib being common choices.
CONCLUSION
Rate of resolution of mastocytosis is only shown in urticaria pigmentosa and mastocytoma. Better management guidelines are required to improve the health of these patients.
Topics: Adrenal Cortex Hormones; Biological Products; Combined Modality Therapy; Histamine Antagonists; Humans; Mastocytosis; Mastocytosis, Cutaneous; Mastocytosis, Systemic; Phototherapy
PubMed: 28770635
DOI: 10.1080/00325481.2017.1364124 -
Clinical and Translational Allergy Dec 2023Mastocytosis manifests with multisystemic symptoms, often involving the nervous system. Numerous cognitive, neuropsychiatric and neurological alterations have been... (Review)
Review
BACKGROUND
Mastocytosis manifests with multisystemic symptoms, often involving the nervous system. Numerous cognitive, neuropsychiatric and neurological alterations have been reported in multiple observational studies.
METHODS
We performed a qualitative systematic literature review of reported data consulting the electronic databases Medline, Scopus, Web of Science, Cochrane, and BASE until June 2023.
RESULTS
We selected 24 studies in which the majority showed that a high proportion of mastocytosis patients suffer cognitive, neuropsychiatric and neurological alterations. The most common disorders and estimated ranges of frequency observed in adults were depression (68%-75%), anxiety, high stress or irritability (27%-54%), cognitive impairment (27%-39%, primarily affecting memory skills), and headaches (55%-69%). Attention challenges and learning difficulties were reported in children at a rate of 13%, while neurodevelopmental disorders occurred at rates of 8%-12%. Frequent white abnormalities in mastocytosis patients with concomitant psychocognitive symptoms have been reported although neuroimaging studies have been performed rarely in this population.
CONCLUSION
Further studies with more comprehensive and homogeneous evaluations and neuroimaging and histological analysis should be performed for a better understanding of these manifestations. An earlier detection and proper management of these symptoms could greatly improve the quality of life of these patients.
PubMed: 38146805
DOI: 10.1002/clt2.12319 -
The British Journal of Dermatology Mar 2015Paediatric mastocytosis was previously considered to be a benign and spontaneously regressing disease. However, this evolution is impossible to predict. To clarify the... (Review)
Review
Paediatric mastocytosis was previously considered to be a benign and spontaneously regressing disease. However, this evolution is impossible to predict. To clarify the characteristics and course of paediatric mastocytosis, we performed a literature review of 1747 cases published between 1950 and April 2014. Lesions occurred before the age of 2 years in 90% of cases, and presented as urticaria pigmentosa (75% of cases), mastocytoma (20%) or diffuse cutaneous mastocytosis (5%). The male-to-female ratio was 1·4. KIT D816V mutation was detected in 34% of 215 tested patients. Clinical regression (complete or partial) occurred in 67% of cases and stabilization in 27%. However, the outcome was fatal in 2·9% of patients.
Topics: Age of Onset; Biopsy; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Mastocytosis, Cutaneous; Mutation; Pregnancy; Prognosis; Proto-Oncogene Proteins c-kit; Urticaria Pigmentosa
PubMed: 25662299
DOI: 10.1111/bjd.13567 -
Journal of Clinical Medicine Aug 2022The term mastocytosis refers to a heterogeneous group of disorders characterised by accumulation of clonal mast cells in different organs, most commonly in the skin.... (Review)
Review
The term mastocytosis refers to a heterogeneous group of disorders characterised by accumulation of clonal mast cells in different organs, most commonly in the skin. Little is known about the role of dermoscopy in the diagnostics of mastocytosis. To date, no systematic review on the dermoscopic features of cutaneous mastocytosis has been performed. The aim of this study was to summarise the current knowledge in the field as well as to identify the knowledge gaps to show possible directions for further studies, based on a systematic search of PubMed, Scopus, and Web of Science databases and related references published before 3 January 2022. Dermoscopic features, type of dermoscope, polarisation mode, magnification, and number of cases were analysed. In total, 16 articles were included in this review (3 case series and 13 case reports), analysing 148 patients with different variants of cutaneous mastocytosis; all of the studies analysed had a low level of evidence (V). The main dermoscopic features of urticaria pigmentosa included brown structureless areas, brown lines arranged in a network, and linear vessels distributed in a reticular pattern, with this last finding also being typical of telangiectasia macularis eruptiva perstans. The presence of either circumscribed yellow structureless areas or diffuse yellowish background was a constant pattern of mastocytoma, while nodular, pseudoangiomatous xanthelasmoid, and plaque-type mastocytosis were typified by light-brown structureless areas and/or pigment network, though the first two variants also showed yellow/yellow-orange structureless areas. Finally, pigmented streaks of radial distribution surrounding hair follicles were described to be a pathognomonic dermoscopic feature of pseudoxanthomatous mastocytosis. Although this review shows that the various clinical forms of cutaneous mastocytosis may feature diagnostic dermoscopic clues, it also underlines the need for further investigation as several relevant data are missing, including evaluation of dermoscopic pattern according to anatomical locations or "lesion age", studies on rare mastocytosis variants, evaluation of the prognostic role of dermoscopy in the context of systemic involvement, and comparative analyses with common clinical mimickers.
