Standardized indolent systemic mastocytosis evaluations across a healthcare system: implications for screening accuracy.

Timely diagnosis of systemic mastocytosis (SM) remains challenging due to care heterogeneity. We implemented a standardized approach for SM screening and diagnosis utilizing a novel healthcare system-wide international screening registry. A retrospective analysis assessed rates of SM, cutaneous mastocytosis (CM), and molecular diagnoses before and two years after care standardization. Accuracy of individual and combined SM screening tests - basal serum tryptase (BST) ≥11.5 and ≥20.0 ng/mL, REMA ≥2, monomorphic maculopapular CM, and elevated BST based upon tryptase genotype - was analyzed. Tryptase genotyping and high-sensitivity KIT p.D816V testing increased substantially two years following care standardization. SM diagnoses doubled from 47 to 94 and KIT p.D816V molecular diagnoses increased from 24 to 79. Mean BST and KIT p.D816V variant allele frequency (VAF) values were significantly lower in patients diagnosed after standardization. Hereditary-alpha tryptasemia prevalence was increased in SM prior to care standardization at 4/30 (13.3%) but reflected the general population prevalence two years later at 5/76 (6.6%). Elevated BST based upon genotype and BST ≥11.5 ng/mL had the highest sensitivities at 84.2% and 88.3%, respectively. Presence of monomorphic MPCM, elevated BST based upon tryptase genotype, and the combination of REMA ≥2 with elevated BST based upon tryptase genotype had specificities >90%. BST >20.0 ng/mL had low sensitivity and specificity and was not required to establish any indolent SM diagnosis. Care standardization increased SM diagnosis rates, particularly in patients with low BSTs. Stratifying BST based upon genotype had the best overall sensitivity and specificity of any indolent SM screening test and improved the REMA score specificity.

Military accession guidelines: An allergy focus.

Medical evaluation for military applicants is an intricate process that requires an understanding of the terminology, standards, and guidelines. Allergy providers are often called to provide medical evaluations for patients who desire to join the military services. Without understanding the complexities and nuances of military medical evaluations, a provider may delay or not be able to assist their patient in obtaining the desired goal of joining the services. This article reviews the terminology of military medical evaluations and the guidelines and processes for these evaluations. We also focus our discussion on common allergic conditions that may be disqualifying for service and provide expert opinions of the subtleties of these conditions to provide the allergist with a practical approach to medical evaluations. Finally, we provide a list of resources that are accessible to any provider engaged in military medical evaluations for accessions.

A masquerade of recurrent anaphylaxis.

Background: We present a case of a 37 year old man with a history of human immunodeficiency virus, latent syphilis, anxiety, posttraumatic stress disorder. attention deficit/hyperactivity disorder, multiple drug intolerance syndrome who presented with concerns of recurrent episodes of rash and respiratory symptoms with questionable "anaphylaxis" episodes without clear etiology or known trigger. Methods: To evaluate some of the potential causes of recurrent anaphylaxis in our patient. Further evaluation through laboratory analysis and ultimately direct visualization of the patient's vocal cords by laryngoscopy assisted in the final diagnosis. Results: Inappropriate adduction of the vocal cords was observed during an acute reaction. Conclusion: The patient's presentation was consistent with inducible laryngeal obstruction and highlights the importance of confirming a suspected diagnosis of anaphylaxis and keeping a broad differential when establishing an etiology.

Updates and Recent Advances on Venom Immunotherapy.

Purpose of Review: Venom immunotherapy has been utilized to treat Hymenoptera venom allergy since the 1920s. Over the last century, significant advances in the fields of immunology and genetics have led to improvements in the practice of venom immunotherapy. This review encompasses recent advances in the use of venom immunotherapy to provide precise, patient-centered care. Recent Findings: Research about the mechanism of action of venom immunotherapy continues to highlight the modification of both the innate and adaptive immune systems. Molecular techniques have allowed for the identification of specific venom allergens to improve the diagnostic accuracy and safety of venom immunotherapy. Research continues to support the safety of accelerated schedules which can impact the cost, adherence, and quality of life for patients receiving this treatment modality. Finally, significant advances have led to the elucidation of risk factors that place patients at risk for reactions during and after venom immunotherapy. Creation of risk profiles for venom-allergic patients can thus inform the process of immunotherapy in order to provide personalized and precise care. Summary: Significant progress in the use of venom immunotherapy makes the practice a dynamic and active field for continued research. Future research needs to build on these recent advances to continue to optimize and enhance this life-saving treatment.

