T-Cell Acute Lymphoblastic Leukemia/Lymphoma (T-ALL) With Negative Screening Immaturity Markers and Gamma-Delta Receptor Expression.

T-cell acute lymphoblastic leukemia/lymphoma (T-ALL) is characterized by the combination of T-cell lineage and the presence of immaturity marker(s). Sometimes, the most common immaturity markers for initial flow cytometry screening in T-ALL may be negative, which can be a diagnostic pitfall. When a lack of common first-line immaturity markers is encountered in combination with gamma/delta T-cell receptor expression, a misdiagnosis of mature gamma-delta T-cell leukemia/lymphoma could be rendered. Here, we discuss two T-ALL cases with the absence of common flow cytometry immaturity markers and positive gamma/delta receptor expression.

Role of Machine Learning and Artificial Intelligence in Arrhythmias and Electrophysiology.

Machine learning (ML), a subset of artificial intelligence (AI) centered on machines learning from extensive datasets, stands at the forefront of a technological revolution shaping various facets of society. Cardiovascular medicine has emerged as a key domain for ML applications, with considerable efforts to integrate these innovations into routine clinical practice. Within cardiac electrophysiology, ML applications, especially in the automated interpretation of electrocardiograms, have garnered substantial attention in existing literature. However, less recognized are the diverse applications of ML in cardiac electrophysiology and arrhythmias, spanning basic science research on arrhythmia mechanisms, both experimental and computational, as well as contributions to enhanced techniques for mapping cardiac electrical function and translational research related to arrhythmia management. This comprehensive review delves into various ML applications within the scope of this journal, organized into 3 parts. The first section provides a fundamental understanding of general ML principles and methodologies, serving as a foundational resource for readers interested in exploring ML applications in arrhythmia research. The second part offers an in-depth review of studies in arrhythmia and electrophysiology that leverage ML methodologies, showcasing the broad potential of ML approaches. Each subject is thoroughly outlined, accompanied by a review of notable ML research advancements. Finally, the review delves into the primary challenges and future perspectives surrounding ML-driven cardiac electrophysiology and arrhythmias research.

Cardiovascular risk assessment in inflammatory bowel disease with coronary calcium score.

The interplay between inflammatory bowel disease (IBD) and atherosclerotic cardiovascular disease (ASCVD) underscores the intricate connections between chronic inflammation and cardiovascular health. This review explores the multifaceted relationship between these conditions, highlighting the emerging significance of the coronary calcium score as a pivotal tool in risk assessment and management. Chronic inflammation, a hallmark of IBD, has far-reaching systemic effects that extend to the cardiovascular system. Shared risk factors and mechanisms, such as endothelial dysfunction, lipid dysfunction, and microbiome dysregulation, contribute to the elevated ASCVD risk observed in individuals with IBD. Amidst this landscape, the coronary calcium score emerges as a means to quantify calcified plaque within coronary arteries, offering insights into atherosclerotic burden and potential risk stratification. The integration of the coronary calcium score refines cardiovascular risk assessment, enabling tailored preventive strategies for individuals with IBD. By identifying those at elevated risk, healthcare providers can guide interventions, fostering informed shared decision-making. Research gaps persist, prompting further investigation into mechanisms linking IBD and ASCVD, particularly in the context of intermediate mechanisms and early atherosclerotic changes. The potential of the coronary calcium score extends beyond risk assessment-it holds promise for targeted interventions. Randomized trials exploring the impact of IBD-modifying therapies on ASCVD risk reduction can revolutionize preventive strategies. As precision medicine gains prominence, the coronary calcium score becomes a beacon of insight, illuminating the path toward personalized cardiovascular care for individuals living with IBD. Through interdisciplinary collaboration and rigorous research, we embark on a journey to transform the paradigm of preventive medicine and enhance the well-being of this patient population.

Advancements in gene therapy approaches for atrial fibrillation: Targeted delivery, mechanistic insights and future prospects.

