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BMJ Case Reports Jun 2024Extranodal involvement in diffuse large B-cell lymphoma (DLBCL) is defined as disease outside of the lymph nodes and occurs in up to one-third of patients, though...
Extranodal involvement in diffuse large B-cell lymphoma (DLBCL) is defined as disease outside of the lymph nodes and occurs in up to one-third of patients, though multiorgan extranodal involvement is rare. Here, we describe a case of a patient presenting with widely metastatic lesions, including involvement of the lung, parotid gland, breast, pancreas, femur and multiple soft tissue masses, with initial concern for primary breast malignancy. Breast pathology and imaging were consistent with triple-expressor, double-hit stage IV high-grade B-cell lymphoma with extensive extranodal involvement. Extranodal involvement is a poor prognostic factor associated with high rates of treatment failure, and novel therapies targeting CD19 are currently being studied for relapsed and refractory DLBCL. Extranodal disease is a complex entity that can involve virtually any organ system and should be considered for new presentations of malignancy.
Topics: Humans; Lymphoma, Large B-Cell, Diffuse; Female; Middle Aged; Breast Neoplasms; Lung Neoplasms; Parotid Neoplasms; Pancreatic Neoplasms; Antineoplastic Combined Chemotherapy Protocols; Fatal Outcome
PubMed: 38945554
DOI: 10.1136/bcr-2023-257416 -
Revista Clinica Espanola Jun 2024To describe the predictors of mortality in hospitalized patients with severe acute respiratory syndrome (SARS) due to COVID-19 presenting with silent hypoxemia.
OBJECTIVE
To describe the predictors of mortality in hospitalized patients with severe acute respiratory syndrome (SARS) due to COVID-19 presenting with silent hypoxemia.
MATERIAL AND METHODS
Retrospective cohort study of hospitalized patients with SARS due to COVID-19 and silent hypoxemia at admission, in Brazil, from January to June 2021. The primary outcome of interest was in-hospital death. Multivariable logistic regression analysis was performed.
RESULTS
Of 46,102 patients, the mean age was 59 ± 16 years, and 41.6% were female. During hospitalization, 13,149 patients died. Compared to survivors, non-survivors were older (mean age, 66 vs. 56 years; P < 0.001), less frequently female (43.6% vs. 40.9%; P < 0.001), and more likely to have comorbidities (74.3% vs. 56.8%; P < 0.001). Non-survivors had higher needs for invasive mechanical ventilation (42.4% vs 6.6%; P < 0.001) and intensive care unit admission (56.9% vs 20%; P < 0.001) compared to survivors. In the multivariable regression analysis, advanced age (OR 1.04; 95%CI 1.037-1.04), presence of comorbidities (OR 1.54; 95%CI 1.47-1.62), cough (OR 0.74; 95%CI 0.71-0.79), respiratory distress (OR 1.32; 95%CI 1.26-1.38), and need for non-invasive respiratory support (OR 0.37; 95%CI 0.35-0.40) remained independently associated with death.
CONCLUSIONS
Advanced age, presence of comorbidities, and respiratory distress were independent risk factors for mortality, while cough and requirement for non-invasive respiratory support were independent protective factors against mortality in hospitalized patients due to SARS due to COVID-19 with silent hypoxemia at presentation.
PubMed: 38945525
DOI: 10.1016/j.rceng.2024.06.010 -
Transplantation and Cellular Therapy Jun 2024Chimeric antigen receptor T-cell (CAR-T) therapy, including axicabtagene ciloleucel (axi-cel) and tisagenlecleucel (tisa-cel), has demonstrated significant efficacy in...
BACKGROUND
Chimeric antigen receptor T-cell (CAR-T) therapy, including axicabtagene ciloleucel (axi-cel) and tisagenlecleucel (tisa-cel), has demonstrated significant efficacy in treating refractory or relapsed diffuse large B-cell lymphoma and B-cell acute lymphoblastic leukemia. Though adverse events such as cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS) are well characterized, the dermatologic adverse event (DAE) profile is less thoroughly described.
