Reliable assessment of the incidence of childhood autoimmune hemolytic anemia.

BACKGROUND: Childhood autoimmune hemolytic anemia (AIHA) is a rare and severe disease characterized by hemolysis and positive direct antiglobulin test (DAT). Few epidemiologic indicators are available for the pediatric population. The objective of our study was to reliably estimate the number of AIHA cases in the French Aquitaine region and the incidence of AIHA in patients under 18 years old. PROCEDURE: In this retrospective study, the capture-recapture method and log-linear model were used for the period 2000-2008 in the Aquitaine region from the following three data sources for the diagnosis of AIHA: the OBS'CEREVANCE database cohort, positive DAT collected from the regional blood bank database, and the French medico-economic information system. RESULTS: A list of 281 different patients was obtained after cross-matching the three databases; 44 AIHA cases were identified in the period 2000-2008; and the total number of cases was estimated to be 48 (95% confidence interval [CI]: 45-55). The calculated incidence of the disease was 0.81/100,000 children under 18 years old per year (95% CI 0.76-0.92). CONCLUSION: Accurate methods are required for estimating the incidence of AIHA in children. Capture-recapture analysis corrects underreporting and provides optimal completeness. This study highlights a possible under diagnosis of this potentially severe disease in various pediatric settings. AIHA incidence may now be compared with the incidences of other hematological diseases and used for clinical or research purposes.

Fresh-frozen plasma transfusion did not reduce 30-day mortality in patients undergoing cardiopulmonary bypass cardiac surgery with excessive bleeding: the PLASMACARD multicenter cohort study.

BACKGROUND: During on-pump cardiac surgery, hemorrhagic complications occur frequently. Fresh-frozen plasma (FFP) is widely transfused to provide coagulation factors. Yet, no randomized clinical trial has demonstrated its benefits on mortality. We assessed the relationship between therapeutic transfusion of FFP and 30-day mortality in cardiac surgery patients suffering from excessive bleeding in a prospective cohort study. STUDY DESIGN AND METHODS: Adult patients who underwent on-pump cardiac surgery and experienced excessive bleeding during the 48-hour perioperative period were recruited from 15 French centers between February 2004 and January 2006. Patients who received a preventive FFP transfusion were excluded. The association between FFP transfusion and all cause 30-day mortality was estimated using a Cox proportional hazards model, adjusted for confounding. A propensity score (PS) sensitivity analysis was also performed. RESULTS: Among 967 patients included in this study, 58.1% received FFP. The median dose was 11.3 mL/kg (interquartile range, 7.6-19.5). The cumulative 30-day mortality rate was 11.3% (95% confidence interval [CI], 9.5-13.5). FFP transfusion was associated with a higher 30-day mortality (hazard ratio [HR], 3.2; 95% CI, 1.7-6.1) in univariate analysis; however, after adjusting for prognostic factors, there was no longer any association (HR, 1.5; 95% CI, 0.8-3.0, p = 0.20). The results of the PS analysis were consistent with the adjusted analysis. CONCLUSION: Among on-pump cardiac surgery patients experiencing excessive perioperative bleeding, there is no evidence of a beneficial impact of FFP transfusion on mortality.

CD34+ cells obtained from "good mobilizers" are more activated and exhibit lower ex vivo expansion efficiency than their counterparts from "poor mobilizers".

BACKGROUND: The classification of patients into "good" or "poor" mobilizers is based on CD34+ cell count in their peripheral blood (PB) after granulocyte-colony-stimulating factor (G-CSF) injection. We hypothesized that, apart from their mobilization from marrow to the blood, the response to G-CSF of CD34+ cells also includes activation of proliferation, metabolic activity, and proliferative capacity. STUDY DESIGN AND METHODS: Mobilized PB CD34+ cells purified from samples obtained by cytapheresis of multiple myeloma or non-Hodgkin's lymphoma patients of both good (>50 CD34+ cells/microL) and poor (< or =50 CD34+ cells/microL) mobilizers were studied. The initial cell cycle state of CD34+ cells after selection and their kinetics of activation (exit from G(0) phase) during ex vivo culture were analyzed. Their proliferative capacity was estimated on the basis of ex vivo generation of total cells, CD34+ cells, and colony-forming cells (CFCs), in a standardized expansion culture. Indirect insight in metabolic activity was obtained on the basis of their survival (viability and apoptosis follow-up) during the 7-day-long conservation in hypothermia (4 degrees C) in the air or in atmosphere containing 3% O(2)/6% CO(2). RESULTS: CD34+ cells obtained from good mobilizers were in lower proportion in the G(0) phase, their activation in a cytokine-stimulated culture was accelerated, and they exhibited a lower ex vivo expansion efficiency than those from poor mobilizers. The resistance to hypothermia of good immobilizers' CD34+ cells is impaired. CONCLUSION: A good response to G-CSF mobilization treatment is associated with a higher degree of proliferative and metabolic activation of mobilized CD34+ cells with a decrease in their expansion capacity.