Atrial fibrillation (AF) remains a complex and challenging arrhythmia to treat, necessitating innovative therapeutic strategies. This review explores the evolving landscape of gene therapy for AF, focusing on targeted delivery methods, mechanistic insights, and future prospects. Direct myocardial injection, reversible electroporation, and gene painting techniques are discussed as effective means of delivering therapeutic genes, emphasizing their potential to modulate both structural and electrical aspects of the AF substrate. The importance of identifying precise targets for gene therapy, particularly in the context of AF-associated genetic, structural, and electrical abnormalities, is highlighted. Current studies employing animal models, such as mice and large animals, provide valuable insights into the efficacy and limitations of gene therapy approaches. The significance of imaging methods for detecting atrial fibrosis and guiding targeted gene delivery is underscored. Activation mapping techniques offer a nuanced understanding of AF-specific mechanisms, enabling tailored gene therapy interventions. Future prospects include the integration of advanced imaging, activation mapping, and percutaneous catheter-based techniques to refine transendocardial gene delivery, with potential applications in both ventricular and atrial contexts. As gene therapy for AF progresses, bridging the translational gap between preclinical models and clinical applications is imperative for the successful implementation of these promising approaches.

Postcardiac Injury Syndrome After Cardiac Surgery: An Evidence-Based Review.

Postcardiac injury syndrome (PCIS) serves as a comprehensive term encompassing a spectrum of conditions, namely postpericardiotomy syndrome, postmyocardial infarction (MI) related pericarditis (Dressler syndrome), and post-traumatic pericarditis stemming from procedures like percutaneous coronary intervention or cardiac implantable electronic device placement. These conditions collectively give rise to PCIS, triggered by cardiac injury affecting pericardial or pleural mesothelial cells, leading to subsequent inflammation syndromes spanning from uncomplicated pericarditis to substantial pleural effusion. A thorough literature search conducted on MEDLINE/PubMed utilizing search terms including "postacute cardiac injury syndrome," "postcardiac injury syndrome," "postcardiotomy syndrome," "postpericardiotomy syndrome," and "post-MI pericarditis" was instrumental in collating pertinent studies. To encapsulate the amassed evidence, relevant full-text materials were meticulously selected and amalgamated narratively. The pathophysiology of PCIS is proposed to manifest through an autoimmune-mediated process, particularly in predisposed individuals. This process involves the development of anti-actin and antimyosin antibodies after a cascade of cardiac injuries in diverse forms. Treatment strategies aimed at preventing recurrent PCIS episodes have shown efficacy, with colchicine and nonsteroidal anti-inflammatory drugs, including ibuprofen, demonstrating positive outcomes. Conversely, corticosteroids have exhibited no discernible benefit concerning prognosis or recurrence rates for this ailment. In summary, PCIS serves as a unifying term encompassing a spectrum of cardiac injury-related syndromes. A comprehensive review of relevant literature underscores the autoimmune-mediated pathophysiology in susceptible individuals. The therapeutic landscape involves the proficient use of colchicine and Nonsteroidal anti-inflammatory drugs to deter recurrent PCIS episodes, while corticosteroids do not appear to contribute to improved prognosis or reduced recurrence rates. This nuanced understanding contributes to an enhanced comprehension of PCIS and its multifaceted clinical manifestations, potentially refining its diagnosis and management.

Physical Activity in Pediatric Inflammatory Bowel Disease: A Scoping Review.

BACKGROUND: Inflammatory bowel disease (IBD) is a chronic, systemic condition affecting the gastrointestinal tract. IBD can be severe and are associated with impairment in growth, school absences, abdominal pain, and fatigue. Physical activity (PA) could have an anti-inflammatory effect in addition to other benefits. It is important to address the possible risks, physiological effects of PA, and potential barriers, and facilitators for PA participation in pediatric IBD. However, potential barriers and facilitators to PA have yet to be adequately described. METHODS: We conducted a scoping review to map and describe the current literature on PA in pediatric IBD populations between 1980 and April 2022 using Preferred Reporting Items for Systematic Reviews and Meta-analysis guidelines for Scoping reviews. RESULTS: Nineteen articles were identified including 10 descriptive, 6 interventional, and 3 physiological responses to PA studies. Patients and healthy controls demonstrated similar responses to exercise. Barriers to participation were low self-esteem, body image, and active IBD symptoms. Facilitators included personal interest, activity with friends, and support from family. CONCLUSION: This review highlighted that PA participation may reduce in children with IBD-related symptoms. Short- and medium-term impacts of PA on immune modulation require further study; it is possible that regular PA does not negatively affect biomarkers of disease activity.

Safety and Efficacy of Ferric Carboxymaltose in Heart Failure With Preserved Ejection Fraction and Iron Deficiency.

