What's in a name? The heterogeneous clinical spectrum and prognostic factors in a cohort of adults with hemophagocytic lymphohistiocytosis.

PURPOSE: Hemophagocytic lymphohistiocytosis (HLH) in adults is rare but frequently fatal. Diagnosis is often delayed and treatment approaches vary significantly in contrast to the protocol-driven approach typically used in pediatric HLH. To improve care of these complex patients, this study retrospectively examined the prevalence, clinical characteristics, therapies and outcomes of adult HLH patients at two large tertiary care centers. METHODS: Adult patients with HLH confirmed by retrospective review of electronic medical records using HLH2004 criteria during admissions to the University of Texas Southwestern and Parkland Memorial Hospitals between June 2007 and June 2017 were studied. RESULTS: Of 31 patients included, 67.7% were male with mean age of 46 years. Average time from admission to diagnosis was 10.5 days. 48% of patients had malignancy, with T-cell lymphoma being most common. Infections were seen in 70%. Autoimmune disorders were found in 9.6%. In total, 13 patients survived (44.8%). Median survival was 8 months with increased mortality in malignancy-associated HLH (median 0.56 months versus 36.5 months, p < 0.001). T-cell lymphoma carried a worse prognosis than other malignancies. Central nervous system disease, hypoalbuminemia, elevated bilirubin, elevated soluble interleukin 2 receptor, and elevated lactate dehydrogenase, were also associated with poor survival. Treatment varied significantly. No individual treatment improved survival. CONCLUSION: This study corroborates prior limited data in adult HLH patients regarding poor survival, particularly in malignancy-associated HLH. Earlier recognition of this disease and a multidisciplinary approach to streamline diagnosis and optimize treatment are needed to improve outcomes in adult HLH patients.

Sarcomas.

Malignant bone tumors (osteosarcoma, Ewing sarcoma) and soft-tissue sarcomas (rhabdomyosarcoma, nonrhabdomyosarcoma) account for approximately 14% of childhood malignancies. Successful treatment of patients with sarcoma depends on a multidisciplinary approach to therapy, including oncology, surgery, radiation oncology, radiology, pathology, and physiatry. By combining systemic treatment with chemotherapy and primary tumor control using surgery and/or radiation, survival rates for localized disease range from 70% to 75%. However, children with metastatic or recurrent disease continue to have dismal outcomes. A better understanding of the biology underlying both bone and soft-tissue sarcomas is required to further improve outcomes for children with these tumors.

Obstetric admissions to the intensive care unit: outcomes and severity of illness.

OBJECTIVE: To determine whether mortality prediction based on a current model of outcome prediction is accurate in obstetric patients. METHODS: Consecutive obstetric admissions to a medical intensive care unit from 1991 to 1998 were reviewed to determine whether mortality prediction is feasible in obstetric patients based on a widely used model. The Simplified Acute Physiologic Score (SAPS II) was used to predict the probability of hospital mortality. RESULTS: The Simplified Acute Physiologic Score overestimated mortality in all patients (19 predicted deaths, eight observed) but accurately predicted mortality in patients admitted to the intensive care unit for medical reasons (seven predicted, five observed). The Simplified Acute Physiologic Score did not predict mortality in patients admitted for obstetric indications or postpartum hemorrhage. Median SAPS II scores were significantly higher in those patients who died, compared with survivors. For all groups, SAPS II scores were correlated with intensive care unit length of stay but not hospital length of stay. CONCLUSION: The Simplified Acute Physiologic Score accurately predicts hospital mortality in obstetric patients admitted to the intensive care unit for medical reasons but not for indications related to pregnancy and delivery. An alternate model that predicts outcomes in obstetric patients admitted for obstetric indications should be developed.

Occurrence and outcomes of sepsis: influence of race.

OBJECTIVE: To evaluate premorbid conditions and sociodemographic characteristics associated with differences in hospitalization and mortality rates of sepsis in blacks and whites. DESIGN: Secondary data analysis of the publicly available New Jersey State Inpatient Database for 2002. SETTING: Acute care hospitals in New Jersey. PATIENTS: All black and white adult patients with sepsis hospitalized in 2002. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 5,466 black and 19,373 white adult patients with sepsis were identified with the International Classification of Diseases, Ninth Revision, Clinical Modification codes for septicemia. Blacks were significantly younger than whites (61.6 +/- 0.25 and 72.8 +/- 0.11 yrs, respectively, p < .0001). Blacks had greater hospitalization rates than whites, with the greatest disparity between the ages of 35 and 44 yrs (relative risk, 4.35; 95% confidence interval, 3.93-4.82). Compared with whites, blacks had higher age-adjusted rates for hospitalization and mortality but similar case fatality rates. They were more likely than whites to be admitted to the hospital through the emergency room (odds ratio, 1.4; 95% confidence interval, 1.27-1.50) and to the intensive care unit (odds ratio, 1.14; 95% confidence interval, 1.07-1.21), and they were 3.96 times (95% confidence interval, 3.44-4.56) more likely to be uninsured. Black patients with sepsis had a greater likelihood of human immunodeficiency virus infection, diabetes, obesity, burns, and chronic renal failure than white patients and had a smaller likelihood of cancer, trauma, and urinary tract infection. CONCLUSIONS: In this study, age-adjusted case fatality rates for hospitalized white and black patients with sepsis were similar. These data are not suggestive of systematic disparities in the quality of treatment of sepsis between blacks and whites. However, blacks had higher rates of hospitalization and population-based mortality for sepsis. We speculate that disparities in disease prevention and care of preexisting conditions before sepsis onset may explain these differences.

