Pub Date : 2023-08-23DOI: 10.1016/j.clicom.2023.08.002
Yoshiyasu Takefuji , Junya Toyokura
Goal of health policies is to protect and promote the health of communities. We examined COVID-19 policy outcomes of the 50 US states according to policymaker assumptions over time. With daily cumulative population mortality chosen as an indicator to evaluate and score outcomes of individual health policies, Hawaii had the best score and Arizona has the worst score. Our policy outcome analysis tool could identify and quantify policymakers’ faulty assumptions against COVID-19, and concludes that the more COVID-19 deaths, the greater the economic loss.
{"title":"Time-series COVID-19 policy outcome analysis of the 50U.S. states","authors":"Yoshiyasu Takefuji , Junya Toyokura","doi":"10.1016/j.clicom.2023.08.002","DOIUrl":"https://doi.org/10.1016/j.clicom.2023.08.002","url":null,"abstract":"<div><p>Goal of health policies is to protect and promote the health of communities. We examined COVID-19 policy outcomes of the 50 US states according to policymaker assumptions over time. With daily cumulative population mortality chosen as an indicator to evaluate and score outcomes of individual health policies, Hawaii had the best score and Arizona has the worst score. Our policy outcome analysis tool could identify and quantify policymakers’ faulty assumptions against COVID-19, and concludes that the more COVID-19 deaths, the greater the economic loss.</p></div>","PeriodicalId":100269,"journal":{"name":"Clinical Immunology Communications","volume":"4 ","pages":"Pages 51-54"},"PeriodicalIF":0.0,"publicationDate":"2023-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49753029","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The acute respiratory distress syndrome (ARDS) is a common cause of respiratory failure in critically ill patients and is defined by the acute onset of noncardiogenic pulmonary edema, hypoxemia, and the need for mechanical ventilation. ARDS occurs most often in the setting of pneumonia, sepsis, aspiration of gastric contents or severe trauma, and is present in ∼10% of all intensive care unit patients worldwide. Pathologic specimens from patients with ARDS most frequently reveal diffuse alveolar damage, and laboratory studies have demonstrated both alveolar epithelial and lung endothelial injury, resulting in accumulation of protein-rich inflammatory edema fluid in the alveolar space. The current therapeutic regimen is comprised of supportive measures such as lung protective ventilation, restrictive fluid management, paralyzing drugs, and prone positioning. Although vast improvements have been made in ARDS-treatment during the last five decades, mortality among patients with severe ARDS remains at an unacceptable rate of 45%.This article reviews the evolution of the currently used definition, established pathophysiological mechanism, highlights the current best clinical practice to treat ARDS, gives a brief outlook on cutting edge trends in ARDS research and closes with an expert opinion on the subject. The ongoing digital revolution will help to individualize ARDS-treatment and will therefore presumably improve survival and quality of life.
{"title":"Novel approaches that promote lung endothelial and epithelial repair and anti pro inflammatory cytokines could be a future promising agent in the management of ARDS","authors":"Montaser Alrjoob , Alaa Alkhatib , Rana Padappayil , Husam Bader , Doantrang Du , Chandler Patton","doi":"10.1016/j.clicom.2023.07.005","DOIUrl":"https://doi.org/10.1016/j.clicom.2023.07.005","url":null,"abstract":"<div><p>The acute respiratory distress syndrome (ARDS) is a common cause of respiratory failure in critically ill patients and is defined by the acute onset of noncardiogenic pulmonary edema, hypoxemia, and the need for mechanical ventilation. ARDS occurs most often in the setting of pneumonia, sepsis, aspiration of gastric contents or severe trauma, and is present in ∼10% of all intensive care unit patients worldwide. Pathologic specimens from patients with ARDS most frequently reveal diffuse alveolar damage, and laboratory studies have demonstrated both alveolar epithelial and lung endothelial injury, resulting in accumulation of protein-rich inflammatory edema fluid in the alveolar space. The current therapeutic regimen is comprised of supportive measures such as lung protective ventilation, restrictive fluid management, paralyzing drugs, and prone positioning. Although vast improvements have been made in ARDS-treatment during the last five decades, mortality among patients with severe ARDS remains at an unacceptable rate of 45%.This article reviews the evolution of the currently used definition, established pathophysiological mechanism, highlights the current best clinical practice to treat ARDS, gives a brief outlook on cutting edge trends in ARDS research and closes with an expert opinion on the subject. The ongoing digital revolution will help to individualize ARDS-treatment and will therefore presumably improve survival and quality of life.</p></div>","PeriodicalId":100269,"journal":{"name":"Clinical Immunology Communications","volume":"4 ","pages":"Pages 45-50"},"PeriodicalIF":0.0,"publicationDate":"2023-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49753027","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-07-22DOI: 10.1016/j.clicom.2023.07.003
Anna Vanoverschelde , Samer R. Khan , Virgil A.S.H. Dalm , Layal Chaker , Guy Brusselle , Bruno H. Stricker , Lies Lahousse
Objectives
Elderly become more susceptible to lower respiratory tract infections, resulting in antibiotic prescriptions. Immunoglobulins (Ig) play an important role in host defense and protection against infections. Therefore, we aimed to investigate whether lower Ig levels are a risk factor for antibiotic use in the general elderly population.
