Drug Allergy Research Articles

EAACI/ENDA position paper on drug provocation testing.

In drug hypersensitivity, drug provocation testing (DPT), also called drug challenge, is the gold standard for investigation. In recent years, risk stratification has become an important tool for adjusting the diagnostic strategy to the perceived risk, whilst still maintaining a high level of safety for the patient. Skin tests are recommended before DPT but may be omitted in low-risk patients. The task force suggests a strict definition of such low-risk patients in children and adults. Based on experience and evidence from studies of allergy to beta-lactam antibiotics, an algorithm on how to adjust DPT to the risk, and when to omit skin tests before DPT, is presented. For other antibiotics, non-steroidal anti-inflammatory drugs and other drugs, skin tests are poorly validated and DPT is frequently necessary. We recommend performing DPT with chemotherapeutics and biologicals to avoid unnecessary desensitization procedures and DPT with skin tests negative contrast media. We suggest DPT with anesthetics only in highly specialized centers. Specifics of DPT to proton pump inhibitors, anticonvulsants and corticosteroids are discussed. This position paper provides general recommendations and guidance on optimizing use of DPT, whilst balancing benefits with patient safety and optimizing the use of the limited available resources.

Deciphering drug discovery and microbial pathogenesis research in tuberculosis during the two decades of postgenomic era using entity mining approach.

We examined literature on Mycobacterium tuberculosis (Mtb) subsequent to its genome release, spanning years 1999-2020. We employed scientometric mapping, entity mining, visualization techniques, and PubMed and PubTator databases. Most popular keywords, most active research groups, and growth in quantity of publications were determined. By gathering annotations from the PubTator, we determined direction of research in the areas of drug hypersensitivity, drug resistance (AMR), and drug-related side effects. Additionally, we examined the patterns in research on Mtb metabolism and various forms of tuberculosis, including skin, brain, pulmonary, extrapulmonary, and latent tuberculosis. We discovered that 2011 had the highest annual growth rate of publications, at 19.94%. The USA leads the world in publications with 18,038, followed by China with 14,441, and India with 12,158 publications. Studies on isoniazid and rifampicin resistance showed anenormous increase. Non-tuberculous mycobacteria also been the subject of more research in effort to better understand Mtb physiology and as model organisms. Researchers alsolooked at co-infections like leprosy, hepatitis, plasmodium, HIV, and other opportunistic infections. Host perspectives like immune response, hypoxia, and reactive oxygen species, as well as comorbidities like arthritis, cancer, diabetes, and kidney disease etc. were also looked at. Symptomatic aspects like fever, coughing, and weight loss were also investigated. Vitamin D has gained popularity as a supplement during illness recovery, however, the interest of researchers declined off late. We delineated dominant researchers, journals, institutions, and leading nations globally, which is crucial for aligning ongoing and evolving landscape of TB research efforts. Recognising the dominant patterns offers important information about the areas of focus for current research, allowing biomedical scientists, clinicians, and organizations to strategically coordinate their efforts with the changing priorities in thefield of tuberculosis research.

Evaluation of risk factors for recurrence of cutaneous adverse reactions due to anti-seizure medications in children: A retrospective study.

Background: Cutaneous adverse reactions (CARs) are one of the most important reasons for anti-seizure medication (ASM) discontinuation in epilepsy. However, such discontinuations can cause an increase in seizures. This study investigates the risk factors for ASM-related rash recurrence in children. Methods: This retrospective case-control study consisted of the patient group with a single rash due to ASMs (group 1), the patient group with rash recurrence (group 2), and the control group. While the demographic and clinical features of group 1 and the control group were compared in terms of a single rash, group 1 and group 2 were compared for rash recurrence. Results: Group 1, group 2, and control group consisted of 112, 33, and 166 patients, respectively. Female gender was a risk factor for a single rash (P < 0.001) but not for recurrence (P = 0.439). Presence of atopic disease [odds ratio (OR): 9.5, 95% confidence interval (CI): 3.8-23.1, P < 0.001], family history of drug allergy (OR: 26.3, 95% CI: 9.6-72.1, P < 0.001), and polytherapy (OR: 23.5, 95% CI: 8.7-62.9, P < 0.001) were risk factors for rash recurrence. Aromatic nature of both the ASMs associated with the first rash (OR: 14.4, 95% CI: 3.2-63.2, P < 0.001) and rash recurrence (OR: 11.3, 95% CI: 4.6-27.5, P < 0.001) were determined as risk factors separately. Conclusion: Careful use of aromatic drugs may prevent recurrence of ASM-related CAR in children, particularly in cases of personal history of allergic disease and family history of drug allergy.

