American journal of hematology
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Multicenter Study
Clinical outcomes of splenectomy in children: report of the splenectomy in congenital hemolytic anemia registry.
The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. ⋯ Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short-term AEs and 11% rate of long-term AEs. As we accumulate more subjects and longer follow-up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision-making process.
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A minority of super-utilizing adults with sickle cell disease (SCD) account for a disproportionate number of emergency department (ED) and hospital admissions. We performed a retrospective cohort study comparing the rate of admission before and after the opening of a clinic for adults with SCD. Unique to this clinic was an intensive management strategy, focusing on super-utilizing adults with 12 or more admissions per year. ⋯ Similarly, the decrease in rate of 30 day re-admission was larger for the super-utilizers (13.5 per patient-year to 1.8, P < 0.001), than the whole cohort (2.6 per patient-year to 0.7, P = 0.006). Among the super-utilizers, the reduced rate of admission from the pre- to post-clinic intervention year equated to 252 fewer ED/hospital admissions and 227 fewer 30 day re-admissions. This management strategy focusing on super-utilizing adults with SCD lowered admission and 30 day re-admission rate.
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Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by the activation of the mononuclear phagocytic system. The diagnosis of HLH in adults is challenging not only because the majority of the reported data are from pediatric patients, but also because HLH occurs in many disease entities. This study reports the clinical and laboratory findings and prognostic factors of adult HLH in a large cohort managed at a single medical center from 2003 to 2014. ⋯ Ferritin >50,000 µg/L correlated with 30-day mortality. Survival after a diagnosis of HLH is dismal, especially among those with malignancy-associated HLH. The development of a registry for adults with HLH would improve our understanding of this syndrome, validate diagnostic criteria, and help develop effective treatment strategies.