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Die Mastzelltryptase ist eine Serinprotease, welche fast ausschließlich in der ubiquitär vorhandenen Mastzelle vorkommt, sodass man sagen kann, ihre Höhe korreliert mit der der Mastzellen. Material und Methode: 4185 erhobene Patientendaten in den Jahren 2005 und 2006 auf Höhe ihrer Serum-Trytasewerte und ICD-10 verschlüsselten Diagnosen sowie das Alter. Ergebnisse: Es zeigten sich Erkrankungen mit durchschnittlich erhöhten Serum-Tryptasewerten sowie ein altersabhängiger Anstieg der Serum-Tryptasewerte. Diskussion: Bei der Frage, ob die Abnahme der Serum-Tryptasewerte routinemäßig durchgeführt werden soll, um so frühzeitig Risikopatienten für anaphylaktische Reaktionen identifizieren zu können, konnten unsere Daten die Forderung einiger Arbeitsgruppen unterstützen, dies zu tun. Zusammenfassung: die Serum-Tryptase stellt ein allgemein deutlich unterschätzter wichtiger Prognosefaktor für mögliche anaphylaktische Reaktionen auf verschiedenste Allergene dar und zeigt gerade im Bereich der onkologischen Erkrankungen häufig überdurchschnittlich erhöhte Werte.

The description of a monoclonal mast cell activation syndrome in patients with anaphylaxis, who fulfill one or two minor-criteria of mastocytosis, has led to a search for new unrecognized mast cell activation syndromes.New classification of mast cell diseases including well-known diseases is provided in order to be able to better recognize and describe new entities.The term mast cell activation has been defined by verifiable scientific objective and subjective criteria, and known and idiopathic mast cell activation syndromes have been classified.Mast cell activation cannot be defined by symptoms alone, as different diseases and conditions, including those with contribution of different cell types and somatization disorders may lead to similar symptoms. For this reason the preclinical checkpoint mast cell activation was defined to require typical symptoms in combination with demonstration of mast cell mediator release in (an acute) episode(s) as well as with a good response to mast cell mediator-directed therapy. Mast cell activation syndromes were classified in primary (e.g. mastocytosis), secondary (e.g. IgE-mediated allergy) and idiopathic forms.Only through a deeper understanding of mast cell diseases, can new previously unrecognized idiopathic mast cell activation syndrome entities be described and analyzed.

Anaphylaxis is a potentially life-threatening reaction and should be treated immediately. Diagnosis is made based on history and clinical features. However, measurement of sequential serum tryptase can provide additional clues and should be performed soon as possible. Referral to specialist allergy service is believed to reduce rate of recurrence. New specific in vitro techniques to indentify the triggering agent help optimize secondary preventive measures. Thus in vitro diagnosis plays an important, albeit complementary, role both in the acute and secondary phases facilitating a safe diagnosis and aiding in the overall management of the disease.

The quality and impact of care in patients with mastocytosis has not yet been investigated in detail.Here, we report monocentric findings of a retrospective questionnaire from 84 adult inpatients with mastocytosis regarding the quality and impact of pre-inpatient clinical care after a period of inpatient stay. It was possible to analyse the data derived from 50 patients (40 female, 10 male; 74 % with systemic mastocytosis, 26 % with cutaneous mastocytosis).Correct diagnoses were discovered to have taken over 2 years for 68 % of patients (up to 11 years). 58 % of patients required the consultation of three or more clinicians until the final diagnosis was reached; 3 patients consulted more than 9 doctors. The correct diagnosis was finally made in 94 % of patients by a dermatologist. The main symptoms expressed by the patients were skin disorders (92 %), itching (66 %) and exhaustion (48 %). From a patient perspective, the main triggering factors given were food and food additives (63 %), emotional stress (46 %) and physical exertion (44 %). Interdisciplinary inpatient treatment provided the patients with a better understanding of their disease in 90 %. Outpatient supervision was performed mainly by dermatologists (46 %), family doctors (40 %) and other clinicians (14 %). 22 % required the assistance of various doctors for their outpatient care. Only 14 % of patients reported that their daily life was relatively unaffected by mastocytosis. 22 % felt the disease slightly affected their quality of life, 20 % moderately, 26 % strongly and 18 % said the disease had a very strong impact on their quality of life. 82 % of patients browsed the Internet in order to find information regarding mastocytosis, although for 26 % this information was not deemed useful and 10 % felt rather unsettled.Patients with severe mastocytosis requiring hospitalization reported clear restrictions in their daily life. Such patients require an improved interdisciplinary diagnostic approach and therapy.

