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Mastocytosis is a term used to describe a heterogeneous group of disorders characterized by clonal proliferation of mast cells in various organs. The organ most often affected is the skin. Mastocytosis is a relatively rare disorder that affects both sexes equally. It can occur at any age, although it tends to appear in the first decade of life, or later, between the second and fifth decades. Our understanding of the pathophysiology of mastocytosis has improved greatly in recent years, with the discovery that somatic c-kit mutations and aberrant immunophenotypic features have an important role. The clinical manifestations of mastocytosis are diverse, and skin lesions are the key to diagnosis in most patients., (Copyright © 2015 Elsevier España, S.L.U. and AEDV. All rights reserved.)

Mastocytosis is a term used to describe a heterogeneous group of disorders characterized by clonal proliferation of mast cells in different organs. The organ most often affected is the skin. The World Health Organization classifies cutaneous mastocytosis into mastocytoma, maculopapular cutaneous mastocytosis, and diffuse mastocytosis. The systemic variants in this classification are as follows: indolent systemic mastocytosis (SM), aggressive SM, SM with an associated clonal hematological non-mast cell lineage disease, mast cell leukemia, mast cell sarcoma, and extracutaneous mastocytoma. The two latest systemic variants are rare. Although the course of disease is unpredictable in children, lesions generally resolve by early adulthood. In adults, however, the disease tends to persist. The goal of treatment should be to control clinical manifestations caused by the release of mast cell mediators and, in more aggressive forms of the disease, to reduce mast cell burden., (Copyright © 2015 Elsevier España, S.L.U. and AEDV. All rights reserved.)

Objectives: To determine the incidence, immunological mechanisms, severity and clinical course of perioperative allergic reactions., Patients and Methods: Prospective epidemiological study lasting 2 years (1996-97). In 20 hospitals in Catalonia (Spain), we studied patients who suffered allergic reactions equal to or greater than grade Ib according to the classification of Laxenaire. Serum and urine samples were collected during the first and sixth hours after the onset of a reaction. Complement factors, total serum and latex-specific serum IgE antibodies, hemostatic markers, serum tryptase and urinary methylhistamine were assayed. Tests for allergy to the drugs used during the perioperative period were performed on all patients who consented., Results: Anesthetic procedures were performed 328,430 times in the 20 hospitals. Thirty-two allergic reactions were reported (1 case/10,263 anesthesias); the frequency was greater during general anesthesia (1 case/6,978 anesthesias). Women suffered 58.3% of the reactions, and the mean patient age was 47.8 +/- 16.5 years. Fifty-six percent of the reactions were severe (grades III-IV), and 68.7% occurred upon immediate exposure. Induction was the moment of greatest risk (50%). Treatment was required by 90.6% of the patients, but no deaths or serious sequelae occurred. Complete analyses could be carried out immediately for 25 patients. High urinary methylhistamine and/or serum tryptase levels were found for 57% of the patients with grade Ib reactions, for 80% of those with grade II reactions, and for 91.7% of those with grade III reactions (p = 0.05). High serum tryptase levels were the only findings for 53.8% of the grade III reactions (p = 0.007). Skin tests were positive for 62.5% of the patients., Conclusions: The frequency of allergic reactions in Catalonia is 1 case per 10,263 anesthesias performed, but the rate is higher in general anesthesia. Fifty-six percent of the reactions were severe. Most developed immediately and the moment of induction involved the greatest risk. Early assessment of methylhistamine and tryptase levels has been shown to be useful and positivity is linearly associated with severity of reaction. In 62.5% of the patients, positive results were seen in skin tests performed later.

To assess the role of some pro-inflammatory cells in inflammatory processes in lung cancer by measuring their respective activation markers in different portions of bronchoalveolar lavage (BAL) fluid. Prospective study in a university hospital. We studied 52 BAL samples, 37 from patients with lung cancer and 15 from a control group, using a radioimmunoassay technique to analyze for tryptase (T), hyaluronic acid (HA) and eosinophil cationic protein (ECP) in separate bronchial and bronchoalveolar samples from BAL fluid. Statistical analysis was performed using the R-SIGMA program. Patients with tumors had significantly higher T and HA levels in BAL fluid than did control patients, in both bronchial and bronchoalveolar portions. Lung cancer patients had higher T and ECP levels in bronchoalveolar portions. Mast cells and fibroblasts, at least, play a part in lung cancer, mainly in the distal portions of the bronchial tree.

Mast cells play a central role in allergic diseases, following activation by means of immunologic mechanisms. Mast cell degranulation releases preformed and newly generated mediators and potential indicators of mast cell activation. Tryptase is the most useful marker of selective mast cell activation with regard to specific pathological states in which it can be applied with positive results, especially allergic diseases.

In the last years, the role of nasal mast cells and the eosinophils in the immediate hypersensitivity reaction of allergic rhinitis has been well documented. Tryptase and the eosinophil cationic protein (ECP) are specific activation markers for mast cells and esosinophils respectively. To determine the possible diagnostic value of these markers in allergic rhinitis we measured the levels in both serum and native nasal fluid using sandwich RIA-assays. Twenty eight seasonal allergic patients (16 with active allergy and 12 with extraseasonal allergy) and 11 patients with chronic sinusitis were studied. Twenty one normal healthy donors served as controls. We could not detect increased levels of tryptase and ECP in the serum of all patients and healthy donors. In contrast, patients with active allergy showed very higher levels of ECP than patients of the three other groups. Similarly, nasal ECP was higher in patients with nasal pathology than in healthy subjects. The highest levels were found in the patients with active allergic rhinitis. Our results show that both native nasal fluid tryptase and ECP can be used as markers of local inflammation and that the assessment of their levels can be useful in the diagnosis and follow up of mucosal inflammation in the nose.