Long-term adherence to imported fire ant subcutaneous immunotherapy.

BACKGROUND: Imported fire ant (IFA) whole-body extract subcutaneous immunotherapy (IT) is a safe and effective treatment for IFA hypersensitivity, with a recommended length of treatment of 3 to 5 years. OBJECTIVE: To evaluate long-term IFA IT adherence in patients with IFA allergy living in an endemic area. METHODS: During 2007 to 2014, patients with IFA-sting systemic reactions and a recommendation to start IFA IT were prospectively enrolled in this study. Subjects were contacted annually for interval IT adherence. Institutional review board approval was obtained. RESULTS: A total of 87 subjects, ages 2 to 64 years, with a recommendation to initiate IFA IT, were enrolled. Subjects were predominantly adult (76%) and female (55%), and 30% had asthma. Of these patients, 77 (89%) initiated treatment within 1 year of recommendation; 18 (23%) adhered to a 3-year course; and 10 (13%) adhered to a 5-year course. At 3 years, there were no significant differences in adherence between male and female subjects (28% vs 19%, P = .33), children and adults (25% vs 22%, P = .79), or those with or without asthma (30% vs 20%, P = .31). Adherence in subjects with mild initial reactions was lower than in subjects with moderate-to-severe reactions (0% vs 25%, P = .05) at 3 years only. Conventional buildup and concurrent flying Hymenoptera venom immunotherapy were predictive of adherence. Reasons for discontinuation were relocation to a nonendemic area (29%) and inconvenience (27%). CONCLUSION: This study showed poor long-term adherence to IFA IT at 3 and 5 years. Initial sting severity, buildup protocol, and concurrent flying Hymenoptera venom immunotherapy were predictors for long-term IT adherence.

COVID-19 Vaccine Distribution: Bolstering the Air Force Immunizations Model.

From its emergence and declaration as a worldwide pandemic, coronavirus disease-2019 (COVID-19) has been associated with significant medical and logistical challenges. Initial obstacles ranged from the need to develop testing platforms to the determination of effective treatments to decrease the significant morbidity and mortality associated with the disease. Due to significant scientific breakthroughs, the most recent pressing challenge has been the distribution of vaccines against severe acute respiratory syndrome coronavirus 2 to prevent the spread of the infection across the world. Historically, the U.S. Military has been involved in immunizations delivery to prevent disease among its troops. This experience has led to the establishment of programs designed to distribute safe vaccinations to service members and beneficiaries. It is upon this infrastructure that the Air Force (AF) medical community was thrust to devise and execute COVID-19 vaccine delivery implementation across all military treatment facilities. In the AF, specialty trained allergist and immunologists provide the technical expertise for the establishment and execution of the AF immunizations program. These medical professionals have the proficiency, knowledge, and experience to run efficient mass immunization events in order to provide vaccines in a safe and rapid environment. Despite this, significant challenges attributable to the variable and novel nature of COVID-19 vaccine logistical requirements have led to unanswered questions and debate that needed resolution before and during the launch of this program. Here we describe the process for establishing a mass vaccination program for COVID-19 vaccine delivery and our experience-based problem solving approach at a large AF military treatment facility.

Anaphylaxis to Stinging Insect Venom.

Hymenoptera stinging insects are common culprits for allergic reactions. Anaphylaxis to insect stings can be life threatening and is associated with a significant risk of recurrence. Insect allergy requires referral to an allergist/immunologist for education and for diagnostic evaluation that will direct further management and treatment. Venom immunotherapy is safe and effective; it prevents sting anaphylaxis in up to 98% of patients. Potential risk factors for side effects during testing and treatment should be assessed for every patient to mitigate risk and to guide treatment recommendations and the duration of immunotherapy.

Characteristics of venom allergy at initial evaluation: Is fire ant hypersensitivity similar to flying Hymenoptera?