Heart Failure with Preserved Ejection Fraction (HFpEF) is a prevalent cardiovascular condition characterized by a complex pathophysiology and limited therapeutic options. Coinciding iron deficiency often compounds the clinical picture, contributing to symptom burden and adverse outcomes. The review underscores the urgency for effective treatments in light of its increasing incidence and considerable healthcare burden. It highlights the clinical significance of addressing iron deficiency in HFpEF patients. FCM emerges as a promising therapeutic modality, demonstrating the ability to rapidly restore iron stores and enhance patients' quality of life while reducing hospitalization rates and mortality. The review thoroughly elucidates the impact of iron deficiency on HFpEF symptoms and outcomes, elucidating how FCM effectively mitigates these challenges. Detailed discussions encompass FCM's mechanism of action, pharmacokinetics, and safety profile. Notably, FCM's adaptability to diverse patient profiles and clinical settings is emphasized, reinforcing its clinical utility. Clinical evidence, including study designs, patient cohorts, and key findings, affirms FCM's potential as a valuable therapeutic option. Real-world data analysis further underscores FCM's practicality and safety beyond controlled clinical trials. The review concludes by addressing future research directions and critical research gaps, accentuating the need for mechanistic insights, long-term outcome studies, and refined patient selection criteria. As FCM increasingly integrates into clinical practice, it offers promise in revolutionizing HFpEF management, addressing an unmet need in this intricate cardiovascular condition.

Commotio cordis in non-sports-related injury: A scoping review.

Commotio cordis is a rare but life-threatening condition characterized by sudden cardiac arrest resulting from a blunt chest impact. While commotio cordis has traditionally been associated with sports-related activities, a significant proportion of cases occur in non-sport-related settings, such as assaults, motor vehicle accidents (MVAs), and daily activities. This critical review examines the epidemiology, clinical characteristics, and outcomes of non-sports-related commotio cordis cases, highlighting the need for increased awareness and improved management in these contexts. The review analyzes existing literature, drawing attention to the demographics of non-sports-related cases, which predominantly affect adolescents and young adults, with males being the primary demographic. In contrast to sport-related cases, non-sports-related commotio cordis cases exhibit a wider age range and a higher proportion of female subjects. Mortality rates are significantly higher in non-sports-related commotio cordis cases, largely due to lower rates of cardiopulmonary resuscitation (CPR), limited access to automated external defibrillators (AEDs), and delayed initiation of resuscitative efforts compared to sport-related incidents. This underscores the critical importance of increasing awareness and preparedness in non-sport-related settings. To mitigate the risks associated with non-sports-related commotio cordis, efforts should focus on early recognition of the condition, timely administration of CPR, and the widespread availability and accessibility of AEDs in various environments. Enhanced awareness and education can potentially lead to a reduction in mortality and improved outcomes for individuals affected by commotio cordis outside of sports-related activities. In conclusion, commotio cordis is not exclusive to sports and presents a significant health risk in non-sport-related scenarios. This review emphasizes the urgent need for increased awareness, preparedness, and resuscitation measures in non-sports contexts to address the higher mortality associated with these cases.

Association of Cardiovascular Diseases With Post-Traumatic Stress Disorder: An Updated Review.

The intricate relationship between post-traumatic stress disorder (PTSD) and cardiovascular disease (CVD) has garnered increasing attention due to its bidirectional impact and potential for significant health consequences. Epidemiological evidence suggests that PTSD may serve as a risk factor for incident CVD, while acute CVD events can trigger PTSD, subsequently increasing the risk of recurrent cardiovascular events. This dynamic interplay is characterized by the human stress response, disrupted behavioral and lifestyle factors, and potential physiological mechanisms. Notably, the immediate aftermath of a cardiovascular event presents a critical window for intervention, offering the possibility of preventing the development of PTSD and its associated physiological and behavioral sequelae. However, while candidate mechanisms linking PTSD and CVD have been identified, determining which mechanisms are most amenable to intervention remains a challenge. This article emphasizes the urgency of addressing key unanswered questions in this domain. Despite an evolving understanding of the association between PTSD and CVD, causal relationships remain to be firmly established. Comprehensive investigations into the intricate interplay of behavioral and biological mechanisms are essential for identifying precise targets for intervention. Innovations in research methodologies, including the exploration of PTSD symptom dynamics and their impact on cardiovascular function, hold the potential for identifying crucial intervention points. Drawing parallels from prior challenges in translating identified risk factors into effective interventions, the field must prioritize systematic investigations and early-phase intervention trials. By doing so, researchers and clinicians can potentially develop strategies to mitigate CVD risk in the context of PTSD and improve both cardiovascular and mental health outcomes.

