This article updates current knowledge about epidemiology, prognosis, and risk factors for major complications in mastocytosis. A prevalence of mastocytosis of 1 in 10000 inhabitants has been ...reported, but underdiagnosis is assumed. The prognosis for cutaneous and indolent systemic mastocytosis is excellent. For more advanced forms of disease, prognostic parameters have been identified. A high extent of skin involvement, increased basal serum tryptase values, and extensive blistering are risk factors for severe mast cell activation episodes in children, whereas these associations seem to be less strong or nonexistent for anaphylaxis and osteoporosis in adult patients with indolent systemic mastocytosis.
Background Oral wheat plus cofactors challenge tests in patients with wheat-dependent exercise-induced anaphylaxis (WDEIA) produce unreliable results. Objective We sought to confirm WDEIA diagnosis ...by using oral gluten flour plus cofactors challenge, to determine the amount of gluten required to elicit symptoms, and to correlate these results with plasma gliadin levels, gastrointestinal permeability, and allergologic parameters. Methods Sixteen of 34 patients with a history of WDEIA and ω5-gliadin IgE underwent prospective oral challenge tests with gluten with or without cofactors until objective symptoms developed. Gluten reaction threshold levels, plasma gliadin concentrations, gastrointestinal permeability, sensitivities and specificities for skin prick tests, and specific IgE levels were ascertained in patients and 38 control subjects. Results In 16 of 16 patients (8 female and 8 male patients; age, 23-76 years), WDEIA was confirmed by challenges with gluten alone (n = 4) or gluten plus cofactors (n = 12), including 4 patients with previous negative wheat challenge results. Higher gluten doses or acetylsalicylic acid (ASA) plus alcohol instead of physical exercise were cofactors in 2 retested patients. The cofactors ASA plus alcohol and exercise increased plasma gliadin levels ( P < .03). Positive challenge results developed after a variable period of time at peak or when the plateau plasma gliadin level was attained. Positive plasma gliadin threshold levels differed by greater than 100-fold and ranged from 15 to 2111 pg/mL (median, 628 pg/mL). The clinical history, IgE gliadin level, and baseline gastrointestinal level were not predictive of the outcomes of the challenge tests. The challenge-confirmed sensitivity and specificity of gluten skin prick tests was 100% and 96%, respectively. Conclusion Oral challenge with gluten alone or along with ASA and alcohol is a sensitive and specific test for the diagnosis of WDEIA. Exercise is not an essential trigger for the onset of symptoms in patients with WDEIA.
A recent survey of the European Academy of Allergy and Clinical Immunology (EAACI) Drug Allergy Interest Group (DAIG) on how European allergy specialists deal with beta‐lactam (BL) hypersensitivity ...demonstrated a significant heterogeneity in current practice, suggesting the need to review and update existing EAACI guidelines in order to make the diagnostic procedures as safe and accurate, but also as cost‐effective, as possible. For this purpose, a bibliographic search on large studies regarding BL hypersensitivity diagnosis was performed by an EAACI task force, which reviewed and evaluated the literature data using the GRADE system for quality of evidence and strength of recommendation. The updated guidelines provide a risk stratification in BL hypersensitivity according to index reaction(s), as well as an algorithmic approach, based on cross‐reactivity studies, in patients with a suspicion of BL hypersensitivity and an immediate need for antibiotic therapy, when referral to an allergist is not feasible. Furthermore, the update addresses availability and concentrations of skin test (ST) reagents, ST and drug provocation test (DPT) protocols, and diagnostic algorithms and administration of alternative BL in allergic subjects. Specifically, distinct diagnostic algorithms are suggested depending on risk stratification of the patient into high and low risk based on the morphology and chronology of the reaction, immediate (ie, occurring within 1‐6 hours after the last administered dose) or nonimmediate (ie, occurring more than 1 hour after the initial drug administration), and the reaction severity. Regarding the allergy workup, the main novelty of this document is the fact that in some low‐risk nonimmediate reactions ST are not mandatory, especially in children. For DPT, further studies are necessary to provide data supporting the standardization of protocols, especially of those regarding nonimmediate reactions, for which there is currently no consensus.
Mastocytosis and Anaphylaxis Schuch, Anna; Brockow, Knut
Immunology and allergy clinics of North America,
02/2017, Letnik:
37, Številka:
1
Journal Article
Recenzirano
This article updates current knowledge on epidemiology, risk factors, triggers, and management of anaphylaxis in patients with mastocytosis. Hyperactive mast cells and higher number of effector mast ...cells are speculated to facilitate anaphylaxis in this condition. In children, increased risk is limited to those with extensive skin involvement and high tryptase. In adults, manifestations of anaphylaxis are severe with high frequency of cardiovascular symptoms. Hymenoptera stings are the most common triggers for these reactions; however, idiopathic anaphylaxis and reactions to food or drugs occur. Patients with mastocytosis should be informed about risk of anaphylaxis and prescribing emergency self-medication and installing emergency preparedness before general anesthesia is considered.