BACKGROUND: Hymenoptera venom allergy (HVA) is a well-established cause of anaphylaxis; however, studies comparing patients with imported fire ant (IFA) to flying hymenoptera (FH) allergies are lacking. OBJECTIVE: This study sought to characterize the initial presentation and examine differences between patients with IFA and FH reactions. METHODS: A multiyear (2007-2014), observational, single-institution analysis of patients referred for evaluation of HVA was performed. Data was obtained via physician interview, chart review and specific IgE results. RESULTS: 175 patients were enrolled with no difference between FH and IFA patients when analyzing mean age, sex or likelihood to seek emergency department (ED) care. Asthma was similar in all groups at 21%. ED treatments were also similar (epinephrine: 32/150, 21%; antihistamines: 141/155, 91%; corticosteroids: 67/148, 45%). Reaction severity correlated with likelihood of ED visit (P <.001), use of epinephrine (P <.001) and corticosteroid use (P <.05). Patients presenting to the ED with anaphylaxis received epinephrine in 27/73 (37%) of cases. Overall, 149/175 (85%) patients in our cohort were confirmed to be sensitized to hymenoptera. Of those with positive testing 127/149 (85%) chose to pursue VIT. CONCLUSION: IFA and FH patients have many similarities at presentation. Asthma occurred 2.5 times more frequently our cohort compared to the general US population. Epinephrine administration in our cohort was suboptimal. Children have initial reactions and are treated in the ED similarly to adults, with a notable (although not statistically significant) reduced use of epinephrine. The majority of patients seen in the ED and subsequently followed up in an allergy clinic had their venom allergy confirmed and initiated life-saving immunotherapy.

Safety of Repeated Imported Fire Ant Ultra-Rush Protocols.

Treatment of imported fire ant (IFA) hypersensitivity includes avoidance and venom immunotherapy (VIT) that reduces the risk of reactions to less than 5%. Previous studies have shown that rush immunotherapy (RIT) is safe and efficacious. It is unknown if multiple RIT procedures on individuals with breaks in VIT are safe and effective. This is a retrospective chart review of three patients who have undergone six IFA VIT rush protocols at our institution. Patients were all female with a median age of 27 years. All are active duty service members without medical problems who reside in an IFA endemic area. Reviewed protocols consisted of 10 injections of IFA whole body extract given in 1 day. Local reactions were the only side effect noted. All patients completed the protocols without further incident. Following each protocol, median VIT adherence was 9 months (range 2-72 months). Reasons for stopping VIT were deployment and pregnancy. Non-adherence is common and leads to breaks in VIT. The risk of VIT resumption in patients with previous RIT or VIT is unknown. This case series illustrates the safety and efficacy of repeated IFA VIT rush procedures in patients who for personal and service related reasons had breaks in VIT.

SCUBA Diving and Asthma: Clinical Recommendations and Safety.

The objective of this article is to review the available studies regarding asthma and SCUBA (self-contained underwater breathing apparatus) diving. A literature search was conducted in MEDLINE to identify peer-reviewed articles related to asthma and SCUBA diving using the following keywords: asthma, allergy, and SCUBA diving. SCUBA diving is a popular sport with more than 9 million divers in the USA. SCUBA diving can be a dangerous sport. Bronchospasm can develop in asthmatic patients and cause airway obstruction. Airway obstruction may be localized to the distal airway which prevents gas elimination. Uncontrolled expansion of the distal airway may result in pulmonary barotrauma. There is also the risk of a gas embolism. Asthmatic divers can also aspirate seawater which may induce bronchospasm. Pollen contamination of their oxygen tank may exacerbate atopic asthma in patients. Diving may be hazardous to the lung function of patients with asthma. Despite the risks of SCUBA diving, many asthmatic individuals can dive without serious diving events. Diving evaluations for asthmatic patients have focused on a thorough patient history, spirometry, allergy testing, and bronchial challenges. For patients that wish to dive, their asthma should be well controlled without current chest symptoms. Patients should have a normal spirometry. Some diving societies recommend that an asthmatic patient should successfully pass a bronchial provocation challenge. Recommendations also state that exercise-, emotion-, and cold-induced asthmatics should not dive. Asthmatic patients requiring rescue medication within 48 h should not dive.