Premedication of Ranitidine and the Action of Hypersensitivity Reactions to Paclitaxel

Paclitaxel is used asa chemotherapeutic agent for curing various types of cancer. To reduce the hypersensitivity of the chemotherapeutic effect, premedication of antihistaminic drugs like ranitidine, cetirizine, diphenhydramine, fexofenadine and desloratadine are prescribed. The antihistaminic drugs are given as premedication to prevent the formation of hives on the body. Ranitidine can be replaced by another antihistaminic drug which reduces the side effects and increases the efficacy of the drug. Studies were conducted comparing the antihistamine and non-antihistamine groups and tests like the chi-square test, and the Wilcoxon-Mann-Whitney test to determine the extent of hypersensitivity. There are immediate and non-immediate drug hypersensitivity reactions occurring in cells that initiate the allergic reaction. These allergic reactions like flushing, itching, dizziness, nausea, vomiting, swelling, etc lead to affect the respiratory, cardiovascular, gastrointestinal, and other body parts. In this article, the method used for the study of hypersensitivity reaction is specified with the test to determine the allergic reaction on the skin and desensitization of the rapid hypersensitivity of the drug.

Papel del radiofarmacéutico clínico en la seguridad del paciente durante los estudios de perfusión miocárdica de esfuerzo con vasodilatadores

AimTo assess the radiopharmacist's role in a multidisciplinary team focused on the contraindications of regadenoson in order to ensure the safe use of pharmacologic vasodilator stress agents in patients undergoing SPECT-MPI. MethodsWe ambispectively studied its safe use in 1905 patients (54.1% female, mean age: 66.6±11.7 years, range: 20-95years). Sex, age, medical history, medications, drug allergies, and contraindications for stress testing were registered together with recommendations for the nuclear physician in charge. ResultsDetected contraindications and corresponding recommendations were as follows: risk factors for QTc interval prolongation 7.5% — measurement of QTc interval previously to test and monitor ECG; prior stroke or TIA 4.2% — consider carotid stenosis assessment; salicylates/sulfonamides allergy 3.1% — use 99mTc-sestamibi; epilepsy or risk factors for seizures 2.4% — use of adenosine or reconsider test indication; systemic corticosteroid therapy for severe COPD 1.3% — reassessment of patient's condition; acute exacerbation of COPD 0.8% — defer test until acute episode is over; severe asthma 0.4% — do not perform test; methylxanthine ingestion 0.3% — avoid consumption previously; other 6.1% — evaluation of other contraindications. No contraindications were detected in 73.6% of patients. The test was cancelled due to absolute contraindications in 2.9% of the requests. ConclusionsWorking in a systematic way, the radiopharmacist was able to detect a high number of issues related to regadenoson, with one out of four patients presenting some clinical contraindication. The recommendations given by the radiopharmacist were well accepted by the nuclear physicians who changed their approach contributing to increase the safety of patients referred for MPI.

Drug allergy in childhood

Drug allergy in childhood

Patient Adherence to Written Instructions following Complete Allergological Evaluation for Suspected Beta-Lactam Allergy: A Tertiary Hospital Study in Greece.

