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Forty-six members of a family known to have Porphyria were studied. As the disease is often latent clinically, erythrocyte uroporphyrinogen I synthetase activity was determined to classify the subjects as being healthy or carriers. HLA--A, B, C, Bf, GLO antigens were determined. No linkage between acute intermittent Porphyria and the HLA system was noted in this family.

"Natural antibodies" directed against antigens of the Major Histocompatibility Complex have been observed in aged mice but very seldom in humans. It is likely that HLA natural antibodies are more common than previously supposed: our results suggest they may be found in 1% of normal blood donors. Most of these antibodies seem to be weak and can only be detected when B lymphocytes (BL) are used as target cells in the lymphocytotoxicity technique or when indirect immunofluorescence is used with peripheral blood lymphocytes (PBL). The behavior of these natural IgM antibodies was compared to that of weak immune IgG HLA antibodies detected in the same way. Absorption tests showed that "natural antibodies" were very specifically absorbed, whereas "immune antibodies" could also be absorbed by cells not carrying the specific antigen. Furthermore, about half of the "natural antibodies" detected to date carried the HLA-B8 specificity. Various hypotheses have been put forward to attempt to explain the appearance of these antibodies.

Resume Objectifs 1) Analyser les connaissances, attitudes et pratiques en matiere de don de sang a Lome ; 2) identifier les freins au don de sang a Lome ; 3) proposer des solutions pour ameliorer les prestations du CNTS. Methodologie Questionnaire administre a un echantillon de 300 personnes selectionnees dans la population de Lome du 6 au 21 octobre 2003. Resultats L'âge des enquetes a varie entre 18 et 67 ans avec deux tiers de jeunes de 18 a 37 ans. Les eleves et etudiants representaient 24,33 % de l'echantillon, les salaries 55 % et les chomeurs 17,67 %. Plus de la moitie des individus (54,67 %) etaient celibataires et 96,33 % des personnes etaient instruites. La quasi-totalite (96,33 %) des individus savait qu'on donnait du sang a Lome. Les principales sources d'information etaient les amis, les medias et les seances de sensibilisation publique. Soixante et un pour cent des enquetes connaissaient un endroit de collecte de sang. Tous les enquetes etaient unanimes sur le role vital du sang. Quelle que soit leur religion, ils ont affirme le caractere sacre du sang. La majorite (85 %) etait disposee a donner du sang mais seulement 95 individus etaient deja donneurs. Les raisons avancees pour refuser de donner du sang etaient la peur de contracter des maladies, le manque d'information, les croyances religieuses, la vente du sang collecte et la peur du resultat du test de depistage du VIH. Quatre vingt douze virgule trente trois pour cent des enquetes ont affirme que le sang devrait etre donne sans remuneration. Conclusion Il existe un interet reel pour le don de sang au sein de la population de Lome mais il faudra elaborer de nouvelles strategies de promotion du don de sang pour convertir l'attitude favorable en veritable pratique du don de sang.

Resume En France, six trousses immunoenzymatiques (EIA) sont, a notre connaissance, soit commercialisees (Behring, Immunotech), soit en cours d'enregistrement a l'Agence du medicament (Biokit Instrumentation Laboratory, Murex et Organon Teknika). Elles depistent les anticorps anti-treponemiques totaux (Behring, Biokit, Murex, Organon Teknika), ou uniquement des anticorps de type IgG (Captia Syphilis-G Mercia Diagnostics Immunotech) ou de type IgM (Captia Syphilis-M Mercia Diagnostics Immunotech). L'objet de ce travail a ete de comparer les performances de sensibilite, de specificite, de reproductibilite et de praticabilite des quatre trousses de depistage des anticorps anti-treponemiques totaux (IgG et IgM). L'etude comparative de sensibilite, etudiee avec deux panels, a montre une bonne correlation des quatre trousses de depistage EIA entre elles et avec le TPHA. La specificite des quatre trousses EIA, determinee dans une population non selectionnee de donneurs de sang, a egalement fourni des resultats tout a fait acceptables. La specificite varie de 99,87 a 100 % selon les trousses. De plus, il a ete note une bonne praticabilite de ces quatre tests Elisa; les coefficients de variation sont tout a fait acceptables : CV des controles negatifs : 4,5–5,8 % et CV des controles positifs : 12,5–16,6 % pour les trousses EIA par competition (Behring, Biokit IL et Organon Teknika); CV des controles negatifs : 12,8 % et CV des controles positifs : 4,7 % pour la trousse par immunocapture Murex. Ces quatre trousses de depistage des anticorps anti- Treponema pallidum totaux ont montre, au cours de cette etude comparative, des performances sensiblement equivalentes. Elles presentent l'avantage d'une lecture objective et d'une possibilite d'automatisation. Elles constituent une alternative interessante aux tests manuels d'agglutination TPHA couramment utilises tant en biologie clinique qu'en biologie transfusionnelle.

