Hemophilia is an X-linked recessive hereditary disorder that classically affects males due to the presence of only one X chromosome in males. Females are usually carriers due to the presence of counterpart X chromosome, but many times manifestations of hemophilia are seen in heterozygous carrier females. This is a result of skewed lionization, in which more normal X chromosomes are converted to bar body, and more abnormal chromosomes remain active in body cells, causing the dominant manifestation of the disease. The severity of manifestations is directly proportional to the level of the clotting factor in the blood. The disease can be severe enough to cause life-threatening bleeding, especially during delivery. Physicians usually reluctant to assume hemophilia in the differential diagnosis of the bleeding disorders in women but manifesting carrier females with hemophilia are not uncommon. Our review of the literature will give an opportunity to understand this issue more precisely as well as will discuss the disease manifestations and its updated management.

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Checkpoint inhibitors (CPI) have become mainstream in standard therapy in various tumors, especially in malignant melanoma. Despite their widespread beneficial effects, these inhibitors are also notorious for immune-related adverse events (irAEs). Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation. We report a case of a 33-year-old male having a history of metastatic melanoma on immunotherapy (status post two cycles of ipilimumab/nivolumab) admitted for persistent fever and elevated liver enzymes. Additional work showed anemia, thrombocytopenia, hypertriglyceridemia, and hyperferritinemia which meet the diagnostic criteria of histiocyte society HLH-2004. The patient was effectively treated with oral prednisone. Moreover, further complications encompassed slurred speech, word-finding difficulties, ataxia, and lower extremity hyperreflexia concerning for autoimmune encephalitis. He was treated with high-dose IV methylprednisolone (1 gram/day for 3 days) with improvement in symptoms. Autoimmune encephalitis associated with HLH can be fatal -high-dose IV methylprednisolone should be considered, but this avenue still needs to be explored.

BackgroundEndoscopy is the cornerstone for the diagnosis and treatment of nonvariceal upper gastrointestinal bleeding. Regarding the management of nonvariceal bleeding, the administration of crystalloid solution and proton pump inhibitors before endoscopy is well established, but the optimal timing of endoscopy has been a matter of debate and a subject of many investigational studies. The need for urgent endoscopy arises to provide prompt redress to acute bleeding, decrease the length of stay, and lower mortality from ongoing bleeding. ObjectiveThis study aimed to determine if endoscopy performed within 24 hours of presentation improves outcomes in terms of mortality, hospital length of stay, and rebleeding in individuals presenting with nonvariceal upper gastrointestinal bleed with any risk. MethodologyWe performed a systematic review of two large databases (PubMed and Google Scholar) to incorporate all studies published after 2000. We included studies with nonvariceal upper gastrointestinal bleeding and excluded those reporting variceal gastrointestinal hemorrhage. ResultsWe reviewed eight studies that qualified after meeting our inclusion and exclusion criteria. We divided these studies into three separate groups based on the timing of endoscopy. Only two studies found a difference in mortality that was statistically significant in patients who underwent endoscopy within 24 hours of presentation. One study showed lower mortality in a patient who underwent urgent endoscopy, but it did not reach statistical significance. Other studies did not show any statistical difference in mortality, hospital length of stay, and rebleeding rates. The studies showed conflicting evidence on the amount of blood transfusion, though urgent endoscopy was found to be difficult in few studies due to blood obscuring the lesion. ConclusionsWhile data suggest that there is a potential benefit in performing endoscopy sooner, there is no concrete evidence to point to a particular time range. Before performing endoscopy, the American Society for Gastrointestinal Endoscopy (2012) recommends adequate resuscitation with crystalloid solutions, blood transfusions, and antisecretory and prokinetic agent therapy. More investigational studies are needed to formulate a time-sensitive flow sheet to approach endoscopy in patients with nonvariceal upper gastrointestinal bleeding. A strict criterion is also needed to delineate patients into low-risk and high-risk groups. Doing so would provide a systematic approach to help with mortality, rebleeding, and healthcare resource utilization.

e19524 Background: CLL accounts for about one-quarter of the new cases of leukemia and affects mainly elderly population. Recent literature highlights increased cardiovascular events in patients with CLL, notably those being treated with targeted therapy such as Bruton tyrosine kinase inhibitors. Here we examine data obtained from the National Inpatient Sample to find out incidence, predictors and outcomes of cardiovascular events in patients with CLL. Methods: We analyzed the National inpatient database from year 2019 and extracted the data of all patients with CLL. Multivariate regression analysis was performed to determine the aforementioned outcomes in CLL patients. Results: CLL patients showed increased odds of all cause in-hospital mortality (Adjusted Odds ratio (AOR) 1.28, 95% CI 1.19-1.38, P<0.001) in comparison to general adult population. CLL patients had higher odds of developing Atrial fibrillation (AOR 1.11, 95% CI 1.06-1.15 P <0.001) compared to patients without CLL. CLL patients had significantly higher odds of cardiac tamponade and pericardial effusion (AOR 1.64, 95% CI 1.10-2.42, P<0.001, 1.65 95% CI 1.43-1.92, P<0.001) respectively. Odds of congestive heart failure (AOR 0.97, 95% CI 0.93-1.01, P0.10) and atrial flutter (AOR 1.03, 95% CI 0.93-1.17, P 0.55) were similar between CLL patients and general adult populations. Patients with CLL have Increased mean hospital length of stay (adjusted mean LOS 0.51, 95% CI 0.42-0.61, p<0.001) and mean total hospital charges (adjusted mean charges 2006$, 95% CI 173$-3840$, P 0.03). [Table] We investigated the predictors of inpatient mortality in CLL patients and found that increase in age, black race, acute kidney injury, alcohol use, malnutrition and weekend admissions were associated with higher odds of mortality, whereas smoking, obesity, and female gender showed lower odds of mortality. Conclusions: CLL patients are at increased risk of developing atrial fibrillation, pericardial effusion, pericardial tamponade and all-cause in hospital mortality compared to their non-CLL counterparts, even after accounting for variables such as age, gender etc. Moreover, CLL patients have higher inpatient mortality associated with certain factors such as age progression, AKI, black race, alcohol abuse and malnutrition. Patients should be referred to cardiologist early and cardiovascular status should be optimized especially before initiating targeted therapy.[Table: see text]