[Who are the recipients of labile blood products? A multicenter nation-wide study--a "donation day." Blood banks, health facilities]. / Qui sont les receveurs de produits sanguins labiles (PSL)? Une étude nationale multicentrique - un jour donné. Etablissement de transfusion sanguine (ETS) - établissements de santé (ES).

During the years 1994-2001, a progressive decrease of the number of blood units transfused has been reported in France. In contrast, since 2002, there is an increasing number of blood units issuing (+7.6% between 2001 and 2006) and this must be investigated. On behalf of the French Society of Blood Transfusion, the "Recipients" working group promoted a nation wide survey with the support of the regional blood transfusion centres. This survey was aimed at describing the profiles of the transfused patients: socio-demographical patterns, and reasons of the blood transfusion (main and associated diagnoses). A cross-sectional survey was designed. All the patients who received a blood unit during a specific day were considered as the population of the study. They were identified by the regional transfusion centres by means of the "individual issuing form". Survey forms were fully filled for 90% of the patients. It has been considered as a good answer rate. Seven thousand four hundred and twenty-two blood units, delivered to 3450 patients were analyzed. Three groups of pathologies were found as a reason of transfusion: haematology-oncology (52.70% of the prescriptions) with 892 patients (27.8%) for haematological malignancies; surgical procedures (23.99%); intensive care and medicine procedures (21.92%). More than 50% of the recipients are 70 years old and more. This result is explained by the age distribution of inpatients. In a context of lack of donors and consequently difficulties to provide patients with optimal number of blood units, this study is helpful. Variability of blood unit issuings must be detected, analyzed and monitored in real time by the actors of the transfusion process, using computerized dashboards: the blood units provider (in order to adjust the strategy of blood units provision) and the health care establishment as well as care blood components prescribers (reasons of blood transfusion and evaluation of practices).

[Does red blood cell storage time still influence ICU survival?]. / L'âge des culots globulaires transfusés influence-t-il toujours le pronostic des patients en réanimation ?

OBJECTIVE: Few studies have shown that aged packed red blood cells (RBC) transfusion negatively influenced the outcome of ICU patients, probably related to storage lesions which could be decreased by leukodepletion of RBC. The purpose of this study was to evaluate the impact of aged leukodepleted-RBC pack, on the outcome of ICU patients. DESIGN: Retrospective, observational, cohort study in a Medical Intensive Care Unit. PATIENTS: Consecutive patients admitted during the years 2005 and 2006, and requiring a transfusion. We recorded patient's demographic data, number of RBC unit and age of each RBC, length of ICU, mortality during ICU stay. RESULTS: Five hundred and thirty-four patients were included with global mortality was 26.6%, length of stay in ICU six days (3-14) and SAPS II 48 (35-62). RBC equaling to 5.9 were transfused per patients (22.7%<14 days and 57.3%<21 days). The number of RBC was significantly higher in the dead patients group, but the rate of RBC stored less than 21 days was not different (54% versus 60%; p=0.21). In a multivariate logistic model, independent predictors of ICU death were SAPS II (OR=1.02 per point, p<0.001), number of RBC (OR=1.08 per RBC, p<0.001), length of stay in ICU (p<0.001). Similar results were obtained while introducing the age of RBC as time dependent covariates in a multivariate Cox's model. CONCLUSIONS: RBC transfused in our ICU are old. The ICU outcome is independently associated with the number of leucodepleted RBC transfused, but not with their age.

Abnormal T cell subpopulations and circulating immune complexes in the Guillain-Barré syndrome and multiple sclerosis.

Immunologic studies were performed in 21 patients with multiple sclerosis (MS) and 16 with the Guillain-Barré syndrome (GBS). Levels of thymus-derived (T) cells measured by "total" and "active" rosette formation between sheep erythrocytes and peripheral blood mononuclear cells (TEt, TEa) were within normal limits in all the patients, with the exception of four GBS patients, including one who also had received chemotherapy for lymphoma and three who were receiving steroids. When lymphocytes from the 21 patients were incubated with the bone-marrow-derived (B) lymphoblastoid cell line PGLC-33H, there were, for 12 of 18 MS patients and 11 of 16 GBS patients, significant decreases in a subpopulation of peripheral blood T lymphocytes that form "PGLC rosettes" (PGR) with the PGLC-33H cells. (Peripheral blood T cells from normal individuals formed PGR with 23.9 +/- 3.8 percent of PGLC-33H cells.) Using the 125l-C1q binding assay, immune complexes were detected in the serum of 14 of 19 MS patients and 15 of 16 GBS patients. An association between increased C1q binding and decreased PGR values was found in 10 of 18 MS patients and 12 of 17 GBS patients. The results suggest that in both diseases the etiology may involve a decrease in the subset of T cells that bind to the IgM-producing cell line PGLC-33H, in association with the appearance of circulating immune complexes containing the infectious viral agent.

Isolation of soluble immune complexes by affinity chromatography using staphylococcal protein A--Sepharose as substrate.

