American journal of hematology
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The myelodysplastic (MDS) are a very heterogeneous group of myeloid disorders characterized by peripheral blood cytopenias and increased risk of transformation to acute myelogenous leukemia (AML). MDS occurs more frequently in older male and in individuals with prior exposure to cytotoxic therapy. ⋯ At the present time, there are no approved interventions for patients with progressive or refractory disease particularly after hypomethylating based therapy. Options include cytarabine-based therapy, transplantation, and participation on a clinical trial.
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Multicenter Study Clinical Trial
Clinical effect of reduced-intensity conditioning regimen containing antithymocyte globulin for hematopoietic cell transplantation from unrelated-donors.
The impact of reduced-intensity conditioning (RIC) on the outcomes of hematopoietic cell transplantation (HCT) from unrelated -donors (UD) remains to be determined. We therefore assessed 128 patients, aged 16 to 66 years, with acute leukemia (n = 105) or myelodysplastic syndrome (n = 23) in a UD-HCT trial using RIC with busulfan, fludarabine, and antithymocyte globulin. Patients were transplanted with unmanipulated bone marrow (BM, n = 41) or mobilized peripheral blood mononuclear cells (M-PB, n = 87) and received cyclosporine and methotrexate for graft-versus-host disease (GVHD) prophylaxis. ⋯ M-PB, 2/87; P = 0.009). Donor-patient HLA-disparity did not correlate with GVHD, 1-year TRM, and graft failure. RIC containing antithymocyte globulin led to decreased GVHD-associated, as well as overall, TRM after UD-HCT.
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Randomized Controlled Trial
Plasma glial fibrillary acidic protein levels in children with sickle cell disease.
To determine if glial fibrillary acidic protein (GFAP) is associated with brain injury in children with sickle cell disease (SCD), we measured plasma GFAP among cross-sectional groups of unselected children with SCD, subsets of children with SCD and normal brain MRI or MRI evidence of cerebral infarct, healthy pediatric controls, and adults with brain injury. Children with SCD had higher plasma GFAP than healthy pediatric controls (mean concentrations 0.14 ± 0.37 vs. 0.07 ± 0.08 ng/mL; P 5 0.003); also, 16.0% (16/100) of children with SCD and cerebral infarct had GFAP elevations above the 95th percentile of healthy pediatric controls (P 5 0.04). ⋯ GFAP was associated with elevated systolic blood pressure in the preceding year and correlated positively with white blood cell count and negatively with age and performance IQ. Plasma GFAP is elevated among children with SCD and may be associated with subclinical brain injury.
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Sickle cell disease (SCD) accounts for ~100,000 hospitalizations in the US annually. Quality of care for hospitalized SCD patients has been insufficiently studied. Therefore, we aimed to examine whether four potential determinants of quality care, [1] hospital volume, [2] hospital teaching status, [3] patient socioeconomic status (SES), and [4] patient insurance status, are associated with three quality indicators for patients with SCD: [1] mortality, [2] length of stay (LOS), and [3] hospitalization costs. ⋯ Four hundred and twenty five patients died, yielding a mortality rate of 0.6%. Multivariable analyses revealed that SCD patients admitted to lower SCD-specific volume hospitals had [1] increased adjusted odds of mortality (quintiles 1–4 vs. quintile 5: OR, 1.36; 95% CI, 1.05, 1.76) and [2] decreased LOS (quintiles 1–4 vs. quintile 5, effect estimate 20.08; 95%CI, 20.12, 20.04). These are the first data describing associations between lower SCD-specific hospital volumes and poorer outcomes.