IntroductionSickle cell anemia has many sequelae that result in emergency department (ED) use, but a minority of patients with sickle cell disease are frequent utilizers and make up the majority of ED visits. If patients who are likely to be frequent ED can be identified in steady state, they can be treated with disease modifying agents in an attempt to reduce ED use frequency. We sought to identify steady state markers for frequent ED use.MethodsWe identified all patients with SS/Sβ0 seen at our facilities in 2012. Health care utilization over the entire year was calculated and ED visit numbers categorized as either 0–1, 2–5, or 6 or more visits a year. Steady state and acutely active laboratory parameters were collected and analyzed using analysis of variance models and odds ratios.Results432 adult sickle cell patients were identified, ages 18–87, 54% female, and 38% had been prescribed hydroxyurea. Of the 432 patients,192 had 0–1 visits in the year, 144 had 2–5 visits in the year, and 96 had >6 visits for a total of 2259 visits. Those who had >6 visits accounted for 1750 (77%) of the total visits for the year. When steady state laboratory markers were examined, each additional 50x109/L platelets was associated with 22% greater risk (p < .001); each 1x109/L of WBC was associated with 11% greater risk (p = .003), and each 1g/dL Hb was associated with 23% lower risk (p = .007) of >6 ED visits/year. We did not observe a relationship between baseline HbF, LDH or reticulocyte count with >6 ED visits.ConclusionPatients with elevated white blood cell counts, elevated platelet counts, and low hemoglobin levels exhibited higher risk for frequent ED utilization and could be candidates for early and aggressive therapy with disease modifying agents.

: Ovarian vein thrombosis (OVT) remains poorly understood with no consensus regarding its importance or treatment. In this retrospective study, we investigated the clinical features, risk factors, treatment patterns, and prognosis of patients with OVT, including venous thromboembolism (VTE) recurrences. Adult patients who presented to our medical center with an identifiable diagnosis of OVT over a 10-year period were included in this retrospective observational study. Individual patient charts were reviewed to collect baseline and outcomes data. We identified 223 women with OVT. Median follow-up was 857 days. Only 36.6% presented with abdominal pain and 61.4% reported a history of gynecologic surgery. Overall, right or left OVT incidence was similar (44.6 and 41.4%, respectively) but peripartum patients were more likely to have right OVT (60.0%, P = 0.03). VTE recurred in 22 (9.9%) women, all of which were remote from the OVT and there were no recurrences in peripartum patients. Mean (± SD) time to recurrence was 409 (± 421) days. Only 7.6% of OVT patients were anticoagulated for OVT; these women had a 38% reduction in VTE recurrence but because of low numbers, this was not statistically significant. VTE recurrence after OVT was associated with greater mortality in all patients, including patients with cancer. OVT is associated with an increased rate of non-OVT recurrence. Peripartum OVT patients appear to constitute a different patient population as they were younger, exhibited different risk factors, and had no increased incidence of recurrence. Although only a minority of patients with OVT was anticoagulated, this group had a reduction in VTE recurrence. A prospective study is needed to determine the utility of anticoagulation for women with OVT.

IntroductionWhite Blood Cell (WBC) count, %HbF, and serum creatinine (Cr), have been identified as markers for increased mortality in sickle cell anemia (SCA) but no studies have examined the significance of longitudinal rate of change in these or other biomarkers for SCA individuals.MethodsClinical, demographic and laboratory data from SCA patients seen in 2002 by our hospital system were obtained. Those who were still followed in 2012 (survival cohort) were compared to those who had died in the interim (mortality cohort). Patients lost to follow-up were excluded. Age adjusted multivariable Cox proportional hazards models were constructed to assess hazard ratios of mortality risk associated with the direction and degree of change for each variable.Results359 SCA patients were identified. Baseline higher levels of WBC, serum creatinine and hospital admissions were associated with increased mortality, as were alkaline phosphatase and aspartate aminotransaminase levels. Lower baseline levels of %HbF were also associated with increased mortality. When longitudinal rates of change for individuals were assessed, increases in Hb or WBC over patient baseline values were associated with greater mortality risk (HR 1.54, p = 0.02 and HR 1.16, p = 0.01 with negative predictive values of 87.8 and 94.4 respectively), while increasing ED use was associated with decreased mortality (HR 0.84, p = 0.01). We did not detect any increased mortality risk for longitudinal changes in annual clinic visits or admissions, creatinine or %HbF.ConclusionsAlthough initial steady state observations can help predict survival in SCA, the longitudinal course of a patient may give additional prognostic information.