Low-oxygen and high-carbon-dioxide atmosphere improves the conservation of hematopoietic progenitors in hypothermia.

BACKGROUND: During short-term storage of hematopoietic cells (HCs) at 4°C a substantial decline in number and in functional capacity of progenitors occurs after 3 days. We hypothesized that physiologic O2 and CO2 concentrations of hematopoietic tissue microenvironment (approx. 3% O2 and approx. 6% CO2) could improve cell viability and functionality during storage at 4°C. STUDY DESIGN AND METHODS: Mobilized peripheral blood (PB) CD34+ cells from multiple myeloma or non-Hodgkin's lymphoma patients were stored in flasks containing air (approx. 20% O2 and approx. 0.05% CO2) or 3% O2/6% CO2 atmosphere, for 3, 5, and 7 days at 4°C. The total number of cells, the number of cells in G0 or G1 phase of cell cycle, and the apoptosis rate were determined. The functional capacity of stored cells was assessed by the capacity of progenitors to form colonies in methylcellulose (colony-forming cells [CFCs]) and of stem cells to repopulate the bone marrow (BM) of immunodeficient mice (SCI D-repopulating cell [SRC] assay). RESULTS: The total number of viable cells and cells in G1 phase as well as the number of total CFCs were significantly higher at 3% O2/6% CO2 than in air at all time points. Cells in G0 phase and SRC were equally preserved in both conditions. CONCLUSION: Atmosphere with low O2 and high CO2 concentration (3% O2/6% CO2) in hypothermia (+4°C) during 7 days of storage prevents cell damage and preserves a high number of functional HSCs and progenitors mobilized in PB by granulocyte-colony-stimulating factor.

Two cases of fatal shock after transfusion of platelets contaminated by Staphylococcus aureus: role of superantigenic toxins.

We detected Staphylococcus aureus superantigenic toxins in the platelet infusion bags that had been used for 2 patients who subsequently developed transfusion-associated Staphylococcus aureus infection. Both patients, who were immunodeficient, developed manifestations of toxic shock syndrome and septic shock, and they died soon after the onset of infection.

Comparison of CD34+ cell collection on the CS-3000+ and Amicus blood cell separators.

BACKGROUND: Mobilized PBPCs, detectable on the basis of CD34 expression, can be collected on various cell separators. The CD34+ cell collection efficiencies of two cell separators (CS-3000+ and Amicus, Baxter) were tested on two comparable groups of oncology patients. STUDY DESIGN AND METHODS: Leukapheresis assisted by the standard manufacturer's software and variables settings was performed in 37 (CS-3000+) and 34 (Amicus) patients (total of 83 and 67 collections, respectively) after chemotherapy plus G-CSF treatment. RESULTS: The total CD34+ cell count per leukapheresis components as well as per kg of patient's body weight were twofold higher by using the Amicus than the CS-3000+ device. Platelet contamination in Amicus components was twice as low compared to the CS3000+. Mean Amicus CD34+ collection efficiency (CD34+eff) (54.9 +/- 27.2%) was significantly higher (p < 0.015) than the CS-3000+ (46.4 +/- 16.7%) one. However, Amicus CD34+eff decreased progressively as the peripheral blood CD34+ concentrations increases over 200 CD34+ cells per microL. A parallel increase in the WBC counts in these cases seems to be the principal cause of decrease in CD34+eff (evident for WBCs >40 x 10(3)/microL and most pronounced for WBCs >60 x 10(3)/microL). CONCLUSIONS: Mean CD34+eff and CD34+ cell yields were better on Amicus than on CS-3000+. CD34+eff of Amicus, however, seems to be related to the initial WBC counts, decreasing progressively when WBC increased over 4 x 10(3) per microL that coincided with the increase in CD34+ cell concentrations. For these cases, the volume and duration of cycles should be adapted to optimize CD34+ collections by using Amicus separators.