Beta-lactam (BL) antibiotics are among the most prescribed groups of drugs worldwide and have been implicated in a variety of allergic reactions. There is a paucity of literature regarding patient adherence to prescribed instructions following comprehensive allergy assessments. The objective was to follow up the clinical course of BL allergy in patients who underwent thorough allergological investigation for suspected BL allergy at a tertiary hospital and ascertain patients' compliance with the provided written instructions. An observational study in patients referred for suspected BL allergy who underwent a comprehensive allergy workup (in vivo ± in vitro tests, DPT in culprit and/or alternative BL) and who subsequently received written instructions was conducted. Data on the nature of the reported drug hypersensitivity reaction, the culprit BL drug, the allergological workup, and the detailed instructions provided in a written drug allergy report were collected retrospectively. Patients' compliance with the instructions was recorded by a telephone survey using a pre-defined questionnaire. Among the 212 patients meeting the inclusion criteria, 87 patients (72.4% women; mean age 50.1 years; age range 6-84 years) responded to the telephone survey and were included in this study. Surprisingly, 45 out of 87 (51.7%) patients did not adhere to the written instructions. The primary factor contributing to non-compliance was the fear of re-occurrence of a drug-induced allergic reaction (personal and/or triggered by their treating physician reluctance), accounting for 77.7% of cases. The analysis demonstrated that the initial reaction's severity and type, as well as the outcomes of skin testing, did not correlate with compliance to instructions (p > 0.05). Surprisingly enough, a drug provocation test (DPT), irrespectively of the result, emerged as a negative predictor for adherence, with only 40.6% of DPT patients complying compared to 77.8% of those who did not undergo DPT (p = 0.005; odds ratio = 0.195; 95% confidence interval: 0.058-0.655). Variables such as performing DPT with alternative or incriminated drugs or the result of the DPT (positive-negative) were not associated with patient compliance. Conversely, the type of instructions provided exhibited a noteworthy correlation with compliance. Patients who were explicitly instructed to entirely avoid all BL antibiotics demonstrated markedly higher adherence rates (83.3%) compared to those who were advised to have a partial or complete release of BLs (31.8% and 58.1%, respectively; p < 0.05). Notably, among compliant patients who received either the original culprit drug or the alternative (32 out of 87, 36.7%), no allergic reactions were reported. In contrast, among the 12 patients with written avoidance of all BLs, subsequent BL intake led to immediate reactions (Grade I and IV) in 2 patients (16.6%). A notable disparity in patient adherence to written instructions prohibiting or releasing beta-lactams was demonstrated. Less than half of the patients ultimately complied with the provided instructions, underscoring the need for tailored patients' education and strategies to improve adherence in the management of suspected BL allergy.

Ciprofloxacin Ophthalmic DRESS Syndrome: Case Report

Introduction: Drug rash with eosinophilia and systemic symptoms (DRESS syndrome), also known as drug-induced hypersensitivity syndrome, is a rare severe systemic hypersensitivity drug reaction. Diagnosing DRESS syndrome is challenging due to non-specific manifestations that can make it difficult to recognize. Therefore, addressing and discussing this issue is extremely important, considering the potential lethality of a treatable syndrome. Clinical case: We describe the clinical characteristics of a 39-year-old female who presented with DRESS syndrome associated with the use of ciprofloxacin ophthalmic. The patient manifested a mild maculopapular rash located in the pelvic limbs with subsequent ascending distribution and generalized erythematoviolaceous affecting four extremities, trunk, facial region and scalp, together with fever and lymphadenopathy. Paraclinical tests revealed leukocytosis and eosinophilia. According to RegiScar's ranking, it scored 6 points, classifying it as the definitive case for DRESS. He concluded a schedule with prednisone 1 mg/kg/day with a slow reduction dose, obtaining a good response to treatment. Conclusions: DRESS syndrome should be suspected in a patient receiving medical treatment who presents with the following signs and symptoms: rash, fever (38°C), facial edema, and lymphadenopathy. Fortunately, this reaction is usually reversible, with a low incidence of residual damage or mortality, in the case of timely discontinuation of antibiotics and use of topical or systemic corticosteroids.