Resume Malgre un traitement efficace par la penicilline, la syphilis reste un probleme mondial de sante publique, avec des disparites importantes de prevalence et de prise en charge medicale selon les pays. Des etudes nord americaines signalent une augmentation de l'incidence de la syphilis aux Etats-Unis, essentiellement dans des populations socialement fragilisees (toxicomanes, prostitutees, marginaux, exclus des soins, etc.). Les enquetes epidemiologiques effectuees recemment en France (etude RENASYPH 1991–1993, statistiques dans 7 centres d'examens de sante en 1993) sur une population tout venant n'ont pas montre d'augmentation de l'incidence de la syphilis venerienne. Cependant, devant l'ampleur de l'epidemie VIH et le nombre croissant d'individus marginalises, la syphilis reste une maladie a surveiller en France. L'Interet et la fiabilite des techniques serologiques traditionnelles dans le diagnostic et le suivi de la syphilis ont ete demontres depuis longtemps. De nouvelles techniques sont en cours de developpement. Des techniques immunoenzymatiques sont proposees comme alternative au TPHA et pourraient etre envisagees comme test de depistage unique a l'avenir. Le western-blot pourrait alors etre propose comme test de confirmation d'un depistage positif. Le developpement des techniques de biologie moleculaire permettrait un diagnostic plus precoce et plus precis des infections treponemiques, meme dans les situations ou la serologie classique est peu performante (syphilis neonatale, immunodeprimes).

Resume Ces controles sont effectues a l'aide d'un panel interne commun specifique VIH, HTLV et Ag HBs. Durant ces 32 derniers mois, 5 trousses Ac anti-HIV1 +2 (Abbott, Behring, Biotest, Murex D. et Organon T., soit 199 lots), 4 trousses Ac anti-HTLV I/II (Abbott, Murex D., Organon T. et Ortho C.D., soit 153 lots) et 5 trousses Ag HBs (Abbott, S.D. Pasteur, Murex D., Organon T. et Ortho C.D., soit 244 lots) ont essentiellement ete utilisees et verifiees par les 14 laboratoires participants. L'analyse des resultats montre que les valeurs obtenues par l'echantillon le plus representatif de chaque panel se situe dans les limites m±2DS dans une proportion de 95 a 100% pour les trousses VIH1 +2, de 94 a 97% pour les trousses HTLV et de 92 a 100% pour les trousses Ag HBs. La specificite des trousses verifiees est en moyenne egale ou superieure a 99,5%, comme l'exige l'Agence du Medicament dans une population de donneurs de sang.

Resume Le securite microbiologique des greffes de tissus est un probleme d'actualite. Plusieurs publications recentes temoignent du risque de transmission d'agents infectieux par es greffons, qu'il s'agisse de bacteries, de champignon, de parasites, de virus ou d'agents transmissibles non convetionnels (ATNC). Les textes reglementaires font obligation d'une selection des donneurs fondee sur les antecedents, l'etat clinique et des examens biologiques. Ces examens biologiques visent a rechercher des marqueurs des virus de l'immunodeficience humaine (VIH), des hepatites B et C (VHB, VHC), HTLV, cytomegalovirus (CMV), Epstein-Barr (EBV), de la syphilis et de la toxoplasmose. Les conditions de prelevement, de preparation et de conservation des greffons sont aussi des elements essentiels de la securite microbiologique. Ces conditions seront precisement definies dans les textes reglementaires organisant l'activite des banques de tissus. Parmi les questions en suspens, une meilleure connaissance du risque bacterien, l'organisation d'une biovigilance et l'utilisation de nouvelles techniques biologiques de detection des infections apparaissent comme prioritaires.

Resume Le risque de transmission de maladies virales par le sperme existe. La loi exige lors des dons de sperme en vue d'AMP la negativite des tests de depistage des infections a VIH, HTVL et des marqueurs biologiques des hepatites B et C et du cytomegalovirus. Le decret n o 94–174 concernant le CMV pose des problemes d'interpretation et d'application, et differents experts sont consultes actuellement. Depuis mai 1994, les dons issus de donneurs seropositifs en anticorps anti-CMV sont mis en attente. Une etude retrospective portant sur les dons de sperme effectues au CECOS de Strasbourg en vue d'AMP montre que seuls les anticorps anti-HBc et anti-CMV sont retrouves positifs. La prevalence des anticorps anti-HBc est estimee a 1,1 dons par an. 48,1 % des dons sont mis en attente en raison de la presence d'anticorps anti-CMV.