This report describes a technique for the general isolation of immune complexes, based on a combination of gel filtration and affinity chromatography. The first step is the preparation of a globulin-enriched fraction by precipitation with ammonium sulfate at 50% saturation, or of an immune-complex-enriched fraction by precipitation with 5% polyethylene glycol 6000. The enriched fraction is then subfractionated by gel filtration in Ultrogel AcA 34. The immune complexes elute close to the void volume in the macroglobulin peak, separated from monomeric IgG molecules. This peak (sometimes subdivided into two fractions) is then submitted to affinity chromatography on a protein A--Sepharose cooumn. Most immune complexes contain IgG molecules and therefore bind to the column. Almost no protein is bound when normal serum is fractionated according to this method, and no immunoglobulins are detectable in the acid-eluted fraction from the protein A--Sepharose column. In two patients with soluble immune complexes in their sera we eluted immunoglobulin-containing fractions from the column; in one, these fractions had high rheumatoid factor titers; and in the second, with a clinical diagnosis of systemic lupus erythematosus, a similar fraction contained RNA.

Mixed IgG-IgM cryoglobulinemia with glomerulonephritis. Immunochemical, fluorescent and ultrastructural study of kidney and in vitro cryoprecipitate.

Comparative studies of renal biopsy specimens and in vitro cryoprecipitate were carried out in a patient with mixed immunoglobulin G (IgG)-immunoglobulin M (IgM) cryoglobulinemia associated with glomerulonephritis. The IgM isolated from the cryoprecipitate was an antibody with anti-IgG activity. Proliferative endocapillary glomerulonephritis was found in the kidney, with large amorphous deposits in the capillary walls. On immunofluorescent examination, these deposits contained IgG and C3. Ultrastructural studies of both cryoprecipitate and glomerular deposits revealed unusual structures designated as "cylindrical or annular bodies." The morphologic characteristics of these bodies were exactly the same in the kidney and in the cryoprecipitate. These findings suggest an identity between the glomerular deposits and the circulating cryoglobulin, supporting the hypothesis that the glomerulonephritis reported here is an immune-complex disease.

[Are the indications and choice of labile blood products adapted? National survey on prescriber's knowledge]. / Les indications et le choix des produits sanguins labiles sont-ils adaptés? Enquête nationale sur les connaissances des prescripteurs.

OBJECTIVE: There is little data available on current practice related to prescription of labile blood products (LBP) by French physicians. The aim of this study was to assess whether prescriptions were conform to Anaes (French Medicine's agency) guidelines, with regard not only to indications but also quality of the products, so as to define the improvements that could be made. METHOD: Thirty-four clinical case reports, classified by specialties were sent to prescribing physicians working in the regional health centers, from 17 different blood banks, from October 1997 to February 1998. The prescribers were requested to answer only the questions that were specific to their particular field of experience. Each case description included multiple choice questions on the indication for transfusion of concentrated of red blood cells (RBC) and/or platelets (CP) and/or plasma, and the possible requirements for specification or modification of the guidelines applicable to these products. The primary end point of analysis was the adequation of the answers to the Anaes recommendations. RESULTS: Answers were obtained regarding 5092 clinical cases from 818 physicians. The participation rate was of 30%. The specialties were as follows: 34% anesthesiologists, 14% oncologists-haematologists, 13% internal medicine specialists, 11% emergency physicians, 10% paediatricians, 8% obstetricians, 7% geriatricians, and 3% transplantation surgeons. Eighty-two percent of the answers came from physicians working in the public health services. The adequation with the indication for transfusion was of 90.3% for RBC, 92.3% for platelets and 93.8% for plasma. The percentages of correct answers regarding the indications for specification or modification of the LBP were as follows: 90.3% were correct for irradiation (of either RBC or platelets); 68.8% and 53.2% respectively for leukocyte depletion from RBC and platelets; 64% for phenotyped RBC; 68.2% for compatibilized RBC; and 57.3% for apheresis platelet concentrates. There was no difference in results depending on the type of center, private or public, and the quality of LBP prescribed. The answers obtained from the anaesthesiologists' clinical cases were less accurate with regard to RBC but more accurate with regard to PC compared with other specialists. CONCLUSION: This study shows the correct management of the indications for transfusion by the prescribing physicians who participated in the study, but the lack of knowledge with regard to the indications for specifications and/or transformations of LBP. The respect of the indications for transfusion is the corner stone of safe transfusion and this phase should be optimized with improved dissemination of information on transfusion and training for the physicians and programs that would improve the quality.

Transmission of HIV by transfusion of HIV-screened blood: the value of a national register. The 'Recipients' Study Group of the French Society of Blood Transfusion.