Methods
After exclusion of current antibiotic users, Cox proportional-hazards regression models were performed to investigate the effect of stable serum IgM, IgG and IgA levels on time to first antibiotic prescription within the Rotterdam Study. Regression models were adjusted for age, sex, body mass index, smoking status and diabetes. We introduced quadratic terms and additionally categorized Igs to explore and quantify potential non-linearity of the association. The restricted cubic splines technique was used to plot the natural log of the hazard across Ig level.
Results
In total, 8,639 participants were included (mean age 64 years, 57% female, medium follow-up 3.2 years). No significant association between IgM and time to antibiotic prescription was observed. IgG and IgA levels (in g/L) showed a U-shaped relationship with time to antibiotic prescription (linear IgG HR 0.959, 95% CI 0.930–0.989; quadratic IgG² HR 1.002, 95% CI 1.000–1.003; linear IgA HR 0.949, 95% CI 0.910–0.990; quadratic IgA² HR 1.009, 95% CI 1.004–1.013).
Conclusion
Both low and high IgG and IgA levels were associated with a higher incidence of antibiotic prescriptions in stable middle-aged and older individuals. Increased awareness for the potential increased infection risk when persons have low or high Ig levels, even within the reference ranges, is needed.
{"title":"Serum immunoglobulin levels and risk of antibiotic prescription in middle-aged and older individuals: A population-based cohort study","authors":"Anna Vanoverschelde , Samer R. Khan , Virgil A.S.H. Dalm , Layal Chaker , Guy Brusselle , Bruno H. Stricker , Lies Lahousse","doi":"10.1016/j.clicom.2023.07.003","DOIUrl":"https://doi.org/10.1016/j.clicom.2023.07.003","url":null,"abstract":"<div><h3>Objectives</h3><p>Elderly become more susceptible to lower respiratory tract infections, resulting in antibiotic prescriptions. Immunoglobulins (Ig) play an important role in host defense and protection against infections. Therefore, we aimed to investigate whether lower Ig levels are a risk factor for antibiotic use in the general elderly population.</p></div><div><h3>Methods</h3><p>After exclusion of current antibiotic users, Cox proportional-hazards regression models were performed to investigate the effect of stable serum IgM, IgG and IgA levels on time to first antibiotic prescription within the Rotterdam Study. Regression models were adjusted for age, sex, body mass index, smoking status and diabetes. We introduced quadratic terms and additionally categorized Igs to explore and quantify potential non-linearity of the association. The restricted cubic splines technique was used to plot the natural log of the hazard across Ig level.</p></div><div><h3>Results</h3><p>In total, 8,639 participants were included (mean age 64 years, 57% female, medium follow-up 3.2 years). No significant association between IgM and time to antibiotic prescription was observed. IgG and IgA levels (in g/L) showed a U-shaped relationship with time to antibiotic prescription (linear IgG HR 0.959, 95% CI 0.930–0.989; quadratic IgG² HR 1.002, 95% CI 1.000–1.003; linear IgA HR 0.949, 95% CI 0.910–0.990; quadratic IgA² HR 1.009, 95% CI 1.004–1.013).</p></div><div><h3>Conclusion</h3><p>Both low and high IgG and IgA levels were associated with a higher incidence of antibiotic prescriptions in stable middle-aged and older individuals. Increased awareness for the potential increased infection risk when persons have low or high Ig levels, even within the reference ranges, is needed.</p></div>","PeriodicalId":100269,"journal":{"name":"Clinical Immunology Communications","volume":"4 ","pages":"Pages 23-29"},"PeriodicalIF":0.0,"publicationDate":"2023-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49752593","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-07-22DOI: 10.1016/j.clicom.2023.07.002
Nina C. Nwade , Sachi I. Desse , Akanksha Nagarkar , Nia E.R. James , Ryan Svoboda , Ginette A. Okoye , Jillian M. Richmond , Angel S. Byrd
Cutaneous T-cell lymphoma (CTCL) serves as an umbrella term for numerous lymphomas that reside in or recirculate through the skin. One such systemic lymphoma is Sézary Syndrome (SS). Although SS is not as common in Skin of Color (SOC), minority patients presenting with the condition do have a different presentation than their White counterparts. In this graphical review, we provide an overview of SS immunopathogenesis and varying presentations. Additionally, a summary of current treatment options is provided, highlighting ongoing clinical trials and opportunities to include SOC patients to promote health equity. SOC patients often have poorer prognosis due to biological differences and health disparities resulting in delayed diagnosis. This exemplifies the potential advantages and promising solutions of precision medicine implementation. To this end, the impact and benefits of precision medicine are summarized.