OBJECTIVES
This study aims to provide the first comprehensive analysis of DAEs associated with axi-cel and tisa-cel using real-world data from the FDA Adverse Event Reporting System (FAERS) database.
STUDY DESIGN
FAERS database reports citing axi-cel or tisa-cel in patients aged 16 years or older were included, excluding duplicate reports and off-label indications. Disproportionality analysis by reporting odds ratio (ROR) was utilized to detect increased reporting of drug-adverse event combinations.
RESULTS
Of the 11,256,845 reports in the FAERS database, 5,559 identified CAR-T therapy as the primary suspected drug. After exclusions, 3,666 reports were analyzed (2,168 for axi-cel and 1,498 for tisa-cel). Among these, 2.7% of axi-cel and 5.1% of tisa-cel cases reported DAEs. There was a statistically significant increased reporting of 2 DAE groups associated with CAR-T therapy: severe cutaneous eruptions (ROR 5.18, 95% CI 1.29, 20.76) and vascular cutaneous (ROR 2.91, 95% CI 1.51, 5.60). The median time to DAE onset was 3 days after CAR T-cell infusion. Death was a reported outcome in 11.9% and 13.0% of axi-cel and tisa-cel DAE cases, respectively, and in 50% and 25% of severe cutaneous eruptions and vascular cutaneous cases, respectively.
CONCLUSIONS
This study reveals a significantly increased reporting rate of severe cutaneous eruptions and vascular cutaneous DAEs associated with CAR-T therapy, with both event groups associated with high mortality. These results emphasize the importance of monitoring dermatologic toxicities in clinical practice to ensure timely identification and management of potentially severe adverse events.
PubMed: 38945480
DOI: 10.1016/j.jtct.2024.06.024 -
Revista Portuguesa de Cardiologia :... Jun 2024Chronic thromboembolic pulmonary hypertension (CTEPH) is part of group 4 of the pulmonary hypertension (PH) classification and generally affects more than a third of... (Review)
Review
Chronic thromboembolic pulmonary hypertension (CTEPH) is part of group 4 of the pulmonary hypertension (PH) classification and generally affects more than a third of patients referred to PH centers. It is a three-compartment disease involving proximal (lobar-to-segmental) and distal (subsegmental) pulmonary arteries that are obstructed by persistent fibrothrombotic material, and precapillary pulmonary arteries that can be affected as in pulmonary arterial hypertension. It is a rare complication of pulmonary embolism (PE), with an incidence of around 3% in PE survivors. The observed incidence of CTEPH in the general population is around six cases per million but could be three times higher than this, as estimated from PE incidence. However, a previous venous thromboembolic episode is not always documented. With advances in multimodality imaging and therapeutic management, survival for CTEPH has improved for both operable and inoperable patients. Advanced imaging with pulmonary angiography helps distinguish proximal from distal obstructive disease. However, right heart catheterization is of utmost importance to establish the diagnosis and hemodynamic severity of PH. The therapeutic strategy relies on a stepwise approach, starting with an operability assessment. Pulmonary endarterectomy (PEA), also known as pulmonary thromboendarterectomy, is the first-line treatment for operable patients. Growing experience and advances in surgical technique have enabled expansion of the distal limits of PEA and significant improvements in perioperative and mid- to long-term mortality. In patients who are inoperable or who have persistent/recurrent PH after PEA, medical therapy and/or balloon pulmonary angioplasty (BPA) are effective treatment options with favorable outcomes that are increasingly used. All treatment decisions should be made with a multidisciplinary team that includes a PEA surgeon, a BPA expert, and a chest radiologist.
PubMed: 38945473
DOI: 10.1016/j.repc.2024.04.006 -
The Journal of Pediatrics Jun 2024To investigate the extent of extra-skeletal manifestations along with inpatient outcomes and complications associated with osteogenesis imperfecta (OI).