A multiple signal amplification photoelectrochemical biosensor based on biotin-avidin system for kanamycin sensing in fish and milk via synergism of g-C3N4 and Ru@SiO2

BackgroundThe residues of kanamycin can accumulate in the human body for a long time and pose serious health risks, including hearing loss, kidney poisoning, and drug allergic reactions. Therefore, it is crucial to develop a rapid, highly sensitive, and low-cost method for detecting kanamycin residues in foods. However, the current methods have limitations such as low sensitivity, expensive instruments, and multiple steps, which make them impractical for use in resource-limited environments and emergencies. In this study, the creation of a multiple-signal amplification photoelectrochemical biosensor to address these aforementioned issues is discussed. ResultsHerein, we proposed a multiple signal amplification photoelectrochemical (PEC) biosensor based on carboxylated g-C3N4 and avidin functionalized Ru@SiO2 for the ultrasensitive detection of kanamycin. The carboxylated g-C3N4 was a highly efficient photoactive substance for amplifying photoelectric signals and a substrate for aptamer immobilization. The DOS and PDOS of g-C3N4 were studied by simulation, and the sensing mechanism of the probe at the molecular level was revealed. Meanwhile, using Ru@SiO2 as a signal amplifying unit, through the cooperative work between Ru@SiO2 and g-C3N4, the photoelectric signal could be double amplified to produce an excellent photocurrent response. Under optimized conditions, the photocurrent response of the PEC biosensor to kanamycin was obtained at concentrations from 0.1 nM to 1000 nM with a lower detection limit of 4.1052 × 10−11 mol L−1. This protocol demonstrates high sensitivity, brilliant specific recognition ability, excellent reproducibility, and acceptable stability. SignificanceThe first combination of g-C3N4 and avidin-Ru@SiO2 as photocurrent materials greatly enhanced the sensitivity of the PEC biosensors. Moreover, the specificity and sensitivity of the PEC biosensor were further improved through the specific interaction between kanamycin and aptamer. The photoelectric conversion mechanism based on g-C3N4 and two pathways for enhancing the photocurrent by Ru(byp)32+ were proposed. Through simulations of the DOS and PDOS of g-C3N4, the sensing mechanism of the probe at the molecular level was revealed. Under the optimum conditions, the PEC biosensor exhibited a wide linear concentration range and a low detection limit.

Changing patterns in the epidemiology of drug allergy.

Drug allergy (DA) remains a complex and unaddressed problem worldwide that often deprives patients of optimal medication choices and places them at risk for life-threatening reactions. Underdiagnosis and overdiagnosis are common and due to the lack of standardized definitions and biomarkers. The true burden of DA is unknown, and recent efforts in data gathering through electronic medical records are starting to provide emerging patterns around the world. Ten percent of the general population engaged in health care claim to have a DA, and the most common label is penicillin allergy. Up to 20% of emergency room visits for anaphylaxis are due to DA and 15%-20% of hospitalized patients report DA. It is estimated that DA will increase based on the availability and use of new and targeted antibiotics, vaccines, chemotherapies, biologicals, and small molecules, which are aimed at improving patient's options and quality of life. Global and regional variations in the prevalence of diseases such as human immunodeficiency virus and mycobacterial diseases, and the drugs used to treat these infections have an impact on DA. The aim of this review is to provide an update on the global impact of DA by presenting emerging data on drug epidemiology in adult and pediatric populations.

Lymphocyte transformation tests predict delayed-type allergy to piperacillin/tazobactam in patients with cystic fibrosis