Rapid increase in hospitalization and mortality rates for severe sepsis in the United States: a trend analysis from 1993 to 2003.

OBJECTIVE: To determine recent trends in rates of hospitalization, mortality, and hospital case fatality for severe sepsis in the United States. DESIGN: Trend analysis for the period from 1993 to 2003. SETTING: U.S. community hospitals from the Nationwide Inpatient Sample that is a 20% stratified sample of all U.S. community hospitals. PATIENTS: Subjects of any age with sepsis including severe sepsis who were hospitalized in the United States during the study period. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Utilizing International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes for septicemia and major organ dysfunction, we identified 8,403,766 patients with sepsis, including 2,857,476 patients with severe sepsis, who were hospitalized in the United States from 1993 to 2003. The percentage of severe sepsis cases among all sepsis cases increased continuously from 25.6% in 1993 to 43.8% in 2003 (p < .001). Age-adjusted rate of hospitalization for severe sepsis grew from 66.8 +/- 0.16 to 132.0 +/- 0.21 per 100,000 population (p < .001). Age-adjusted, population-based mortality rate within these years increased from 30.3 +/- 0.11 to 49.7 +/- 0.13 per 100,000 population (p < .001), whereas hospital case fatality rate fell from 45.8% +/- 0.17% to 37.8% +/- 0.10% (p < .001). During each study year, the rates of hospitalization, mortality, and case fatality increased with age. Hospitalization and mortality rates in males exceeded those in females, but case fatality rate was greater in females. From 1993 to 2003, age-adjusted rates for severe sepsis hospitalization and mortality increased annually by 8.2% (p < .001) and 5.6% (p < .001), respectively, whereas case fatality rate decreased by 1.4% (p < .001). CONCLUSIONS: The rate of severe sepsis hospitalization almost doubled during the 11-yr period studied and is considerably greater than has been previously predicted. Mortality from severe sepsis also increased significantly. However, case fatality rates decreased during the same study period.

Facing the challenge: decreasing case fatality rates in severe sepsis despite increasing hospitalizations.

OBJECTIVE: To determine recent trends in severe sepsis-related rates of hospitalization, mortality, and hospital case fatality in a large geographic area and to determine the impact of age, race, and gender on these outcomes. DESIGN: Trend analysis for the period of 1995 to 2002. SETTING: Acute care hospitals in New Jersey. PATIENTS: Subjects > or = 18 yrs of age with severe sepsis who were hospitalized in New Jersey during the period of 1995 to 2002. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We analyzed data from the 1995-2002 New Jersey State Inpatient Databases (SID) developed as part of the Healthcare Cost and Utilization Project (HCUP), covering all acute care hospitals in the state. On the basis of the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes for septicemia and organ dysfunction, we identified 87,675 patients with severe sepsis. The percentage of patients with severe sepsis among all hospitalized patients with sepsis grew steadily, from 32.7% to 44.7% (p < .0001), during these years. The crude rate of hospitalization with severe sepsis increased 54.2%, from 135.0/100,000 population in 1995 to 208.2/100,000 population in 2002 (p < .0001). Over time, the crude mortality rate rose by 35.8% (p < .0001), whereas the crude case fatality rate (number of deaths/number of cases) fell from 51.0% to 45.0% (p < .0001). For any given year, the rates of hospitalization and mortality were greater among older patients. After adjustment by age, the rates among blacks were greater than among whites, and they were greater among males than females. At the same time, there was no significant difference in the age-adjusted hospital case fatality rates with regard to gender and race. There was a significant increase in age-adjusted gender- and race-specific rates for hospitalization and mortality from 1995 to 2002. Blacks were more likely than whites to be admitted to the intensive care unit: for males, odds ratio = 1.19 (95% confidence interval, 1.13-1.26), and for females, odds ratio = 1.35 (95% confidence interval, 1.29-1.42). However, although case fatality rate was increased among patients admitted to the intensive care unit, this was not reflected in an increased case fatality among blacks. In addition, age-adjusted gender- and race-specific case fatality rates declined during 1995-2002. CONCLUSIONS: In spite of increasing rates of hospitalization and mortality, there is a decreasing case fatality rate for severe sepsis. These data suggest that advances in critical care practice before and during the study period have resulted in improved outcomes for this population.