National Trends in Inpatient Hospital Outcomes of Children with Osteogenesis Imperfecta and the Importance of Extra-skeletal Manifestations: A Kids' Inpatient Database Study.
OBJECTIVE
To investigate the extent of extra-skeletal manifestations along with inpatient outcomes and complications associated with osteogenesis imperfecta (OI).
STUDY DESIGN
This cross-sectional study utilized the Kids' Inpatient Database (KID) as part of the Healthcare Cost and Utilization Project (HCUP) to investigate inpatient hospital outcomes and management in patients with OI from 1997 through 2016. Data regarding hospital characteristics, cost of treatment, inpatient outcomes, and procedures were collected and analyzed.
RESULTS
There were 7,291 admissions that listed OI as a diagnosis in the KID database from 1997 through 2016. Unexpectedly, over one third of all admissions in these children with OI presented with an extra-skeletal manifestation. The rate of major complications was 3.85%. The rate of minor complications was 19.4%, most commonly respiratory problems. Mortality rate was 18.2 % in the neonatal period and 1.0% in all other admissions. Total charges of hospital stay increased over the years.
CONCLUSION
We identified a striking prevalence of extra-skeletal manifestations in OI along with inpatient outcomes and complications associated with OI, of which respiratory complications were predominant. We observed a significant financial burden for patients with OI and identified additional risks for financial crisis, in addition to disparities in care identified among socioeconomic groups. These data contribute to a more holistic understanding of OI from diagnosis to management.
PubMed: 38945443
DOI: 10.1016/j.jpeds.2024.114174 -
International Journal of Cardiology Jun 2024Anatomic variations of the patent foramen ovale (PFO) are commonly observed, yet limited research has investigated their impact on clinical outcomes following...
BACKGROUND
Anatomic variations of the patent foramen ovale (PFO) are commonly observed, yet limited research has investigated their impact on clinical outcomes following transcatheter closure. We aimed to explore the association between PFO morphology and clinical outcomes.
METHODS
Consecutive patients with cryptogenic stroke who underwent PFO closure were prospectively enrolled at a single center from September 2019 to April 2023. Patients were categorized into simple and complex groups based on PFO morphology. Composite events were compared between the two groups during a median follow-up of 24 months, including all-cause mortality, recurrent stroke, residual moderate or severe shunt, and symptomatic atrial fibrillation.
RESULTS
A total of 247 patients were enrolled, with a mean age of 41.9 ± 13.0 years and 45.3% males. Ninety-one (36.8%) patients had complex PFO. These individuals were older (45.4 ± 12.5 years vs. 39.9 ± 12.9 years; P = 0.001), more males (56.0% vs. 39.1%; P = 0.010), had longer procedure times (54 ± 32 min vs 46 ± 29 min; P = 0.044), and had a higher rate of using delivery sheath-assisted crossing of the PFO (22.0% vs 12.8%; P = 0.040) than those with simple PFO. The estimated event rates were 27.9% and 11.3% (P = 0.006) in the complex and simple PFO groups, respectively (12.9 events and 5.2 events per 100 person-years; P = 0.001). After adjusting for age, sex, hypertension, diabetes, smoking, device type, and left atrial diameters, complex PFO remained independently associated with composite events (HR 2.10, 95%CI 1.06-4.17, P = 0.034).
CONCLUSIONS
Patients with complex PFO may suffer from a higher risk of adverse events following transcatheter PFO closure.
PubMed: 38945369
DOI: 10.1016/j.ijcard.2024.132307 -
The French Journal of Urology Jun 2024Compare scoring systems using Fournier gangrene severity index (FGSI), Uludag Fournier gangrene severity index (UFGSI), Laboratory Risk Indicator for Necrotizing...
