Citations

7049 Background: Langerhans cell histiocytosis (LCH) can manifest as single system (SS) disease, multisystem (MS) disease, or pulmonary LCH (smoking-related). There is a paucity of data on prognostic factors including risk organ (RO) involvement (liver, spleen, and bone marrow) in adult LCH, which we sought to address in this study. Methods: Single-center retrospective study of patients ≥18y diagnosed with LCH from 1998 to 2020. Univariate and multivariate analyses for progression free survival (PFS) and overall survival (OS) were conducted using age, sex, organ involvement, LCH subtype, year of diagnosis, BRAF V600E status, and treatments. Results: We included 219 patients with LCH; median age 43y (range 19-88), females 51%, SS unifocal (23%), SS multifocal (6%), pulmonary (31%) and MS (40%). Commonly involved organs included lung (53%), bone (42%), skin (24%), pituitary (16%), and CNS (12%). BRAF V600E was positive in 40/88 (46%). Median follow-up duration was 6.1y (95% CI, 5.1- 7.1). On univariate analysis, factors associated with worse PFS were bone LCH, RO involvement, multifocal/MS LCH, and radiation therapy alone; those with worse OS included RO involvement, MS disease, BRAF V600E+, and age ≥45y at diagnosis. In multivariate analysis, BRAF V600E and age ≥45y at diagnosis were associated with worse mortality (Table). Median PFS was not reached (NR-NR) for SS unifocal LCH, 5mo (0-12.7) for SS multifocal LCH, 110mo (84.7-135.3) for pulmonary LCH, and 27mo (17.2-36.8) for MS LCH. 5-year OS was 97.4% for SS unifocal LCH, 100% for SS multifocal LCH, 96.1% for pulmonary LCH, and 79.9% for MS LCH. 41 (18.7%) developed a second primary malignancy (SPM), of which 11 were hematologic neoplasms. There was a trend towards a higher prevalence of SPMs in patients with BRAF V600E (28% vs. 17%; p = 0.22). Conclusions: In our large single-center study, PFS for multifocal and MS LCH was worse than SS unifocal or pulmonary LCH. RO involvement was not associated with outcomes in multivariate analysis. Overall prognosis was excellent for all subtypes except MS LCH. BRAF V600E and older age were associated with worse OS. The prevalence of SPMs was very high and needs to be explored further.[Table: see text]

Background: Multimorbidity is common in patients with atrial fibrillation (AF), yet comorbidity patterns are not well documented. Methods: The prevalence of 18 chronic conditions was obtained in patients with AF from 2013-2017 from a 27-county region in the Midwest. All patients had AF documented on an electrocardiogram or Holter monitor. To compare comorbidity patterns between patients with and without AF, controls were matched 1:1 on age (± 5 years) and sex. For AF patients and controls separately, the Somers’ D statistic was used to quantify the non-random concordance of each pair of chronic conditions beyond chance (ranging from -1 [perfect disagreement] to 1 [perfect agreement]; 0 indicating coincidental agreement by chance alone). Significance of the Somers’ D statistic was evaluated using a permutation approach with the Benjamini-Hochberg procedure for controlling the false discovery rate at 0.1%. Results: Among the 16,543 patients with AF (median age 76 years, 57% men), the most common conditions were hypertension (70%), hyperlipidemia (61%), arthritis (41%), and diabetes (40%). Many pairs of chronic conditions co-occurred more frequently than expected. However, no material differences were observed in the co-occurrence of conditions in patients with AF compared to controls (data not shown). Among patients with AF, the co-occurrence of conditions differed by age (Figure). In those ≤65 years, the strongest co-occurrence of conditions was depression/anxiety, although several condition pairs had Somers’ D statistics of >0.4. In those aged 65-74 years, hypertension/hyperlipidemia and depression/anxiety had the strongest co-occurrence, whereas hypertension/hyperlipidemia was strongest for those aged ≥75 years. Conclusion: Although differences in the co-occurrence of conditions did not exist between AF and community controls, among the patients with AF, the patterns of co-existing conditions differed by age. A better understanding of the clinical consequences of multimorbidity in AF patients is needed.