Anaphylaxis is a clinical emergency which all healthcare professionals need to be able to recognize and manage. The European Academy of Allergy and Clinical Immunology Anaphylaxis multidisciplinary ...Task Force has updated the 2014 guideline. The guideline was developed using the AGREE II framework and the GRADE approach. The evidence was systematically reviewed and recommendations were created by weighing up benefits and harms. The guideline was peer‐reviewed by external experts and reviewed in a public consultation. The use of clinical criteria to identify anaphylaxis is suggested with blood sampling for the later measurement of tryptase. The prompt use of intramuscular adrenaline as first‐line management is recommended with the availability of adrenaline autoinjectors to patients in the community. Pharmacokinetic data should be provided for adrenaline autoinjector devices. Structured, comprehensive training for people at risk of anaphylaxis is recommended. Simulation training and visual prompts for healthcare professionals are suggested to improve the management of anaphylaxis. It is suggested that school policies reflect anaphylaxis guidelines. The evidence for the management of anaphylaxis remains mostly at a very low level. There is an urgent need to prioritize clinical trials with the potential to improve the management of patients at risk of anaphylaxis.
Drug hypersensitivity reactions (DHRs) are common, and the skin is by far the most frequently involved organ with a broad spectrum of reaction types. The diagnosis of cutaneous DHRs (CDHR) may be ...difficult because of multiple differential diagnoses. A correct classification is important for the correct diagnosis and management. With these guidelines, we aim to give precise definitions and provide the background needed for doctors to correctly classify CDHR.
Immediate and nonimmediate hypersensitivity reactions to iodinated contrast media (ICM) have been reported to occur in a frequency of about 0.5%‐3% of patients receiving nonionic ICM. The diagnosis ...and management of these patients vary among guidelines published by various national and international scientific societies, with recommendations ranging from avoidance or premedication to drug provocation test. This position paper aims to give recommendations for the management of patients with ICM hypersensitivity reactions and analyze controversies in this area. Skin tests are recommended as the initial step for diagnosing patients with immediate and nonimmediate hypersensitivity reactions; besides, they may also help guide on tolerability of alternatives. Re‐exposition or drug provocation test should only be done with skin test‐negative ICMs. The decision for performing either re‐exposition or drug provocation test needs to be taken based on a risk‐benefit analysis. The role of in vitro tests for diagnosis and pretreatment for preventing reactions remains controversial.
Patients with mastocytosis have an increased risk for severe anaphylaxis, particularly to Hymenoptera venoms. These patients may also develop more often systemic hypersensitivity reactions to certain ...foods. However, this issue has not been systematically investigated.
To determine prevalence and severity of food hypersensitivity (FH) reactions among patients with clonal mast cell disorders (CMDs).
A retrospective study was conducted among 204 (age ≥18 years) consecutive patients who presented with confirmed CMD (170 with mastocytosis and 34 with monoclonal mast cell activation syndrome). All patients underwent thorough allergy workup where self-reported FH reactions were evaluated.
The prevalence of self-reported FH was 20.6%. The frequency of immunologically mediated reactions was uncommon, because only 3.4% were confirmed by relevant history and IgE sensitization. Among patients with FH, 5 had severe anaphylaxis corresponding to an overall prevalence of 2.5%. Most symptoms were restricted to skin (86%), followed by gastrointestinal tract symptoms (45%)-similar to symptoms that occur in patients with mastocytosis also without food intake. Nuts, spicy foods, seafood, and alcohol were the most common incriminated elicitors. There was no significant difference between the groups regarding age, sex, atopic status, or IgE levels.
Anaphylaxis from foods in mastocytosis does exist and is severe, although foods are less frequent elicitors than insect venoms. Furthermore, the frequency of overall FH reactions is comparable with that in the general population and most reactions are mild, nonallergic, and unconfirmed. Consequently, our results do not support the elimination of any diet in patients with CMD without a history of FH.
Mastocytosis is characterized by the pathological accumulation of mast cells (MC) in various organs. In these patients, MC may degranulate and thereby contribute to clinical symptoms, especially when ...a concomitant allergy is present. However, MC activation can not only be induced by high-affinity receptors for IgE, but also by anaphylatoxins, neuropeptides, IgG immune complexes, complement-components, drugs, products of bacteria or parasites, as well as physical factors such as heat, cold, vibration, stress, sun, or physical effort. Symptoms due to mediators released by activated MC may develop in adults suffering from systemic mastocytosis, but also evolve in children who usually have cutaneous mastocytosis (CM). Clinically, CM is otherwise characterized by typical brown, maculopapular skin lesions or mastocytoma associated with a positive Darier's sign. Pruritus and flushing are common and blistering may also be recorded, especially in diffuse CM (DCM). Pediatric patients with mastocytosis may also have gastrointestinal, respiratory, and neurologic complaints. Although anaphylaxis is not a typical finding, pediatric patients with massive skin involvement and high tryptase levels have a relatively high risk to develop anaphylaxis. This paper reviews MC mediator-related symptoms and anaphylaxis in children with mastocytosis, with special emphasis on risk factors, triggers, and management.
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