Outcomes of primary leadless pacemaker implantation: A systematic review.

BACKGROUND: During the last decade, leadless pacemakers (LPMs) have turned into a prevalent alternative to traditional transvenous (TV) pacemakers; however, there is no consolidated data on LPM implantation in emergencies. METHODS: Digital databases were searched for this review and four relevant studies, including 1276 patients were included in this review with procedure duration, fluoroscopic time, major complications, and mortality as primary outcomes and pacing threshold, impedance, sensing of LPM, and hospital stay as secondary outcomes. RESULTS: Gonzales et al. and Marschall et al. showed the duration of the procedure to be 180 ± 45 versus 324.6 ± 92 and 39.9 ± 8.7 versus 54.9 ± 9.8, respectively. Zhang et al. demonstrated the duration of the procedure and fluoroscopy time to be 36 ± 13.4 and 11.1 ± 3.1, respectively. Similarly, Schiavone et al. exhibited intermediate times of implantation at 60 (45-80) versus 50 (40-65) and fluoroscopic times at 6.5 (5-9.7) versus 5.1 (3.1-9). Hospital stay was more with a temp-perm pacemaker as compared to LPM and pacing parameters were not significantly different in all the studies. CONCLUSION: For underlying arrhythmias, whenever appropriate, our review shows that LPMs may be a better option than temporary pacemakers, even as an urgent treatment.

Predicting stroke and mortality in mitral stenosis with atrial flutter: A machine learning approach.

BACKGROUND: Our study hypothesized that an intelligent gradient boosting machine (GBM) model can predict cerebrovascular events and all-cause mortality in mitral stenosis (MS) with atrial flutter (AFL) by recognizing comorbidities, electrocardiographic and echocardiographic parameters. METHODS: The machine learning model was used as a statistical analyzer in recognizing the key risk factors and high-risk features with either outcome of cerebrovascular events or mortality. RESULTS: A total of 2184 patients with their chart data and imaging studies were included and the GBM analysis demonstrated mitral valve area (MVA), right ventricular systolic pressure, pulmonary artery pressure (PAP), left ventricular ejection fraction (LVEF), New York Heart Association (NYHA) class, and surgery as the most significant predictors of transient ischemic attack (TIA/stroke). MVA, PAP, LVEF, creatinine, hemoglobin, and diastolic blood pressure were predictors for all-cause mortality. CONCLUSION: The GBM model assimilates clinical data from all diagnostic modalities and significantly improves risk prediction performance and identification of key variables for the outcome of MS with AFL.

Familial Association of Granulomatosis With Polyangiitis: A Case-Based Review of Literature.

Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a class of small vessel vasculitis that includes granulomatosis with polyangiitis (GPA), eosinophilic GPA (EGPA), and microscopic polyangiitis (MPA). Despite extensive research, the mechanisms behind AAV etiology remain obscure. The genetics of AAV is a complex area of investigation because of the rarity of familial cases. However, recent multi-center genome-wide association studies (GWAS) have greatly contributed to our understanding of the genetic basis of AAV. In this study, we report a rare occurrence of GPA in two Caucasian family members who presented with similar clinical symptoms and performed a comprehensive review to study the present literature available regarding the heritability of this disease.

Oropharyngeal Secondary Syphilis Mimicking Metastatic Lymphoma in an HIV-Positive Patient.

Syphilis is a bacterial infection commonly transmitted by sexual contact. It has variable manifestations and can mimic other disease processes or infections. This report presents the case of a 48-year-old HIV-positive male who was referred to our head and neck clinic with complaints of tonsillar hypertrophy and ulceration accompanied by a one-month history of ipsilateral cervical lymphadenopathy and facial pain in the setting of recent unexplained weight loss and abnormal radiographic imaging of the neck. In-office tonsillar biopsy and fine-needle aspiration of a neck mass revealed a non-diagnostic atypical lymphoid proliferation. Surgical pathology following an open biopsy in the operating room showed Treponema pallidum infection, which was diagnostic for secondary syphilis.

Human monkeypox virus: An updated review.

The human monkeypox is an emerging zoonotic orthopoxvirus with a clinical presentation similar to that of smallpox. It is difficult to differentiate monkeypox from other orthopedic infections, and laboratory diagnosis is the primary component of disease identification and monitoring. However, current diagnostics are time-consuming, and new tests are needed for rapid and precise diagnosis. Most cases have been reported in Central Africa; however, an increasing number of cases have been reported in Europe, the United States of America (USA), Australia, and the United Arab Emirates. Although investigation of the current global outbreak is still ongoing, viral transmission seems to have occurred during crowded events in Spain and Belgium. New therapeutics and vaccines are being deployed for the treatment and prevention of monkeypox, and more research on the epidemiology, biology, and ecology of the virus in endemic areas is required to understand and prevent further global outbreaks.