PubMed: 36012900
DOI: 10.3390/jcm11164649 -
Systemic Mastocytosis, Kounis Syndrome and Coronary Intervention: Case Report and Systematic Review.Heart, Lung & Circulation Aug 2017A 72-year-old male reported a long-standing history of unexplained syncope. Stress echocardiography demonstrated inducible anterior hypokinesis, and he proceeded to... (Review)
Review
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. Our patient was discharged 56days after his cardiac arrest on appropriate immunotherapy, and has made an excellent event-free recovery. Systemic mastocytosis is the pathological accumulation of mast cells in organs, and it may cause life-threatening syncope and cardiac arrests. It is estimated to affect up to 1 in 10,000 people, however is often underdiagnosed. No previous reviews have examined cardiac manifestations of systemic mastocytosis. We undertook a structured systematic review of cardiac presentations of systemic mastocytosis in adults, screening 619 publications. Twenty-three cases met inclusion criteria; our review suggests that short-term mortality is high (22%), and patients with cardiac presentations are predominantly male (83%). Unexplained cardiac arrest (26%) may be the first presentation of this haematological disorder. From our review of the literature, we have also derived suggested management approaches for cardiologists encountering or suspecting systemic mastocytosis in a variety of clinical scenarios.
Topics: Aged; Coronary Angiography; Heart Arrest; Humans; Immunotherapy; Kounis Syndrome; Male; Mastocytosis, Systemic; Percutaneous Coronary Intervention
PubMed: 28242292
DOI: 10.1016/j.hlc.2016.12.009 -
American Journal of Therapeutics
Meta-Analysis
Topics: Humans; Mastocytosis, Systemic; Staurosporine; Antineoplastic Agents
PubMed: 35446286
DOI: 10.1097/MJT.0000000000001508 -
Journal of Clinical Medicine Feb 2023(1) Background: Systemic mastocytosis is a rare, non-curable disease with potential life-threatening complications in patients receiving cardiac surgery. (2) Methods:... (Review)
Review
(1) Background: Systemic mastocytosis is a rare, non-curable disease with potential life-threatening complications in patients receiving cardiac surgery. (2) Methods: This systematic review of the literature was prompted by the case of a life-threatening anaphylactic reaction during cardiac surgery related to systemic mastocytosis. The search of all types of studies, using several databases (Pubmed, Scopus and Web of Science), was conducted through September 2022 to identify the relevant studies. (3) Results: Twelve studies were included describing cases of patients undergoing cardiac surgery who were diagnosed with systemic mastocytosis. An adverse effect, namely anaphylaxis, has happened in three cases. Different strategies of premedication, intraoperative and postoperative management were used. In our case, the patient was admitted for elective biological aortic valve replacement due to severe aortic stenosis. Intraoperatively, the patient developed an anaphylactic shock during the administration of protamine after separation from the cardiopulmonary bypass. This anaphylaxis reaction was a complication of the pre-existing systemic mastocytosis and could be successfully managed by the administration of epinephrine, antihistamines and corticosteroids. (4) Conclusions: This systematic literature search and case report highlight the importance of careful preoperative planning, as well as coordination between cardiac surgeons, anesthesiologists and hemato-oncological specialists, in patients with rare but complication-prone diseases such as systemic mastocytosis.