Specific immunotherapy (SIT) of hymenoptera venom allergy, in particular, has become an example for the effectiveness of the treatment of IgE-mediated allergic diseases. In vitro diagnostic procedures and the measurement of serum tryptase for risk assessment are well-established in the process of diagnose finding and therapy preparation. For monitoring and validation of the effectiveness of the SIT, however, in vitro diagnostic procedures remain controversial. Potentially useful approaches include detection of specific IgE, specific IgG4, basophile activation - represented by the CD 63 expression - and the lymphocyte proliferation and its IL10 release. Preliminary data suggest that the latter method appear appropriate, whereas the detection of basophile activation did not produce definite results.

Wheat-dependent exercise-induced anaphylaxis (WDEIA) is a rare IgE-dependent anaphylaxis to wheat, which occurs in sensitized people after ingestion of wheat followed by physical exercise. The major allergen associated with WDEIA is the wheat protein Omega (omega)-5-Gliadin (Tri a 19). We present three cases of WDEIA, demonstrating that this disease might be more frequent than anticipated and that neither prick test nor specific IgE to allergen extracts but rather detection of specific IgE against the recombinant protein Tri a 19 leads to diagnosis.

Anaphylaxis is an acute systemic hypersensitivity reaction with symptoms of immediate-type allergy, which involves particularly the skin, respiratory tract, cardiovascular system and gastrointestinal tract. The severity of anaphylactic reactions is variable but some are fatal. Hymenoptera venom anaphylaxis affects about 3% and food hypersensitivity 2.6-3.2% of the general population; drugs are the other frequent cause. Symptoms of anaphylaxis are characteristic, but none of them is obligatory--even urticaria is absent in about 10%--and each symptom may be found also in other conditions. Hence, there are numerous differential diagnostic considerations, and anaphylaxis may be overlooked in many cases. Diagnosis of anaphylaxis is based on the occurrence of characteristic symptoms, especially when they develop upon exposure to a potential trigger. It can be significantly supported by evidence of release of mediators in the course of the reaction. For clinical purposes, demonstration of an increase of mast cell tryptase serum concentration above the individual baseline value is useful. The correct diagnosis of anaphylaxis is not only important with regard to treatment of an acute reaction, but also for subsequent allergologic diagnostics and long-term management of the patient.

Mucosa-immunologic aspects are gaining an increasing awareness in the pathophysiology of type I allergies. Humoral mucosal immune responses are dominated by secretory IgA, but there is evidence for a relevant role of IgG in nasal mucosa-associated lymphoid tissue.was to measure allergen-specific immunoglobulins (IgA and IgG) in nasal secretions as an expression of a humoral mucosal immune response in allergic rhinitis. For tissue eosinophilia we studied nasal Eosinophilic Cationic Protein (ECP) and for mast cell activation nasal tryptase.Nasal secretions of 40 patients suffering from allergic rhinitis were analyzed for allergen-specific IgA, IgG, and IgE, and for ECP and tryptase. Patients were highly sensitized against the major allergens of house dust mites, timothy, and birch pollen. 43 non-atopic individuals served as controls. In order to study possible effects of the actual pollen season on the studied parameter we secondly compared patients allergic to seasonal allergens co- (n = 28) and extra-seasonally (n = 41). In order to determine a possible influence of allergen-specific IgA in eosinophilic degranulation we additionally studied 5 patients after nasal allergen challenge.In allergic rhinitis we found significantly increased levels of allergen-specific immunoglobulins of all studied subclasses and allergens in nasal secretions. Comparison of nasal ECP and tryptase showed significantly increased concentrations in allergic individuals as well. Co-seasonally we found elevated allergen-specific IgE, ECP, and tryptase but lower concentrations of allergen-specific IgA and IgG. There was no association between late phase eosinophilia and IgA concentrations after local allergen challenge.The occurrence of allergen-specific immunoglobulins in nasal secretions is interpreted as a local humoral mucosal immune response. The physiologic role of local allergen-specific immunoglobulins is not clear to date. Involvement in degranulation of eosinophils or mast cells, like suggested before, seems unlikely.