Patients with sickle cell disease (SS/Sβ0) often utilize the emergency department (ED) for treatment of painful vaso-occlusive crisis and other sequalae of their disease. However there is significant variation in use, with a minority of patients making up the majority of visits. We studied whether objective steady state laboratory parameters might be associated with frequent ED use and whether hydroxyurea use modified this relationship. Methods: We identified all patients with sickle cell disesase seen at our medical center in 2012. Patients were identified as having sickle cell disease if hemoglobin (Hb) electrophoresis demonstrated sickle hemoglobin (HbS), fetal hemoglobin (HbF) and HbA2 but no Hemoglobin A, C, or other detectable hemoglobinopathies. ED, clinic, and inpatient admissions over the entire year were calculated and ED use was categorized as either 0-1, 2-5, or >6 visits a year. Steady state laboratory tests were defined as those not within a day of an ED visit or a week of a hospital admission. All 2012 steady state parameters retrieved for a given laboratory test for a given patient were averaged. “Active” laboratory tests, defined as those within one day of an ED visit, were averaged separately. HbF and weight were not separated by activity. Data were analyzed for normality; parametric values were assessed by mean and SD, non-parametric values were assessed as medians and interquartile ranges. Parametric and non-parametric bivariate tests of association were used as appropriate. Results: 432 adult sickle cell patients were identified, ages 18-87yrs; 54% were female. 181 patients had 0-1 ED visits within the year, 143 had 2-5 visits in the year and 96 had >6 visits for a total of 2259 visits. Patients who had >6 visits accounted for 1750 (77%) of the total visits for the year. When steady state labs were examined, high WBC and platelet counts were most strongly associated with frequent admissions. Steady state WBC of >12.0 x10^9/L were significantly more likely to have >6 visits/year (OR 2.6; 95% CI: 1.6-4.2, p=.0004). Platelet counts of >420 x10^9/L were also strongly associated with >6 ED visits (OR 2.6, 95% CI:1.5-4.4, p=.0007). LDH and AST were also shown to correlate significantly with ED (p=0.02 and 0.005 respectively) use while Hb and albumin were negatively associated (p<0.001 and 0.02 respectively). Hydroxyurea scripts were associated with increased ED visits (p<0.001); 38.1% of the population had been given a script for hydroxyurea within the year; patients with frequent ED use were 2.8x more likely to have been given a script for hydroxyurea within the year. These patients had higher MCV and Hb levels and lower, but still elevated, WBC and platelet counts per ED visit cohort stratification, a suggestion that hydroxyurea was being taken, but despite this, had similar significant associations with ED visits as the non-hydroxyurea group. MCV, MCHC, absolute reticulocyte count, weight, and %HbF did not demonstrate an association with ED visit frequency. When “active” parameters were examined, leukocytosis was present in all categories and WBC count could no longer predict a predisposed cohort; Hb level, LDH and AST were also no longer significant. Albumin and platelet count remained associated with ED visits. When older patients >40 yrs (n=124) were compared with <40yrs (n=308 ), high WBC and platelet counts remained significantly associated with high (>6) ED visits in both cohorts. Conclusions: Our data suggest that baseline WBC and platelet count are strongly associated with frequent ED utilization and may be better predictors than Hb, HbF, and other red cell or hemolytic parameters. Hydroxyurea use was not associated with fewer ED visits but those patients with frequent ED use still have relatively higher WBC and platelet counts than those with less frequent ED use, suggesting that hydroxyurea under-dosing may be an important issue. To what extent objective steady state parameters can identify a population that needs more aggressive baseline care and to what extent maximization of therapy can decrease frequent ED visits needs further study. Disclosures No relevant conflicts of interest to declare.