Presentation
They usually present within 2 years of age, mostly within first 3 months 1. [f1000research.com]
Based on this presentation, the child received a diagnosis of a solitary mastocytoma with no systemic involvement. [consultant360.com]
There is, at present, no evidence of a reproducibly effective therapy for this systemic disease, and symptoms can only be controlled by various medications. [link.springer.com]
These solitary collections of mast cells are most often present at birth, but they can also develop during the first few weeks of life. [aocd.org]
Presents a full-color design that includes clear illustrative examples of the relevant pathology and clinical issues, for quick access to the answers you need. Incorporates the codified WHO classification for all lymphomas and leukemias. [books.google.com]
Entire Body System
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Constitutional Symptom
In most cases, only yearly checkups are necessary, and no treatment is required; preventive recommendations are warranted in individuals with systemic disease and constitutional symptoms. 6 There is no cure for more severe forms of mastocytosis, but a [consultant360.com]
Respiratoric
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Pleural Effusion
J Intern Med 1991 May;229(5):475-7 Abstract quote A 68-year-old man presented with a 6-month history of fatigue, rhinorrhoea, pruritic skin lesions, left pleural effusion, ascites, oedema and weight loss of 10 kg. [thedoctorsdoctor.com]
Musculoskeletal
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Myalgia
These symptoms can include fatigue, syncope, abdominal discomfort, nausea, vomiting, diarrhea, flushing, anaphylaxis, hypotension, myalgia, bone pain, headache, ocular discomfort, and gastrointestinal malabsorption. [consultant360.com]
[…] texture Severe itching (pruritus) Systemic conditions such as: Abdominal pain Difficulty breathing Rapid heart rate (tachycardia) Headache Bone, joint, and muscle signs and symptoms such as: Bone pain (ostealgia) Joint pain (arthralgia) Muscle pain (myalgia [dovemed.com]
[…] self limited to aggressive Spectrum of symptoms depending on type Symptoms related to mast cell degranulation: episodic blisters, flushing, diarrhea, abdominal pain, musculoskeletal pain, hypotension, peptic ulcers Musculoskeletal symptoms: arthralgia, myalgia [pathologyoutlines.com]
Skin
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Urticaria
Gross P (1934) Urticaria pigmentosa (solitary lesion). Arch Dermatol Syph 29:451 Google Scholar 10. Jelinek JE, Penzinger H (1971) Urticaria pigmentosa in three siblings. Arch Dermatol 103:458 Google Scholar 11. [link.springer.com]
The increased number of mast cells in the skin may lead to diffuse cutaneous mastocytosis, maculopapular cutaneous mastocytosis (the most common form, which is also called urticaria pigmentosa), or a solitary mastocytoma (caused by a single collection [consultant360.com]
Differential diagnosis of urticaria pigmentosa Urticaria pigmentosa should be distinguished from urticaria with pigmentation, drug rash, reticulosis of skin, pigment nevus, histocytosis -X, xanthomatosis. [m.iliveok.com]
Urticaria pigmentosa Mastocytosis Localized mastocytosis • Focal: Single skin lesion: mast cell 'nevus' • Generalized: Urticaria pigmentosa Systemic mastocytosis • Indolent • Progressive • Malignant Mast cell leukemia Mast cell sarcoma Pathogenesis Some [medical-dictionary.thefreedictionary.com]
Severity and duration of aspirin-induced urticaria showed a positive correlation with urinary LTE4 excretion. Naproxen precipitated urticaria in 5 of 7 aspirin-sensitive patients and caused further increase in urinary LTE4. [aaaai.org]
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Dry Skin
On review of systems, there were no other complaints other than occasional dry skin, for which the mother used an over-the-counter soap for sensitive skin. There was no family history of similar lesions. [consultant360.com]
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Skin Atrophy
These immunomodulators offer advantages over corticosteroids in terms of a more selective action, no associated systemic side-effects, and the absence of associated skin atrophy, depigmentation and telangiectasia. [f1000research.com]
Neurologic
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Excitement
This exciting new, full-color reference provides you with the most comprehensive, authoritative, up-to-date information for diagnosing and treating children with cancer. [books.google.com]
Workup
Without specific symptoms relating to a particular organ system, an elaborate and extensive staging workup is not indicated. [link.springer.com]
Pleura
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Pleural Effusion
J Intern Med 1991 May;229(5):475-7 Abstract quote A 68-year-old man presented with a 6-month history of fatigue, rhinorrhoea, pruritic skin lesions, left pleural effusion, ascites, oedema and weight loss of 10 kg. [thedoctorsdoctor.com]
Treatment
Apply the latest best practices through new and updated treatment algorithms. Find therapeutic drug information more easily with guidance incorporated into each chapter. [books.google.com]
TESTS Skin biopsy Treatment Most patients do not receive treatment; mastocytomas often disappear with age. COMMON TREATMENT OPTIONS Oral Antihistamines: relieve itching Surgical Excision [knowcancer.com]
To date, no treatment is known to be effective for this disease, which is associated with short-term survival of the patients. [ncbi.nlm.nih.gov]