A National Register of transfusion-transmitted infections was opened by the French Society of Blood Transfusion on 1 October, 1986. Out of 54 initially reported cases of HIV-infection, allegedly transmitted by blood components, further investigation could be completed in 33 cases. The transfusional origin of contamination was considered as established or probable in 28/33 cases, either because a potentially infectious unit was identified among those transfused to the recipient (23/28), or because the recipient was known to be seronegative before transfusion (5/28), or both (10/28). In 5/33 cases transfusion was considered as presumably responsible for contamination because no other risk factor was found in the recipient. Among the 33 documented cases of HIV-transmission by screened blood, 29 (88%) occurred between 1985 and 1987, and four (12%) during 1988. Out of 19 implicated donors later found seropositive, 16 belonged to a high-risk group for HIV-infection. The majority of HIV-infections occurred as a consequence of blood donation in the window period between contamination and the appearance of detectable antibodies in the donor's serum (11/19). In three instances, however, human and operational errors led to the release of seropositive units. We conclude that the main value of this Register is to provide a potential trend-indicator of transfusion-related infectious risks, to allow objective documentation of reported cases and to contribute to the improvement of blood transfusion practice.

Atypical B cell dyscrasia with Bence-Jones proteinuria and intracellular retention of gamma-chains.

A case of atypical B cell dyscrasia is described. The patient presented with a history of weakness, bone pain, and bleeding. A K-type paraprotein was found in serum and urine. The histology of bone marrow was that of a malignant lymphoma, but no enlargement of peripheral lymphoid organs was detectable. Peripheral blood lymphocytes were increased in number and included two populations of immature cells, one lymphoid and the other lymphoplasmocytoid. Immunofluorescent staining showed both populations to contain K and gamma chains in their cytoplasm. The clinicopathological heterogeneity suggests a malignant clone of B cells undergoing incomplete maturation, with arrest at different stages of the cell cycle.

Immune complex deposits in systemic lupus erythematosus kidney without histological or functional alterations.

This study demonstrates that in systemic lupus erythematosus (SLE), the presence of immune complexes on the glomerular basement membrane (GEM) does not invariabley result in histological and/or functional lesions of the kidney. Among a group of 29 lupus patients, six subjects were selected for thorough investigation, because their renal function was normal or only slightly altered though they had suffered from SLE for 20 months to 18 years. All patients had antinuclear factor, anti-native-DNA antibody and a low level of complement; 3 had anti-denatured-DNA antibody, 2 had denatured DNA-anti-denatured-DNA circulating complexes and 3 had anti-RNA-protein antibody. Kidney biopsies disclosed either no histological lesion or minimal changes in five of them and diffuse proliferative glomerulonephritis in one. By contrast, using the immunofluorescent technique, granular deposits containing the third component of complement (C3) were found on the GBM of all patients; IgG was present in 5 cases, IgM in 3, fibrinogen in two cases and around the tubules of one. Electron microscopy confirmed the presence of subendothelial and mesangial deposits. Our results also showed a good correlation between the importance of deposits and the presence of denatured DNA-anti-denatured-DNA circulating complexes. From the data obtained in these 6 cases as well as in the 23 other patients of the group, 3 categories of lupus patients could be distinguished with regard to kidney involvement: 1) patients with insignificant histological lesions, no immune deposits and essentially normal function; 2) patients with definite histological lesions, immune deposits and renal insufficiency and 3) patients with few if any histological lesions and little functional impairment contrasting with important immune deposits. The resistance of some patients to the mephrotoxic effects of immune deposits shows that lupus nephritis depends on intricate pathogenic mechanisms and suggests that these are possible antagonized by "protective" factors.

[Serum immune complexes in rheumatoid arthritis (a study of 100 cases). Comparison of the results obtained by four different detection techniques]. / Les complexes immuns dans le sérum au cours de la polyarthrite rhumatoïde. A propos de 100 cas. Comparaison des résultats obtenus par quatre techniques de détection différentes.

The detection of serum immune complexes in cases of rheumatoid arthritis (RA) was performed using four different detection techniques: cryoglobuline (CG), the polyethylene glycol C1q test (PEGC1q), the 125I labelled C1q test (C1qBA) and the detection of anticomplement power (ACP). CG, PEGC1q, and C1qBA are more often positive in cases of sero-positive RA than in sero-negative RA (p less than 0,001 for each test). There are significant and relatively concordant correlations between the positivity of these three tests and certain clinical and laboratory parameters in particular, the level of rheumatoid factor (p less than 0,001) and the presence of extra-articular manifestations. When ACP is found in cases of RA of recent onset, it is associated, in sero-positive cases of RA, with the presence of immune complexes and a more severe form of the disease.

[Erythroblastopenia in a child with immune deficiency and hyper IgM]. / Erythroblastopénie chez un enfant porteur d'un déficit immunitaire avec hyper IgM.

The authors report the case of a boy with a history of recurrent infections, who presented with erythroblastopenia at age 5 years. An immune deficiency with hyper IgM was found. Erythroblastopenia appeared to be related to a serum inhibitor of erythropoiesis. This inhibitor was present in the serum IgM fraction and acts by blocking the erythropoietic activity of a soluble substance in the serum, which is not erythropoietin but which could be a cofactor of this hormone. A series of 5 plasma exchanges, performed in 10 days, permitted to clear the inhibitor and induced remission, still persisting after a 12 month follow-up.