Natural history of benign ethnic neutropenia in individuals of African ancestry.

Background: Benign ethnic neutropenia (BEN), defined by neutrophil count <1.5 k/μL in the absence of other causes, is an asymptomatic condition more commonly observed in individuals of African ancestry. However, the natural history of this condition has been less well described.

At least 20% donor myeloid chimerism is necessary to reverse the sickle phenotype after allogeneic HSCT.

Novel curative therapies using genetic transfer of normal globin-producing genes into autologous hematopoietic stem cells (HSCs) are in clinical trials for patients with sickle cell disease (SCD). The percentage of transferred globin necessary to cure SCD is currently not known. In the setting of allogeneic nonmyeloablative HSC transplants (HSCTs), stable mixed chimerism is sufficient to reverse the disease.

Kinetic assay shows that increasing red cell volume could be a treatment for sickle cell disease.

Although it has been known for more than 60 years that the cause of sickle cell disease is polymerization of a hemoglobin mutant, hydroxyurea is the only drug approved for treatment by the US Food and Drug Administration. This drug, however, is only partially successful, and the discovery of additional drugs that inhibit fiber formation has been hampered by the lack of a sensitive and quantitative cellular assay. Here, we describe such a method in a 96-well plate format that is based on laser-induced polymerization in sickle trait cells and robust, automated image analysis to detect the precise time at which fibers distort ("sickle") the cells.

Nonmyeloablative HLA-matched sibling allogeneic hematopoietic stem cell transplantation for severe sickle cell phenotype.

Importance: Myeloablative allogeneic hematopoietic stem cell transplantation (HSCT) is curative for children with severe sickle cell disease, but toxicity may be prohibitive for adults. Nonmyeloablative transplantation has been attempted with degrees of preparative regimen intensity, but graft rejection and graft-vs-host disease remain significant.

Objective: To determine the efficacy, safety, and outcome on end-organ function with this low-intensity regimen for sickle cell phenotype with or without thalassemia.

Successful fertility restoration after allogeneic hematopoietic stem cell transplantation.

Objective: Myeloablative conditioning regimens given prior to hematopoietic stem cell transplantation (HSCT) frequently cause permanent sterility in men. In patients with sickle cell disease (SCD) we use a nonmyeloablative regimen with sirolimus, alemtuzumab, and low-dose total-body irradiation (300 centigrays) with gonadal shielding preceding allogeneic HSCT. We report here the restoration of azoospermia in a patient with SCD after allogeneic HSCT.

Infusion of hemolyzed red blood cells within peripheral blood stem cell grafts in patients with and without sickle cell disease.

Peripheral blood stem cell (PBSC) infusions are associated with complications such as elevated blood pressure and decreased creatinine clearance. Patients with sickle cell disease experience similar manifestations, and some have postulated release of plasma-free hemoglobin with subsequent nitric oxide consumption as causative. We sought to evaluate whether the infusion of PBSC grafts containing lysed red blood cells (RBCs) leads to the toxicity observed in transplant subjects.

Patient and environmental service employee satisfaction of using germicidal bleach wipes for patient room cleaning.

More healthcare institutions are using bleach products which are sporicidal to reduce Clostridium difficile infection (CDI). There may be patient and employee concerns about the appearance of bleach residue left on surfaces, odors, and respiratory tract irritation. The intervention used bleach wipes for daily and terminal patient room cleaning to reduce transmission of CDI and was implemented on patient care units with a relatively high incidence of CDI.

Mobilization, collection, and processing of peripheral blood stem cells in individuals with sickle cell trait.

Mobilized peripheral blood is increasingly used as the source of hematopoietic stem cells for allogeneic transplantation, currently the only curative approach for sickle cell anemia. However, the safety and feasibility of stem cell mobilization in individuals with sickle cell trait (SCT) has not been documented. This study is a prospective controlled trial to evaluate the safety and feasibility of peripheral blood stem cell (PBSC) mobilization in 8 SCT subjects and 8 control subjects matched for age and race.

Inhibition of Patulin Production with the Insecticide Naled.

Patulin is a wide spectrum biocide produced by several species of Aspergillus , Penicillium and Byssochlamys , and is a potentially important mycotoxin that may be ingested by man. The effect of the insecticide naled on fungal growth and patulin production was evaluated using three concentrations (1, 10, and 100 mg/l, ppm) and several application times both before inoculation and during growth of the fungus. If added before inoculation, naled at 100 mg/l completely inhibited mycelial growth of Penicillium urticae for 15 days and patulin production for 30 days.