BackgroundAntibiotic treatment is crucial for patients with chronic bacterial infections. Suspected drug allergies often lead to inconsistent therapies and challenging clinical management for patients and caregivers. The objective of this study was to evaluate the value of lymphocyte transformation tests in comparison to skin tests for the prediction of delayed-type allergic reactions. MethodsThis prospective, observational study tested the diagnostic value of skin prick tests, intradermal tests (reading: 15 min and 72 h) and lymphocyte transformations tests for the prediction of allergic reactions in CF patients with physician reported allergy to piperacillin/tazobactam, meropenem and ceftazidime. The tests were performed directly before a 14d intravenous drug challenge. ResultsWe performed 33 drug challenges in 29 subjects. 21 drug challenges were negative (63 %); 12 lead to a reaction (37 %), of those 2 were immediate and 10 were delayed-type. 100 % of the skin prick tests were negative. 97 % (33/34) of the intradermal tests with early reading and 100 % of the intradermal tests with late reading yielded negative results. 5/11 patients who experienced a delayed-type reaction during the drug challenge had a positive lymphocyte transformations test. All 17 patients who did not react had a negative lymphocyte transformations test. For piperacillin/tazobactam, 4/5 patients who experienced a delayed-type reaction during the drug challenge had positive lymphocyte transformations tests. Hence, for piperacillin/tazobactam, the sensitivity of the lymphocyte transformation test for prediction of reactions was 80.0 % and the specificity 100 %. ConclusionWe demonstrate that the lymphocyte transformation test predicts delayed-type allergy to piperacillin/tazobactam in contrast to skin tests.

Flow-based basophil activation test in immediate drug hypersensitivity. An EAACI task force position paper.

Diagnosing immediate drug hypersensitivity reactions (IDHRs) can pose a significant challenge and there is an urgent need for safe and reliable tests. Evidence has emerged that the basophil activation test (BAT), an invitro assay that mirrors the invivo response, can be a complementary test for many drugs. In this position paper, members of Task Force (TF) "Basophil activation test in the evaluation of Drug Hypersensitivity Reactions" from the European Academy of Allergy and Clinical Immunology (EAACI) present the data from a survey about the use and utility of BAT in IDHRs in Europe. The survey results indicate that there is a great interest for using BAT especially for diagnosing IDHRs. However, there are still main needs, mainly in the standardization of the protocols. Subsequently consensus-based recommendations were formulated for: (i) Technical aspects of BAT in IDHRs including type of sample, management of drugs, flow cytometry protocols, interpretation of the results; and (ii) Drug-specific aspects that should be taken into account when performing BAT in relation to betalactams, neuromuscular blocking agents, fluoroquinolones, chlorhexidine, opioids, radio contrast media, chemotherapeutics, biological agents, nonsteroidal anti-inflammatory drugs, COVID vaccine, and excipients. Moreover, aspects in the evaluation of pediatric population have also been considered. All this indicates that BAT offers the clinician and laboratory a complementary tool for a safe diagnostic for IDHRs, although its place in the diagnostic algorithm depends on the drug class and patient population (phenotype, geography, and age). The standardization of BAT is important for generalizing this method beyond the individual laboratory.

Genetic Susceptibility of HLA Alleles to Non-Steroidal Anti-Inflammatory Drug Hypersensitivity in the Taiwanese Population.

Human leukocyte antigen (HLA) genes are important in many immune processes and contribute to many adverse drug reactions. Whether genetic variations in the HLA region are associated with non-steroid anti-inflammatory drug (NSAID) hypersensitivity remains uncertain. Therefore, the aim of our study was to identify HLA genetic variations in patients with NSAID hypersensitivity in the Taiwanese population. This hospital-based, retrospective case-control study enrolled 37,156 participants with NSAID exposure from the Taiwan Precision Medicine Initiative (TPMI), who were all genotyped and imputed to fine map HLA typing. Our study assigned 1217 cases to the NSAID allergy group and 12,170 controls to a matched group. Logistic regression analyses were utilized to explore associations between HLA alleles and NSAID hypersensitivity. Overall, 13,387 patients were genotyped for eight major HLA alleles. Allele frequencies were different between the two groups. In the NSAID allergy group, the genotype frequencies of HLA-A*02:01, HLA-A*34:01, and HLA-DQA1*06:01 were found to be markedly elevated compared to the control group, a significance that persisted even after applying the Bonferroni correction. Furthermore, the risk of NSAID allergy demonstrated a significant association with HLA-A*02:01 (OR = 1.29, p < 0.001) and HLA-A*34:01 (OR = 9.90, p = 0.001), in comparison to their respective counterparts. Notably, the genotype frequency of HLA-B*46:01 exhibited a significant increase in the severe allergy group when compared with the mild allergy group. We identified HLA genotypes linked to the onset and severity of NSAID hypersensitivity. Our findings establish a basis for precision prescription in future clinical applications.