INTRODUCTION
Compare scoring systems using Fournier gangrene severity index (FGSI), Uludag Fournier gangrene severity index (UFGSI), Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC), and neutrophil-lymphocyte ratio (NLR) to predict the outcome of patients with Fournier gangrene Materials and Methods: This is a retrospective cohort study that includes FG patients from 2012 to 2021. NLR, FGSI, UFGSI, and LRINEC values were calculated and analyzed. Each scoring system was analyzed using a receiver operating curve (ROC) analysis to determine its sensitivity, specificity, and area under the curve (AUC). Statistical analysis was performed using SPSS version 25.
RESULTS
A total of 158 patients were included in this study. Regarding the mortality outcome, FGSI comprised the highest value of AUC with 80.9, with a sensitivity of 91.7% and specificity of 68.5%. LRINEC comprised the AUC value of 61.1, with 79.2% sensitivity and 64.2% specificity. NLR comprised an AUC value of 63.7, 91.7% of sensitivity, and 98.1% of specificity. In terms of length of stay, LRINEC and NLR were associated as significant predictor.
CONCLUSION
FGSI, UFGSI, and NLR are significant predictors associated with mortality in patients with Fournier gangrene. FGSI and UFGSI comprised the highest sensitivity and specificity value in predicting mortality prognosis. Moreover, this study highlighted the role of NLR and LRINEC as significant predictors for the length of hospitalization. This study shows that FGSI is still a reliable scoring system for predicting mortality in patients with Fournier Gangrene.
PubMed: 38945366
DOI: 10.1016/j.fjurol.2024.102673 -
Journal of Vascular Surgery. Venous and... Jun 2024We evaluated the impact of completion intraoperative venography on clinical outcomes for axillosubclavian vein (AxSCV) thrombosis due to venous thoracic outlet syndrome...
INTRODUCTION
We evaluated the impact of completion intraoperative venography on clinical outcomes for axillosubclavian vein (AxSCV) thrombosis due to venous thoracic outlet syndrome (vTOS).
METHODS
We performed a retrospective, single-center review of all patients with vTOS treated with First Rib Resection and intraoperative venography from 2011 - 2023. We reviewed intraoperative venographic films to classify findings, collected demographics, clinical and perioperative variables, and clinical outcomes. Primary endpoints were symptomatic relief and primary patency at 3 months and 1 year. Secondary endpoints were time free from symptoms, reintervention rate, perioperative complications, and mortality.
RESULTS
Fifty-one AxSCVs (49 patients, mean age of 31.3 ± 12.6, 52.9% female) were treated for vTOS with first rib resection and external venolysis followed by completion intraoperative venography with a mean follow up of 15.5 ± 13.5 months. Prior to FRR, 32 underwent catheter-directed thrombolysis (62.7%). Completion intraoperative venography identified 16 patients with No Stenosis (Group 1, 31.3%), 17 with No Stenosis after Angioplasty (Group 2, 33.3%), 10 with Residual Stenosis after Angioplasty (Group 3, 19.7%), and 8 with Complete Occlusion (Group 4, 15.7%). The overall symptomatic relief was 44 of 51 (86.3%) and did not differ between venographic classifications (Group 1: 14 of 16, Group 2: 13 of 17, Group 3: 10 of 10, and Group 4: 7 of 8; Log-Rank Test, p = 0.5). The overall 3-month and 1-year primary patency was 42 of 43 (97.7%) and 32 of 33 (97.0%), respectively (Group 1: 16 of 16 and 9 of 9; Group 2: 16 of 17 and 12 of 13; Group 3: 10 of 10, 5 of 5; Group 4: primary patency not obtained). There was one asymptomatic re-thrombosis that resolved with anticoagulation, and three patients underwent reintervention with venous angioplasty for significant symptom recurrence an average 2.89 ± 1.7 months after FRR.
CONCLUSION
Our single-center retrospective study demonstrates that FRR with completion intraoperative venography has excellent symptomatic relief, short- and mid-term patency despite residual venous stenosis and complete occlusion. While completion intraoperative venographic classification did not correlate with adverse outcomes, this protocol yielded excellent results and provides important clinical data for postoperative management. Our results also support a conservative approach to AxSCV occlusion identified after FRR.