{"title":"Immunopathogenesis of cutaneous T-cell lymphoma in skin of color patients part 2: Sézary syndrome","authors":"Nina C. Nwade , Sachi I. Desse , Akanksha Nagarkar , Nia E.R. James , Ryan Svoboda , Ginette A. Okoye , Jillian M. Richmond , Angel S. Byrd","doi":"10.1016/j.clicom.2023.07.002","DOIUrl":"https://doi.org/10.1016/j.clicom.2023.07.002","url":null,"abstract":"<div><p>Cutaneous T-cell lymphoma (CTCL) serves as an umbrella term for numerous lymphomas that reside in or recirculate through the skin. One such systemic lymphoma is Sézary Syndrome (SS). Although SS is not as common in Skin of Color (SOC), minority patients presenting with the condition do have a different presentation than their White counterparts. In this graphical review, we provide an overview of SS immunopathogenesis and varying presentations. Additionally, a summary of current treatment options is provided, highlighting ongoing clinical trials and opportunities to include SOC patients to promote health equity. SOC patients often have poorer prognosis due to biological differences and health disparities resulting in delayed diagnosis. This exemplifies the potential advantages and promising solutions of precision medicine implementation. To this end, the impact and benefits of precision medicine are summarized.</p></div>","PeriodicalId":100269,"journal":{"name":"Clinical Immunology Communications","volume":"4 ","pages":"Pages 30-34"},"PeriodicalIF":0.0,"publicationDate":"2023-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49752599","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-07-20DOI: 10.1016/j.clicom.2023.07.001
Ahmed Safi Vahidy , Faizan Niaz , Samiuddin Tariq , Irtebaat Fatima , Yusra Afzal , Abdulqadir J. Nashwan
Aim
To review the available evidence on the efficacy and safety profiles of four Interleukin-23 inhibitors in patients with Psoriatic Arthritis.
Methods
Several databases were searched till July 2022. A total of 11 RCTs with at least one treatment arm were included. All articles were in English. The primary outcomes were ACR20, HAQ-DI, PASI90, and TEAEs experienced by the patients.
Results
Compared to other groups, Guselkumab had the strongest association with ACR20 response (RR: 2.14; 95% CI: 1.84-2.49, p < 0.00001), while a mean change in HAQ-DI (MD: -0.24; 95% CI: -0.41- -0.13, p = 0.0001) and PASI90 (RR: 9.81; 95% CI: 3.18-30.22; p <0.01) were most strongly associated with Ustekinumab. TEAEs were significantly present in the Guselkumab group (RR: 1.26; 95% CI: 1.00 - 1.59; p = 0.05).
Conclusion
Although our analysis suggests that IL-23 inhibitors are efficacious at treating psoriatic arthritis, further studies are required for long-term outcomes.