Drug allergy management in Egypt, Sri Lanka and the Caribbean: A qualitative study.

Drug allergy management in Egypt, Sri Lanka and the Caribbean: A qualitative study.

Prior drug allergies are associated with worse outcome in patients with idiosyncratic drug-induced liver injury: A machine learning approach for risk stratification

The impact of prior drug allergies (PDA) on the clinical features and outcomes of patients who develop idiosyncratic drug-induced liver injury (DILI) is largely unknown. We aimed to assess the clinical presentation and outcomes of DILI patients based on the presence or absence of PDA and explore the association between culprit drugs responsible for DILI and allergy. We analysed a well-vetted cohort of DILI cases enrolled from the Spanish DILI Registry. Bootstrap-enhanced least absolute shrinkage operator procedure was used in variable selection, and a multivariable logistic model was fitted to predict poor outcomes in DILI. Of 912 cases with a first episode of DILI, 61 (6.7%) had documented PDA. Patients with PDA were older (p = 0.009), had higher aspartate aminotransferase (AST) levels (p = 0.047), lower platelet count (p = 0.011) and higher liver-related mortality than those without a history of drug allergies (11% vs. 1.6%, p < 0.001). Penicillin was the most common drug associated with PDA in DILI patients (32%). A model including PDA, nR-based type of liver injury, female sex, AST, total bilirubin, and platelet count showed an excellent performance in predicting poor outcome in patients from the Spanish DILI Registry (area under the ROC curve [AUC] 0.887; 95% confidence interval [CI] 0.794 – 0.981) and the LATINDILI Network (AUC 0.932; 95% CI 0.884 – 0.981). Patients with suspected DILI should be screened for PDA as they would require a close monitoring for early detection of worsening clinical course.

Atypical Intralymphatic T-Cell Proliferation during DRESS Mimicking Intravascular Lymphoma: Case Report

Drug-induced hypersensitivity syndrome or drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe drug-induced reaction that represents a life-threatening condition. The histopathologic appearance of the skin biopsy is nonspecific but may exceptionally show an atypical intralymphatic lymphocytic infiltrate, and mimic an intravascular lymphoma. Through this article, we will report an exceptional case of a patient presenting with skin lesions pointing to a dress, but whose histological appearance, in the presence of adenopathy during physical examination, can lead, by mistake, to the diagnosis of an intralymphatic lymphoma. We aim to highlight the various diagnostic indices allowing the distinction between these two entities, including the immunohistochemical study, the search for the TcR clonal rearrangement and the anatomo-clinical correlation.

Delayed postoperative reactions to metamizole: a diagnostic challenge.

Background. Metamizole, a non-steroidal anti-inflammatory drug from the pyrazolone group, is a frequent cause of immediate hypersensitivity reactions and, more rarely, of delayed drug hypersensitivity reactions. Due to its favorable pharmacokinetic characteristics, metamizole is widely used in the postoperative period for pain control. Methods. Retrospective study of patients referred for allergological study between January 2012 and June2022 for postoperative hypersensitivity reactions. Clinical and diagnostic data were collected through review of patients' medical records. Twenty patients with postoperative hypersensitivity reactions were referred, of which 10 presented delayed reactions. We analyzed the results of skin prick, intradermal and patch tests performed with an intravenous metamizole solution as well as provocation tests performed with metamizole and acetylsalicylic acid. Cross-reactivity to non-steroidal anti-inflammatory drugs was excluded by confirmation of clinical tolerance to non-steroidal anti-inflammatory drugs or by acetylsalicylic acid provocation test. Results. In 7 of the 10 patients a delayed reaction to metamizole was diagnosed. These reactions were characterized as maculopapular exanthema, occurring in multiple postoperative settings. Skin tests were negative, except in one patient with late mild erythema in the ipsilateral upper limb and no reaction at the site of intradermal injection. Delayed hypersensitivity was demonstrated by late positive metamizole provocation tests. Conclusions. This study demonstrated that for a correct diagnosis a high degree of suspicion about possible delayed hypersensitivity drug reactions to metamizole in the postoperative setting is needed. In the investigation, provocation test with metamizole was decisive for diagnostic confirmation.