Introduction: Cardiovascular disease remains the leading cause of morbidity and mortality in patients with amyloid light chain (AL) amyloidosis. Current prognostic evaluation in AL cardiomyopathy (AL-CM) largely relies upon disparate proxy measurements to assess mortality. With respect to observed myocardial volume changes between systole and diastole, we describe a single measurement derived from cardiac MRI (CMR) to assess mortality of AL-CM. Methods: A cohort of 129 consecutive patients with AL-CM undergoing CMR with amyloid protocol from the years 2001 - 1997 was reviewed. Myocardial volume change at end systole and end diastole was assessed by disc summation with CMR. The myocardial function index (MFI) was calculated by consolidating blood pool and myocardial volume measures as confluent components of myocardial function utilizing operator tracings. The association of MFI and death was assessed with adjusted hazard ratios (HR) derived by a Cox model. Results: Data at time of CMR was reviewed. The mean age was 60 ±10; 68% subjects were female. Median survival from CMR was 47 months. Mean LVEF was assessed by echocardiography to be 55% ± 12. Mean calculated MFI was 19.8% ±7. Univariate analysis suggested MFI is strongly associated with death (HR 0.004; 95%CI 0.00002-0.0838; p = 0.0003). A multivariate analysis re-demonstrated the strong predictive value of MFI when controlled for age (HR 0.006; 95%CI 0.00003 - 0.1156; p = 0.0006). Conclusion: MFI strongly correlates with mortality in AL-CM. In contrast to previously described assessments of ventricular function (extracellular volume imaging, myocardial perfusion imaging, and myocardial contraction fraction), MFI is easily derived from CMR and considers blood pool and myocardial volumes as confluent components of myocardial function. Further studies are needed to assess MFI amongst patients with varying etiologies of heart failure.

Chylothorax has rarely been reported as a pleuropulmonary complication of Waldenström macroglobulinaemia (WM). In general, when a unilateral effusion is discovered particularly in patients with a history of cancer or active malignancy, a broad differential including chylothorax needs to be considered. We present the case of a 50-year-old woman found to have chylothorax secondary to progression of WM as confirmed by cytology and presence of MYD88 L265P mutation in the pleural fluid specimen and subsequent resolution with chemotherapy. This review centres particularly on non-traumatic causes of chylothorax with a focus on WM and includes a unique patient perspective.

Abstract Hospital census prediction has well-described implications for efficient hospital resource utilization, and recent issues with hospital crowding due to CoVID-19 have emphasized the importance of this task. Our team has been leading an institutional effort to develop machine-learning models that can predict hospital census 12 hours into the future. We describe our efforts at developing accurate empirical models for this task. Ultimately, with limited resources and time, we were able to develop simple yet useful models for 12-hour census prediction and design a dashboard application to display this output to our hospital’s decision-makers. Specifically, we found that linear models with ElasticNet regularization performed well for this task with relative 95% error of +/− 3.4% and that this work could be completed in approximately 7 months.

Abstract ObjectiveThe objective of this study was to identify the predictors of in-hospital mortality among patients with diffuse alveolar hemorrhage (DAH).Patients and MethodsWe conducted a retrospective review of 89 patients hospitalized for DAH at our institution between 2001 and 2017: 49 patients who died during hospitalization and 40 patients who survived were compared. We reviewed their presenting signs and symptoms, clinical course, radiologic and pathologic findings, along with medical management. We then performed univariate and multivariate analyses to identify the risk factors associated with in-hospital mortality.ResultsWe identified 12 factors to be associated with mortality when comparing survivor vs non-survivor cohorts: smoking (27 [67%] vs 21 [42%], p = 0.02), malignancy (7 [17%] vs 24 [49%], p = 0.002), interstitial lung disease (0 vs 7 [14%], p = 0.01), liver failure (1 [2%] vs 14 [28%], p = 0.001), autoimmune diseases (16 [40%] vs 4 [8%], p =0.0006), thrombocytopenia (3 [7%] vs 35 [71%], p <0.0001), ICU admission (23 [57%] vs 40 [85%], p=0.004), mean duration of ICU stay (3.5 days [± 6.7] vs 5.5 days [± 5.5], p = 0.4), steroid use (36 [90%] vs 31 [63%], p = 0.003), use of plasma exchange (6 [15%] vs 0, p = 0.005), use of mechanical ventilation (15 [37%] vs 36 [75%], p value = 0.0007) and development of acute respiratory distress syndrome (ARDS) (9 [22%] vs 37 [77%], p <0.0001), respectively. On multivariate analysis, thrombocytopenia (OR 52.08: 95% CI, 8.59-315.71; p <0.0001) and ARDS (OR 11.71: 95% CI, 2.60-52.67; p = 0.0013) were associated with higher odds of mortality in DAH while steroid use (OR 0.05: 95% CI, 0.007-0.39; p = 0.0004) was associated with a lower risk of in-hospital mortality in patients with DAH.ConclusionIn DAH, thrombocytopenia and ARDS were predictors of in-hospital mortality whereas the use of steroid was associated with a more favorable prognosis.