Different Shapes Of Megakaryocytes In Essential Thrombocythemia.

Essential thrombocytopenia is the myeloproliferative neoplasm associated with the JAK2/CALR/MPL mutation. It is characterized by an increase in thrombocytes and abnormal megakaryocytes. WHO established the diagnostic criteria for diagnosing the myeloproliferative disorder, which is the combination of molecular, clinical, and histological findings. The appearance of megakaryocytes on bone marrow biopsy is the distinguishing feature to identify myeloproliferative neoplasm, and this short review would like to emphasize the presentation of megakaryocytes in bone marrow biopsy.

Marsupialization of a Floor-of-Mouth Dermoid Cyst to Temporize Airway Distress.

Dermoid cysts are benign masses of embryologic origin that can present in various anatomical locations throughout the human body. This article presents the case of a 30-year-old male who presented to our emergency department with complaints of tongue swelling accompanied by worsening dysphagia and dysphonia in the context of a chronic, midline mass in the floor of the mouth. Computed tomography (CT) imaging and surgical pathology of the mass ultimately revealed findings consistent with a dermoid cyst causing inferior displacement of the mylohyoid muscle. Initial management consisted of bedside drainage to temporize the airway, with marsupialization and in-office follow-up. Definitive treatment was achieved with surgical excision at a later date.

Reactive Lymphadenopathies.

Lymphadenitis in the pediatric population frequently is benign and self-limited, often caused by infections. In children with refractory symptoms, lymph node biopsy may be indicated to rule out malignancy or obtain material for culture. Acute bacterial infections typically show a suppurative pattern of necrosis with abscess formation. Viral infections are associated with nonspecific follicular and/or paracortical hyperplasia. Granulomatous inflammation is associated with bacterial, mycobacterial, and fungal infections. Toxoplasma lymphadenitis displays follicular hyperplasia, monocytoid B-cell hyperplasia, and clusters of epithelioid histiocytes. Autoimmune and noninfectious inflammatory disorders are included in differential diagnosis of lymphadenitis. Infectious mononucleosis and Kikuchi-Fujimoto lymphadenitis may mimic Hodgkin and non-Hodgkin lymphomas.

Precursor Lymphoblastic Lymphoma in the Extramedullary Tissue: A Rare Manifestation of Chronic Myeloid Leukemia in Blast Crisis.

Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by immature granulocytes in peripheral blood and bone marrow. In 95% of cases, it is always due to the presence of Philadelphia chromosome characterized by the presence of reciprocal translocation between chromosome 9 and 22. However, in 7% -17% of individuals, extramedullary proliferation also occurs, either in skin, lymph nodes, bone or central nervous system (CNS), which could be either myeloid, lymphoid or mixed progenitor in origin. The present case is of a 23-year-old male who presented with lower limb weakness, bowel and urinary incontinence. His complete blood count (CBC) findings showed a raised white blood count (WBC) of 408 X 10E9/L. Peripheral film, bone marrow biopsy and immunohistochemistry showed findings consistent with CML in chronic phase. Bone marrow cytogenetic revealed the presence of Philadelphia chromosome. Simultaneously, magnetic resonance imaging (MRI) was done which revealed extradural mass at L1-L3 level; histopathological and immunohistochemistry findings showed features compatible with precursor B cell lymphoblastic lymphoma. His cerebrospinal fluid (CSF) cytology revealed similar blast cells. This extramedullary presence of lymphoid blast cells in the CNS put the patient in the rare entity of CML in blast crisis. He was started on tablet nilotinib and also received multiple cycles of intrathecal chemotherapy with cytosar, methotrexate and hydrocortisone. He also underwent radiotherapy of extradural mass. His lower limb weakness improved dramatically. However, after receiving the fourth cycle of intrathecal therapy, the patient died consequent to neutropenic sepsis. Extramedullary blast crisis in CML has a poor prognosis. Any patient with CML, presenting with CNS symptoms or lymph node enlargement should be thoroughly investigated for extramedullary blast crisis, as there is a considerable change in management and prognosis from the prototype CML in chronic phase.