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.

Pneumococcal Perplexity: Improving Awareness of Updated Pneumococcal Vaccination Recommendations in Two Large Military Treatment Facilities.

INTRODUCTION: Vaccinations are an essential aspect of preventive medicine. In October 2021, the pneumococcal conjugate vaccine-20 (PCV-20) and PCV-15 were authorized for use in adults by the U.S. FDA. In 2022, the Advisory Committee on Immunization Practices (ACIP) subsequently published updated pneumococcal vaccination recommendations that incorporate both PCV-20 and PCV-15. Pneumococcal vaccination is effective in reducing pneumococcal disease, particularly in high-risk patient groups such as those with chronic lung disease; however, the updated dosing schedule for pneumococcal vaccinations can be quite confusing, especially if patients have previously received "older" vaccinations, such as pneumococcal polysaccharide vaccine-23 or PCV-13. The purpose of this quality improvement project was to increase providers' knowledge of current ACIP pneumococcal vaccination recommendations, including indications and dosing schedule, and to improve pneumococcal vaccination rates among eligible adults and children. MATERIALS AND METHODS: Focused education sessions were presented to primary care and subspecialty residents, fellows, and staff at Brooke Army Medical Center and Wilford Hall Ambulatory Surgical Center regarding current ACIP pneumococcal vaccination recommendations. Sessions included information about PCV-15 and PCV-20 vaccines, indications for vaccination, and dosing schedules. Subjective knowledge of updated ACIP pneumococcal vaccination recommendations was assessed among primary care and subspecialty residents, fellows, and staff via an anonymous survey both pre- and post-intervention. Number of PCV-20 vaccinations given and estimated vaccination rates of patients aged 19 to 64 years with asthma were assessed pre- and post-intervention over a 6 month time span. RESULTS: Of surveyed providers, only 9% discussed vaccinations at every visit and 11% did not discuss vaccinations at all. There was a statistically significant increase in providers' knowledge of pneumococcal vaccination guidelines for children post-intervention (P = .01) but no statistically significant increase in knowledge for guidelines for adults, for patients that have received prior pneumococcal vaccines, or in overall confidence in recommending pneumococcal vaccines. There was a 17% increase in the number of PCV-20 vaccinations given post-intervention (198 pre-intervention, 232 post-intervention). The estimated PCV-20 vaccination rate for adults aged 19 to 64 years with asthma increased from 14.9% pre-intervention to 19.5% post-intervention (P = .33). CONCLUSIONS: There is a significant knowledge gap regarding ACIP pneumococcal vaccination recommendations among military providers and a low pneumococcal vaccination rate for adults aged 19 to 64 years with asthma at Joint Base-San Antonio MTFs. Focused education sessions were effective in increasing providers' knowledge of updated pneumococcal vaccination recommendations, confidence in recommending vaccines, total number of pneumococcal vaccinations given, and estimated pneumococcal vaccination rate for adults with asthma. The validity of conclusions drawn from our data were limited because of discordant numbers of survey respondents as well as potentially inaccurate estimates of pneumococcal vaccination rates pre- and post-intervention. Despite this, the results warrant continued education of pneumococcal vaccines, indications, and dosing schedules.

Transient Neurologic Deficits Following Intramuscular Epinephrine Administration for the Treatment of Anaphylaxis.

Anaphylaxis is an acute, potentially life-threatening severe allergic reaction commonly caused by foods, insect stings, and medications. Intramuscular epinephrine is the cornerstone of treatment for anaphylaxis in order to reverse immediate symptoms and prevent progression to life-threatening hemodynamic or respiratory collapse. By nature of its mechanism of action, epinephrine may induce a number of neurovascular-related adverse effects; even at usual therapeutic doses. Rarely described adverse events include transient ischemic attacks, ischemic stroke, intracerebral hemorrhage, and myocardial infarction. These events may be observed more frequently in patients with cardiovascular risk factors including hypertension, hyperlipidemia, and diabetes mellitus. We present a case of transient neurologic deficits in a patient with underlying cardiovascular disease related to intramuscular epinephrine use for the treatment of anaphylaxis. This case serves to further highlight serious adverse neurologic events that may result from intramuscular epinephrine administration.

Delayed Hyperpigmented Injection Site Reactions Due to Chronic Dupilumab Use.

Dupilumab is a humanized monoclonal antibody approved for the treatment of chronic rhinosinusitis with nasal polyposis (CRSwNP), asthma, atopic dermatitis, eosinophilic esophagitis, and prurigo nodularis. The most common adverse reactions from dupilumab use are temporary injection site reactions and ocular surface reactions; however, a variety of both acute and delayed cutaneous reactions have also been described. We present a case of delayed hyperpigmented injection site reactions following chronic dupilumab use.

Successful Long-Term Aspirin Desensitization in a Pilot With Aspirin-Exacerbated Respiratory Disease.

Aspirin-exacerbated respiratory disease (AERD) is a triad of asthma, chronic rhinosinusitis with nasal polyposis (CRSwNP), and adverse respiratory reactions to the ingestion of aspirin/non-steroidal anti-inflammatory drugs.1 Patients with AERD are frequently plagued with CRSwNP that is difficult to manage with systemic steroids, nasal steroids, and surgical polypectomy, often requiring multiple endoscopic sinus surgeries and frequent otolaryngology follow-up.2,3 There are an abundance of therapies to treat CRSwNP in the setting of AERD, all with varying costs, efficacies, and indications for treatment.4 While limited by side effect profile, aspirin desensitization remains an effective, low-cost treatment for patients with CRSwNP and non-steroidal anti-inflammatory drug sensitivity.5 We describe a case of an active duty U.S. Air Force pilot with AERD whose CRSwNP was successfully treated with aspirin desensitization without detrimental effect on his flying status.