Hemophagocytic lymphohistiocytosis (HLH) in immunocompromised hosts is a fulminant syndrome of immune activation with high rates of mortality that may be triggered by infections or immunodeficiency. Rapid diagnosis and treatment of the underlying disorder is necessary to prevent progression to multiorgan failure and death. We report a case of HLH in a patient with human immunodeficiency virus, disseminated histoplasmosis, Mycobacterium avium complex, and Escherichia coli bacteremia. We discuss management of acutely ill patients with HLH and treatment of the underlying infection versus initiation of HLH-specific chemotherapy.
Background Observational research has shown that delayed presentation is associated with perforation in appendicitis. Many factors that impact the ability to present for evaluation are influenced by time-of-day; for example, child care, work, transportation, and primary care office hours. Our objective was to evaluate for an association between care processes or clinical outcomes and presentation time. Methods Prospective cohort of 7,548 adults undergoing appendectomy at 56 hospitals across Washington State. Relative to presentation time, patient characteristics, time to surgery, imaging use, negative appendectomy (NA), and perforation were compared using univariate and multivariate methodologies. Results Overall, 63% of patients presented between noon and midnight. More men presented in the morning; however, race, insurance status, co-morbid conditions, and WBC count did not differ by presentation time. Daytime presenters (6AM-6PM) were less likely to undergo imaging (94% vs. 98% p<0.05) and had a nearly 50% decrease in median pre-operative time (6.0h vs. 8.7h p<0.001). Perforation significantly differed by time-of-day. Patients who presented during the workday (9AM-3PM) had a 30% increase in odds of perforation compared to early morning/late night presenters (adjusted OR 1.29, 95%CI 1.05–1.59). NA did not vary by time-of-day. Conclusions Most patients with appendicitis presented in afternoon/evening. Socioeconomic characteristics did not vary with time-of-presentation. Patients who presented during the workday more often had perforated appendicitis compared to those who presented early morning or late night. Processes of care differed (both time-to-surgery and imaging use). Time-of-day is associated with patient outcomes, process of care, and decisions to present for evaluation; this has implications for surgical workforce planning and quality improvement efforts.
A 25-year-old woman with a history of ulcerative colitis (UC) presented to the emergency department with lower abdominal pain. She received a diagnosis of UC 6 years earlier and has taken mesalamine to control UC. She was discharged a week before this admission for a UC flare that was managed with prednisone. Review of systems was notable for diarrhea; however, she denied hematochezia, melena, emesis, fevers, or changes in urination.Her last colonoscopy was 3 years ago, and UC without evidence of dysplasia was confirmed. Otherwise, her medical history was unremarkable. Family history was negative for inflammatory bowel diseases and gastrointestinal malignancies. On examination, she was afebrile with normal vital signs. Her abdomen was tender to palpation in the left lower quadrant, which was soft and nondistended.The patient's complete blood cell count and comprehensive metabolic panel results were unremarkable. Computed tomographic scan of the abdomen/pelvis demonstrated a thickened colon from the middescending colon to the rectosigmoid. There was also a 2.7-cm intramural abscess in the descending colon. She started receiving piperacillin/tazobactam but began to have increased abdominal pain, emesis, and inability to tolerate oral intake. A repeated computed tomographic scan performed 4 days after the original scan demonstrated that the intramural mass had increased to 3.5 cm and was partially obstructing the descending colon. She underwent a colonoscopy, which demonstrated multiple areas of inflammation in the rectum through the descending colon (Figure 1). Nothing proximal to the midtransverse colon was visualized because of a large fecal burden. amount of time elapsed since the diagnosis of UC. The risk is estimated to be approximately 2%, 8%, and 18% by year 10, 20, and 30 after diagnosis, respectively. 3 Overall, the rate of colon cancer in patients with UC is on the decline, which is likely secondary to improvements in medical therapy for UC and screening for colon can-cer in addition to early total colectomies. 4 Screening guidelines differ among different international societies. The American Society for Gastrointestinal Endoscopy recommends annual colonoscopies with random biopsies beginning 8 years after the diagnosis of UC. 5 Patients with UC have a 2.4-fold increased rate of developing colon cancer compared with the general population. 2 Ulcerative colitis-associated colon cancer (UCC) does not follow the typical adenoma to carcinoma course. Rather, UCC precursor lesions are usually nonadenomatous dysplastic epithelial cells. 1,2 Many of the genetics insults seen in sporadic colon cancer, such as microsatellite instability, aneuploidy, and mutations in TP53, KRAS, and BCL2 genes, are also seen in UCC. However, these insults are usually found earlier in patients with UC, even before the development of colon cancer. 2 These early insults are likely secondary from reactive oxygen species damage to DNA; hence, the risk of UCC increases with increased episodes and severity of inflammatory flares.Patient...
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