ABSTRACT
ABSTRACT Introduction: Systemic Mastocytosis comprises a group of neoplastic diseases characterized by clonal expansion and infiltration of mast cells into several organs. The diagnosis and treatment of this disease may be challenging for non-specialists. Objective: Make suggestions or recommendations in Systemic Mastocytosis based in a panel of Brazilian specialists. Method and results: An online expert panel with 18 multidisciplinary specialists was convened to propose recommendations on the diagnosis and treatment of Systemic Mastocytosis in Brazil. Recommendations were based on discussions of topics and multiple-choice questions and were graded using the Oxford Centre for Evidence-Based Medicine 2011 Levels of Evidence Chart. Conclusion: Twenty-two recommendations or suggestions were proposed based on a literature review and graded according to the findings.
Subject(s)
Mastocytosis, Systemic/diagnosis , Mastocytosis, Systemic/therapy , Child , AdultABSTRACT
Se exponen datos relacionados con la histología, fisiología y patología de los mastocitos, tanto normales como patológicos, y el papel de la alergia medicamentosa en la mastocitosis, así como las repercusiones psicopatológicas de la enfermedad y las bases neuroquímicas de dichos trastornos.
Data concerning the biological aspects of mastocytes, its pathology and the importance of childhood and adult mastocytosis are described. The role of drug allergy in mastocytosis is discussed. The psychopathological and neurochemical aspects of these conditions are exposed.
Subject(s)
Humans , Drug Hypersensitivity , Mastocytosis, Systemic/diagnosis , Mastocytosis, Systemic/psychology , Mastocytosis, Systemic/therapy , Desensitization, Immunologic , Mastocytosis, Systemic/physiopathology , Mutation/genetics , PsychotherapyABSTRACT
Las mastocitosis incluyen un amplio espectro de patologías que tienen en común la infiltración anormal de mastocitos en diversos órganos, siendo la piel el más frecuentemente comprometido. Se reconocen dos variantes principales de la enfermedad: la mastocitosis cutánea (MC), que sólo compromete la piel, y la mastocítosis sistémica (M S), donde hay compromiso de órganos extracutáneos. La sintomatología de ambas variantes es causada por la infiltración celular y daño directo sobre los tejidos, así como por la liberación de mediadores químicos a la circulación sistémica, lo que hace que su presentación clínica sea altamente variable. Las mastocitosis en niños se presentan generalmente como MC, son de buen pronóstico y evolucionan con remisión de las lesiones en la mayoría de los casos. Las MS son muy poco frecuentes en este grupo etario; sin embargo, su curso crónico y la agresividad que pueden adquirir ponen en relieve la importancia de considerarlas en el diagnóstico diferencial de estos cuadros. En la actualidad no existe tratamiento curativo para las mastocitosis y el manejo es fundamentalmente sintomático.
Mastocytosis is a heterogeneous group of diseases characterized by the abnormal infiltration of mast cells (MCs) in one or more organ systems, being the skin the most common organ affected. Two main variants of the disease are recognized: cutaneous mastocytosis (CM), if abnormal infiltrates are confined to the skin, and systemic mastocytosis (SM) , if extra-cutaneous tissues are involved. Symptoms are extremely variable and result from MC-derived mediators and from destructive infiltration of MCs. The typical presentation of pediatric-onset mastocytosis consists of cutaneous manifestations, and usually regresses spontaneously. SM is a chronic disease with variable clinical course ranging from asymptomatic to highly aggressive and rapidly devastating. SM is rare in children but should be considered in the differential diagnosis. No curative treatment has been yet reported for mastocytosis and only symptomatic therapy is available.