{"title":"IL-23 Inhibitors to treat psoriatic arthritis: A systematic review & meta-analysis of randomized controlled trials","authors":"Ahmed Safi Vahidy , Faizan Niaz , Samiuddin Tariq , Irtebaat Fatima , Yusra Afzal , Abdulqadir J. Nashwan","doi":"10.1016/j.clicom.2023.07.001","DOIUrl":"https://doi.org/10.1016/j.clicom.2023.07.001","url":null,"abstract":"<div><h3>Aim</h3><p>To review the available evidence on the efficacy and safety profiles of four Interleukin-23 inhibitors in patients with Psoriatic Arthritis.</p></div><div><h3>Methods</h3><p>Several databases were searched till July 2022. A total of 11 RCTs with at least one treatment arm were included. All articles were in English. The primary outcomes were ACR20, HAQ-DI, PASI90, and TEAEs experienced by the patients.</p></div><div><h3>Results</h3><p>Compared to other groups, Guselkumab had the strongest association with ACR20 response (RR: 2.14; 95% CI: 1.84-2.49, <em>p < 0.00001</em>), while a mean change in HAQ-DI (MD: -0.24; 95% CI: -0.41- -0.13, <em>p = 0.0001</em>) and PASI90 (RR: 9.81; 95% CI: 3.18-30.22; <em>p <0.01</em>) were most strongly associated with Ustekinumab. TEAEs were significantly present in the Guselkumab group (RR: 1.26; 95% CI: 1.00 - 1.59; <em>p = 0.05</em>).</p></div><div><h3>Conclusion</h3><p>Although our analysis suggests that IL-23 inhibitors are efficacious at treating psoriatic arthritis, further studies are required for long-term outcomes.</p></div>","PeriodicalId":100269,"journal":{"name":"Clinical Immunology Communications","volume":"4 ","pages":"Pages 7-22"},"PeriodicalIF":0.0,"publicationDate":"2023-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49753108","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-06-22DOI: 10.1016/j.clicom.2023.06.004
Jantien W. Wieringa , Mirjam J. Esser , Jan G.M.C. Damoiseaux , Gertjan A. Driessen , Joyce J.B.C. van Beers
The immunomodulating chemotherapeutic drugs rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) have the ablity to pass the placenta during pregnancy and might affect the development of the immune system of exposed infants. In particular rituximab causes a transient and almost complete depletion of CD20 expressing B cells and can remain detectable in the infant several months after birth.
In this case series we report on the clinical and immunological outcomes of 3 infants exposed to R-CHOP during pregnancy because of maternal B-cell lymphoma and review other cases that have been published. We show that R-CHOP in pregnancy has a profound effect on the immune system in the first year of life, including B-cell lymphopenia, hypogammaglobinemia, neutropenia and decreased response to immunization. Immune monitoring of exposed infants is warranted.
{"title":"B cell depletion in infants after intra uterine exposure to immunomodulating chemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP): A case series and review of the literature","authors":"Jantien W. Wieringa , Mirjam J. Esser , Jan G.M.C. Damoiseaux , Gertjan A. Driessen , Joyce J.B.C. van Beers","doi":"10.1016/j.clicom.2023.06.004","DOIUrl":"https://doi.org/10.1016/j.clicom.2023.06.004","url":null,"abstract":"<div><p>The immunomodulating chemotherapeutic drugs rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) have the ablity to pass the placenta during pregnancy and might affect the development of the immune system of exposed infants. In particular rituximab causes a transient and almost complete depletion of CD20 expressing B cells and can remain detectable in the infant several months after birth.</p><p>In this case series we report on the clinical and immunological outcomes of 3 infants exposed to R-CHOP during pregnancy because of maternal B-cell lymphoma and review other cases that have been published. We show that R-CHOP in pregnancy has a profound effect on the immune system in the first year of life, including B-cell lymphopenia, hypogammaglobinemia, neutropenia and decreased response to immunization. Immune monitoring of exposed infants is warranted.</p></div>","PeriodicalId":100269,"journal":{"name":"Clinical Immunology Communications","volume":"4 ","pages":"Pages 1-6"},"PeriodicalIF":0.0,"publicationDate":"2023-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49752922","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-06-03DOI: 10.1016/j.clicom.2023.06.001
Andi Wang , Amer Khojah , Gabrielle Morgan , Lauren M. Pachman
Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by chronic inflammation of the skin and muscle vasculature. Loss of nailfold capillary end row loops (ERL) is associated with disease activity. We now present the first report of serious gastrointestinal (GI) events in two JDM patients, both preceded by a precipitous drop in their ERL, which contributes to the consideration of JDM as a member of the vasculitis group of myopathies. These cases demonstrate that the sudden and significant loss of ERL density appears to be a novel and reliable indicator of severe systemic microangiopathy, including gastrointestinal vasculopathy, in selected children with JDM.