Safety andeffectiveness ofdenosumab inJapanese patients with rheumatoid arthritis: A 2-year post-marketing surveillance study.

To investigate the 2 year safety and effectiveness of denosumab 60 mg in patients with rheumatoid arthritis (RA) in clinical practice in Japan. This 2 year, prospective, observational cohort study included patients who initiated treatment with denosumab 60 mg for the progression of bone erosion associated with RA. Key endpoints were adverse drug reactions (ADRs), progression of bone erosion, and 28-joint Disease Activity Score based on C-reactive protein or erythrocyte sedimentation rate. Univariate and multivariate analyses were conducted to determine the risk factors for ADRs and the progression of bone erosion. In the safety analysis set (n = 1239), the incidence of ADRs was 3.0%; the most common ADRs were hypocalcaemia (1.2%) and osteonecrosis of jaw-related events (0.6%). A history of any drug allergy was a statistically significant risk factor associated with the occurrence of ADRs. In the effectiveness analysis set (n = 815), the incidence of progression of bone erosion was 8.7%. Steinbrocker stage and initial steroid dose were statistically significant risk factors associated with the progression of bone erosion. Denosumab demonstrated safety and effectiveness over a 2 year period in RA patients without any new safety concerns.

X-Linked Inhibitor of Apoptosis (XIAP) deficiency presenting as hemophagocytic lymphohistiocytosis triggered by drug allergy

X-Linked Inhibitor of Apoptosis (XIAP) deficiency presenting as hemophagocytic lymphohistiocytosis triggered by drug allergy

РЕАКЦИЯ ЛЕКАРСТВЕННОЙ ГИПЕРЧУВСТВИТЕЛЬНОСТИ С ЭОЗИНОФИЛИЕЙ И СИСТЕМНЫМИ СИМПТОМАМИ (DRESS-СИНДРОМ) НА КАРБАМАЗЕПИН. Клинический случай и обзор литературы

Abstract. Introduction. A drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially life- threatening T-cell mediated reaction characterized by heterogeneous clinical manifestations and by a variable and unpredictable course. It is difficult to diagnose this reaction due to the long latent period between the start of therapy with a new drug and onset of symptoms, as well as due to no pathognomonic manifestations. Aim. Providing physicians with an algorithm for the diagnosis and treatment of DRESS syndrome exemplified by our own clinical observation. Materials and Methods. A 70-year-old female patient was hospitalized in the Department of Allergology and Immunology, complaining of generalized itchy rash on the face, body, and extremities, swelling of face and hands, and temperature rise to 37.5-38 °C. She was examined using general clinical, laboratory and instrumental methods. Results and Discussion. On examination, we found generalized maculopapular rash, increased levels of hepatic transaminases, reaching the criteria for mild drug-induced liver damage, and peripheral blood eosinophilia 25% (4,260 cells in 1 μl). Using the RegiSCAR scoring system helped to diagnose drug hypersensitivity reaction with eosinophilia and systemic symptoms, while utilizing the algorithm for assessing the causal relationship between the drug and the development of symptoms allowed considering carbamazepine as a possible cause of this reaction. The patient was treated with systemic and topical glucocorticosteroids with positive changes. Conclusions. Using the RegiSCAR scoring system developed by a team of international experts facilitates diagnosing the DRESS syndrome. Identifying and withdrawing the culprit drug is an essential condition of successfully managing patients. Systemic and topical glucocorticosteroids are the drugs of choice for the treatment of drug reactions with eosinophilia and systemic symptoms.