PubMed: 36769805
DOI: 10.3390/jcm12031156 -
Clinical and Experimental Allergy :... Jun 2020BACKGROUND: Mastocytosis is associated with mast cell (MC) mediator-related symptoms for which limited therapies are available. OBJECTIVE: Our aim was to assess the...
BACKGROUND: Mastocytosis is associated with mast cell (MC) mediator-related symptoms for which limited therapies are available. OBJECTIVE: Our aim was to assess the efficacy and safety of omalizumab in the treatment of MC mediator-related symptoms in adult patients with mastocytosis. RESULTS: We identified one multi-centre retrospective cohort study (39 patients), one retrospective cohort study (13 patients), 4 case series and 10 case reports. No published controlled randomized study was identified. We included 69 patients (13 patients with cutaneous mastocytosis and 56 with systemic mastocytosis). The mean age was 48 years. Omalizumab maintenance dose was 300 mg for the majority of patients. The mean duration of treatment was 17 months. Treatment led to a tolerability of venom immunotherapy and to a complete resolution of severe reactions in all patients with post-honeybee sting anaphylaxis. Complete resolution of idiopathic anaphylaxis episodes was noted in 84% of the patients. Complete resolution of palpitations, gastrointestinal, cutaneous, neuropsychiatric, respiratory and musculoskeletal symptoms was observed at a rate of 43%, 29%, 27%, 11%, 9% and 0%, respectively. Efficacy was maintained for the entire duration of the treatment in all but four responders. Adverse events were reported for 13 patients. CONCLUSIONS AND CLINICAL RELEVANCE: Omalizumab appears to prevent some life-threatening reactions associated with mastocytosis and may be a good option to treat the associated symptoms. However, the evidence relied upon is observational, uncontrolled and from a small number of patients. A randomized controlled trial is needed to better understand the place of omalizumab in mastocytosis treatment.
Topics: Adult; Female; Humans; Male; Mastocytosis; Middle Aged; Omalizumab
PubMed: 32107810
DOI: 10.1111/cea.13592 -
Allergy Sep 2015Primary mast cell activation syndromes (MCAS) are a group of disorders presenting with symptoms of mast cell mediator release. (Review)
Review
BACKGROUND
Primary mast cell activation syndromes (MCAS) are a group of disorders presenting with symptoms of mast cell mediator release.
OBJECTIVES
To assess the effectiveness and safety of orally administered H1 -antihistamines in the treatment of primary MCAS compared with placebo and other pharmacologic treatments.
METHODS
We systematically searched five databases and three trial repositories and contacted an international panel of experts to identify published and unpublished trials.
RESULTS
A total of 36 potentially relevant studies were identified. Of these, five crossover trials, enrolling a total of 71 patients (63 adults), met the eligibility criteria. All five of these studies were judged to be at moderate or high risk of bias. Two studies compared an H1 -antihistamine with placebo, two compared two different H1 -antihistamines, and one study compared H1 - and H2 -antihistamines with oral cromolyn sodium. Four of the five randomized controlled trials were historic (reported from 1983-1993), small (enrolling 8-15 patients), and used agents and/or dosing regimens that are now less commonly used in clinical practice (i.e. azelastine, chlorpheniramine, hydroxyzine, and ketotifen). The fifth trial, which enrolled 33 adults with cutaneous and systemic mastocytosis found 4 weeks of treatment with the second-generation H1 -antihistamine rupatadine, compared with placebo, resulted in significant improvements in quality of life, symptom control (itching, wheals and flares, flushing, tachycardia, and headache, but not gastrointestinal symptoms), and reduction in itching and whealing after standardized skin provocation to elicit Darier's sign.
CONCLUSIONS
There is an urgent need for large, well-designed, double-blind, placebo-controlled randomized trials investigating the effectiveness, cost-effectiveness, and safety of second-generation H1 -antihistamines in treatment of primary MCAS.
Topics: Disease Management; Histamine H1 Antagonists; Humans; Mast Cells; Mastocytosis; Randomized Controlled Trials as Topic; Syndrome; Treatment Outcome
PubMed: 26095756
DOI: 10.1111/all.12672