PubMed: 38945363
DOI: 10.1016/j.jvsv.2024.101936 -
The Journal of Thoracic and... Jun 2024Despite excellent outcomes of heart transplants from donors with hepatitis C (HCV D+), many candidates are not listed to even consider HCV D+ offers.
OBJECTIVE
Despite excellent outcomes of heart transplants from donors with hepatitis C (HCV D+), many candidates are not listed to even consider HCV D+ offers.
METHODS
Using the Scientific Registry of Transplant Recipients, we identified adult (≥18y) heart transplant candidates prevalent on the waitlist 2018-03/2023. We compared likelihood of waitlist mortality or heart transplant by candidate willingness to consider HCV D+ offers using competing risk regression.
RESULTS
We identified 19,415 heart transplant candidates, 68.9% of whom were willing to consider HCV D+ offers. Heart candidates willing to consider HCV D+ offers had 37% lower risk of waitlist mortality (SHR 0.63, 95%CI 0.56-0.70, p<0.001) than candidates not willing to consider HCV D+ offers, after adjustment for covariates and center-level clustering. Over the same period, heart transplant candidates willing to consider HCV D+ offers had 21% higher likelihood of receiving a transplant (SHR 1.21, 95%CI 1.7-1.26, p<0.001). As a result, among candidates willing to consider HCV D+ offers, 74.9% received a transplant and 6.1% died/deteriorated after three years, versus 68.3% and 9.1% of candidates not willing to consider HCV D+ offers. Lower waitlist mortality was also observed on subgroup analyses of candidates on temporary and durable mechanical circulatory support.
CONCLUSION
Willingness to consider HCV D+ heart offers was associated with a 37% lower risk of waitlist mortality and a 21% higher likelihood of receiving a transplant. We urge providers encourage candidates to list as willing to consider offers from donors with hepatitis C to optimize their waitlist outcomes and access to transplant.
PubMed: 38945356
DOI: 10.1016/j.jtcvs.2024.06.025 -
The American Journal of Cardiology Jun 2024Radial artery (RA) access has been increasingly utilized for coronary procedures due to lower rates of access-site complications and improved patient satisfaction....
Radial artery (RA) access has been increasingly utilized for coronary procedures due to lower rates of access-site complications and improved patient satisfaction. However, limited data are available for RA access for peripheral vascular intervention (PVI). We performed a retrospective review of 143 patients who underwent PVI via RA access from February 2020 to September 2022 at a single institution. Baseline characteristics and follow-up data were ascertained from a prospectively maintained institutional database. Of 491 PVI, 156 (31.8%) were performed through the RA. Anatomical location for intervention were the femoral (44.8%), iliac (31.1%), popliteal (9.6%) peroneal (2.7%), tibial (9.9%), and subclavian (1.9%) arteries. Procedural access was obtained through the right RA (92.9%), left RA (4.5%), or right ulnar artery (2.6%) using the 6 French R2P Destination Slender sheath in 85 cm, 105 cm, and 119 cm lengths. Atherectomy was used in 34.7%. Mean contrast volume was 105.5 mL and the average fluoroscopy time was 18.5 minutes. Conversion to femoral access occurred in 3 cases (1.9%) due to arterial spasm and non-crossable lesions. Concomitant pedal access occurred in 2 cases (1.3%). Periprocedural complication rate was 3.84%, of which access-site hematoma was most common (3.2%); none required blood transfusion, surgical intervention, or additional hospital stay. There was 1 case (0.64%) of in-hospital stroke. The mortality rate at 30-day, 6-month, and 1-year was 1.4%, 2.8%, and 4.2%, respectively. In conclusion, RA access is feasible for diverse PVI, and future studies are needed to assess safety and benefit compared to femoral artery access.
PubMed: 38945347
DOI: 10.1016/j.amjcard.2024.06.025