With the occurrence of unclear consciousness conditions primarily internal or neurological causes are considered. Of a systemic mastocytosis one thinks only rarely, which can accompany without or with slight clinically visible skin changes. In the following we report on a patient who has repeated unclear shock conditions and required resuscitation several times without a recognizable cause, with whom a systemic mastocytosis could be proven. Clinically very discrete lesions of mastocytosis were recognizable from the skin. Only an increased tryptase level referred to being present an occult systemic mastocytosis. The diagnostics, potential triggers and therapy of this disease are to be discussed on the basis the available case.

Cisatracurium (51W89, Nimbex, Glaxo-Wellcome), an intermediate-acting non-depolarizing neuromuscular blocking agent, is a stereoisomer of atracurium. Histamine releasing propensities and serum tryptase level have been investigated after administration of cisatracurium (3 x ED95, 5 x ED95) or vecuronium (3 x ED90) in surgical patients.After approval by our institutional review board, 62 patients (ASA I-II) were randomly assigned to three groups to receive either 3 x ED95 or 5 x ED95 cisatracurium, or 3 x ED90 vecuronium as a rapid bolus. A prick test was done the day before by scarification of the skin in the forearm. After premedication with 2 mg lormetazepam, anaesthesia was induced with thiopentone (4-12 mg/kg) and maintained with O2/N2O and isoflurane (1.5-2 vol.%). Six minutes after thiopentone, the patients received the relaxant and after further 6 min 0.1-0.2 mg fentanyl was given and the trachea was intubated. Heart rate (HR) and blood pressure (BP) were monitored every minute. Blood samples for histamine were withdrawn 5 min prior 3 and 5 min after each drug administration (thiopentone, relaxants). Plasma histamine was measured by radioimmunoassay (RIA) with a sensitivity of approximately 10 pg/ml. Additionally, serum tryptase was measured by RIA at baseline (-10 and -1 min) and 15 and 60 min after the relaxant administration. Levels for histamine1000 pg/ml and for tryptase2 micrograms/ml were considered significant. Cutaneous signs of histamine release were documented.Ten patients showed a positive prick-test reaction. Only after thiopentone some cutaneous signs (4 flush, 1 erythema) of histamine release were observed. There were no cutaneous signs of histamine release correlating with cardiovascular changes. Analysis of the blood samples demonstrated no significant increase in the histamine level in all three groups. Only in 1 patient was a significant higher histamine level (1133 pg/ml) measured 5 min after 5 x ED95 cisatracurium. All measurements of serum tryptase were within the physiological limits.In this study, with the particular time course of drug administration, neither cisatracurium nor vecuronium increased plasma histamine levels. Only after 5 x ED95 cisatracurium was 1 elevated histamine level documented after 5 min. In several studies increased histamine levels have been described, but without clinical manifestations. It is known that cutaneous signs can occur without increased plasma histamine levels due to the structural heterogeneity of mast cells. The cutaneous reactions in this study were caused by thiopentone. The tryptase values were within normal limits even in the patient with histamine release. No relationship between the positive results in the prick test and the incidence of cutaneous reactions and/or histamine release for drugs used in the induction of anaesthesia was observed. Whether cisatracurium has a potential for immunologic release is unknown.

In this article we present the symptomatic features and discuss relevant diagnostic and therapeutic aspects of anaphylactoid reactions. In addition we give practical advice as to how to avoid and manage allergic or pseudoallergic reactions during anaesthesia. Measurements of serum tryptase-levels and of methylhistamine in urine have been introduced in clinical diagnostic routine. Skin tests and determination of specific antibodies are essential to identify responsible substances. Preventive measures like careful premedication, calm atmosphere, slow injection of drugs, the use of diluted solutions, and the use of drugs with a low potential for anaphylactoid reactions are important. Substances like inhalation anaesthetics, propofol, etomidate, ketamine, midazolam, fentanyl, alfentanil and bupivacain without epinephrine should be used.