Ameet Dandale were involved in clinical diagnosis, work-up, treatment and writing up of this case report. [f1000research.com]
Prognosis
Mast cell sarcomas, part of the systemic forms of mastocytosis, are very rare tumors characterized by a destructive growth of highly atypical mast cells, with secondary spread, poor prognosis, and low survival rates. [ncbi.nlm.nih.gov]
Articles in this issue include Human Mast Cell Signal Transduction; Mast cell tryptase role in homeostasis and coagulation; Mastocytosis: Current Classification and Diagnostic criteria; Epidemiology, risk factors and prognosis of mastocytosis; Mast cell [books.google.com]
Most mastocytosis cases have a good overall prognosis and are characterized by low mast cell burden, particularly at the early stages of the disease with limited or no systemic involvement. [consultant360.com]
In SM-AHNMD, the prognosis depends on the course of the associated haematological disorder. Agressive systemic mastocytosis and mast cell leukaemia have a poorer prognosis. [patient.info]
In general, the prognosis of childhood mastocytosis is very good. [ecnm.net]
Etiology
(Etiology) The cause of Mastocytosis is usually genetic. Specifically, it is a small change (mutation) in the region of DNA of a receptor called C-KIT. [dovemed.com]
Extracutaneous mastocytoma Mast cell sarcoma (MSS) Epidemiology Uncommon Affects males more than females Bimodal age; affects pediatric age groups and adults Bone marrow and bone involvement are present in up to 90% of SM cases, followed by other organs Etiology [pathologyoutlines.com]
Etiology The etiology of mastocytosis remains unknown. Children who develop this disease usually have a self-limited disorder, whih often resolves before adulthood. [sdominko.tripod.com]
Etiology Mastocytosis probably is a hyperplastic response to an abnormal stimulus. [emedicine.medscape.com]
This observation, along with clinical disease heterogeneity has long suggested that mastocytosis is a disease of complex etiology. [thedoctorsdoctor.com]
Epidemiology
Articles in this issue include Human Mast Cell Signal Transduction; Mast cell tryptase role in homeostasis and coagulation; Mastocytosis: Current Classification and Diagnostic criteria; Epidemiology, risk factors and prognosis of mastocytosis; Mast cell [books.google.com]
Indolent systemic mastocytosis (ISM) Systemic mastocytosis with an associated clonal hematopoietic non mast cell lineage disease (SM-AHNMD) Aggressive systemic mastocytosis (ASM) Mast cell leukemia (MCL) Extracutaneous mastocytoma Mast cell sarcoma (MSS) Epidemiology [pathologyoutlines.com]
Table 1: Classification of mastocytosis (modified from Horny et al. 2008) Click here to view Epidemiology Epidemiological data in Indian population are exactly not known. [ijpd.in]
A mutation of the oncogene C-KIT D816V is found in many (but not all) cases. [ 9 ] Epidemiology [ 10 ] This is a rare and heterogeneous group of disorders, of which urticaria pigmentosa is the most common manifestation. [patient.info]
[…] mastocytosis and HIV seroconversion, proposing similarities of immunoglobulin E receptors on mast cells and basophils and the association of basophils in HIV pathophysiology. [14] Autism spectrum disorders may be increased in children with mastocytosis. [15] Epidemiology [emedicine.medscape.com]
Pathophysiology
It brings together the pathophysiology of disease with detailed clinical guidance on diagnosis and management for the full range of childhood cancers, including aspects important in optimal supportive care. [books.google.com]
Pathophysiology Mastocytosis is now classified with the myeloproliferative neoplasms. [5] Increased local concentrations of soluble mast cell growth factor in lesions of cutaneous mastocytosis are believed to stimulate mast cell proliferation, melanocyte [emedicine.medscape.com]
[…] systemic allergic reactions Monitoring therapeutic progress in conditions that are associated with secondary, localized, low-grade persistent, mast-cell proliferation and activation such as interstitial cystitis Clinical Information Discusses physiology, pathophysiology [mayomedicallaboratories.com]
Pathophysiology Gain-of-function somatic mutations in the KIT tyrosine kinase domain in mast cells are considered necessary, but not sufficient alterations to explain all of the clinical manifestations of SM. [cancertherapyadvisor.com]
In addition to the age of onset, adult and pediatric forms of mastocytosis differ in their clinical course and pathophysiology. [sdominko.tripod.com]
Prevention
However if these lesions are causing irritation, covering a mastocytoma with a special type of bandage (hydrocolloid dressing) will prevent rubbing of the lesion, stopping the release of the mast cell’s proteins and chemicals, ultimately preventing it [aocd.org]
In most cases, only yearly checkups are necessary, and no treatment is required; preventive recommendations are warranted in individuals with systemic disease and constitutional symptoms. 6 There is no cure for more severe forms of mastocytosis, but a [consultant360.com]
Treatment was continued with only a once a day application of topical tacrolimus for a month after clinical resolution to prevent further recurrence ( Figure 4 ). [f1000research.com]
Mast cell degranulation must be avoided in extensive cases to prevent possible anaphylaxis. Chemicals that induce mast cell degranulation include alcohol, aspirin, opiates, scopolamine, amphotericin B and tubocurarine, among others. [healio.com]