{"title":"Nailfold capillary dropout precedes the presentation of pneumatosis intestinalis and micro-perforation in juvenile dermatomyositis","authors":"Andi Wang , Amer Khojah , Gabrielle Morgan , Lauren M. Pachman","doi":"10.1016/j.clicom.2023.06.001","DOIUrl":"https://doi.org/10.1016/j.clicom.2023.06.001","url":null,"abstract":"<div><p>Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by chronic inflammation of the skin and muscle vasculature. Loss of nailfold capillary end row loops (ERL) is associated with disease activity. We now present the first report of serious gastrointestinal (GI) events in two JDM patients, both preceded by a precipitous drop in their ERL, which contributes to the consideration of JDM as a member of the vasculitis group of myopathies. These cases demonstrate that the sudden and significant loss of ERL density appears to be a novel and reliable indicator of severe systemic microangiopathy, including gastrointestinal vasculopathy, in selected children with JDM.</p></div>","PeriodicalId":100269,"journal":{"name":"Clinical Immunology Communications","volume":"3 ","pages":"Pages 74-76"},"PeriodicalIF":0.0,"publicationDate":"2023-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49767286","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-05-20DOI: 10.1016/j.clicom.2023.05.001
{"title":"Erratum regarding missing Informed Consent Statements and Conflict of Interest Statements in previously published articles","authors":"","doi":"10.1016/j.clicom.2023.05.001","DOIUrl":"https://doi.org/10.1016/j.clicom.2023.05.001","url":null,"abstract":"","PeriodicalId":100269,"journal":{"name":"Clinical Immunology Communications","volume":"3 ","pages":"Pages 59-60"},"PeriodicalIF":0.0,"publicationDate":"2023-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49767280","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
X-linked inhibitor of apoptosis (XIAP) deficiency is a primary immunodeficiency associated with recurrent hemophagocytic lymphohistiocytosis (HLH) episodes. The clinical phenotypes of XIAP deficiency vary, ranging from splenomegaly to life-threatening inflammation. We report a case of XIAP deficiency with unusual late-onset HLH presentation likely triggered by a drug allergy. A previously healthy adolescent boy presented to the hospital with fever and rash seven days after starting antibiotics for a neck abscess. Laboratory evaluation demonstrated cytopenias, elevated liver enzymes, and increased inflammatory markers. Initially, antibiotics were discontinued due to concern for drug rash. He continued to deteriorate clinically and became hypotensive. Additional testing revealed decreased NK cell function, as well as elevated ferritin, triglycerides, and soluble IL-2 receptor. SLAM-Associated Protein (SAP) and XIAP evaluation by flow cytometry demonstrated decreased XIAP expression. Subsequently, genetic testing revealed a known pathogenic mutation in BIRC4 (c.421_422del), confirming the diagnosis of XIAP deficiency.
{"title":"X-Linked Inhibitor of Apoptosis (XIAP) deficiency presenting as hemophagocytic lymphohistiocytosis triggered by drug allergy","authors":"Amer Khojah , Ameera Bukhari , Aaruni Khanolkar , Imad Khojah , Abigail Lang","doi":"10.1016/j.clicom.2023.02.008","DOIUrl":"https://doi.org/10.1016/j.clicom.2023.02.008","url":null,"abstract":"<div><p>X-linked inhibitor of apoptosis (XIAP) deficiency is a primary immunodeficiency associated with recurrent hemophagocytic lymphohistiocytosis (HLH) episodes. The clinical phenotypes of XIAP deficiency vary, ranging from splenomegaly to life-threatening inflammation. We report a case of XIAP deficiency with unusual late-onset HLH presentation likely triggered by a drug allergy. A previously healthy adolescent boy presented to the hospital with fever and rash seven days after starting antibiotics for a neck abscess. Laboratory evaluation demonstrated cytopenias, elevated liver enzymes, and increased inflammatory markers. Initially, antibiotics were discontinued due to concern for drug rash. He continued to deteriorate clinically and became hypotensive. Additional testing revealed decreased NK cell function, as well as elevated ferritin, triglycerides, and soluble IL-2 receptor. SLAM-Associated Protein (SAP) and XIAP evaluation by flow cytometry demonstrated decreased XIAP expression. Subsequently, genetic testing revealed a known pathogenic mutation in <em>BIRC4</em> (c.421_422del), confirming the diagnosis of XIAP deficiency.</p></div>","PeriodicalId":100269,"journal":{"name":"Clinical Immunology Communications","volume":"3 ","pages":"Pages 46-50"},"PeriodicalIF":0.0,"publicationDate":"2023-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49765146","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}