Resume Les hemoglobinopathies, maladies hereditaires de l'hemoglobine (Hb), sont devenues, au cours de ces trente dernieres annees, un probleme de sante publique de plus en plus preoccupant et souvent meconnu en Europe du Nord. Deux strategies s'offrent actuellement au biologiste pour depister les anomalies de l'Hb : — soit l'utilisation des techniques classiques (electrophorese de l'Hb a pH alcalin, dosage de l'Hb A2 et de l'Hb F) — soit l'utilisation de la chromatographie liquide haute performance (CLHP), chaine conventionnelle avec programme adapte ou automate, type Variant Bio-Rad, avec un programme tel que “β-thalassemia short program” qui dose avec precision les fractions Hb A2 et Hb F et qui permet de suspecter la presence des mutants les plus frequents de l'Hb (Hb S, D et E). Ces deux strategies sont des strategies de depistage. La suspicion de la presence d'un mutant necessitera la mise en place d'analyses complementaires (tests de stabilite, de solubilite, electrophorese a pH acide, isoelectrofocalisation). La CLHP devrait remplacer dans un proche avenir les techniques classiques. Elle offre l'avantage d'etre precise et rapide, de necessiter une faible quantite d'echantillon et d'etre adaptee aux analyses en grande serie. Cependant, elle reste encore tres onereuse actuellement.

Resume Le depistage systematique des anticorps anti-VHC est obligatoire sur chaque don de sang en France depuis le 1 er mars 1990. Quatre trousses de depistage des anticorps anti-VHC et quatre procedures de validation sont successivement proposees par les societes Ortho-Chiron et Abbott. Cette etude multicentrique a pour but de comparer les resultats obtenus sur une serotheque de 10 090 echantillons, provenant de donneurs de sang, avec les differentes trousses anti-VHC disponibles en evitant tout biais de recrutement. Ces resultats objectivent le gain de sensibilite obtenu depuis l'introduction des trousses de deuxieme generation, ils soulignent la bonne specificite des tests de depistage. La comparaison des trousses de validation est plus delicate car les criteres d'interpretation sont encore a preciser.

We evaluated the validity of DNA enzymatic amplification (PCR) in a population at risk for HIV-1 infection, consisting of hemophiliacs and children born to seropositive mothers. All but one of the seropositive hemophiliacs and controls were found positive with the three sets of primers. All the seronegative patients and controls were found negative in PCR. No correlation with the anti-nef serology was found, one seropositive being anti-nef negative and three seronegative anti-nef positive. The results obtained with PCR are in good agreement with classical serology, and this would suggest that the possible period of latency may not be as long as suspected. No seroconversion has been described in hemophiliacs since solvent-detergent inactivated blood products have been in use, and seronegative hemophiliacs no longer constitute a population at risk. Studies on seronegative sexual partners of seropositive patients would be of great interest. For newborns from seropositive mothers, PCR is the only possible technique in early age before seronegativation of the healthy children. Further studies will be required to determine the fiability and sensitivity of the test.

To evaluate the advantage with regard to toxicity, response rate, time to progression and survival of combination chemoimmunotherapy over single-agent chemotherapy in patients with metastatic colorectal carcinoma (CRC), 30 patients were randomized to receive a combination of 5-fluorouracil (5-FU) by continuous i.v. infusion and plasma perfusion (PP) over protein A-Sepharose (group A), or a combination of 5-FU and PP over sepharose (group B) or 5-FU alone (group C). 5-FU was given at 1,000 mg/m2/d on days 1-5 of a 4-weekly cycle until progression. Patients of groups A and B received bi-weekly on-line PPs until disease progression or for a maximum of 19 treatments. PP was well tolerated and no severe or life-threatening toxicity was observed. The response rates were 10% for the group A (1 PR), 0% for the group B and 20% for the group C (1 CR + 1 PR). The times to tumor progression for patients in groups A and C were 22 months, 12 and 11 months, respectively and the median survival times were 17 months, 10 months and 9 months. Although the time to progression and survival tended to be higher in patients treated with protein A. PP, these differences were not statistically significant. This is the first report of a randomized trial showing some therapeutic advantage in combining protein A. PP with 5-FU in CRC patients. Further randomized studies are required to demonstrate the real true value of this chemoimmunotherapeutic approach.

1) analyse the knowledge, attitudes and practices of Lome population about blood donation; 2) identify obstacles to blood donation among the population of Lome; 3) suggest some solutions to improve the performance of the National Blood Transfusion Centre (CNTS).We have conducted a sample survey among the population of Lome from the 6th to 21st October 2003. Three hundred persons filled a questionnaire.In the sample, 183 (61%) were males. Their age ranged from 18 to 67 years with 2/3 of young peoples between 18 and 37 years. Pupils/students were 24,33%, wage-earners, 55% and the unemployed, 17,67%. More than the half of the investigated persons (54,67%) were unmarried and 96, 33% persons were literate. Nearly all the people (96,33%) knew about blood donation in Lome and they have received information mainly from friends, media and sensitization by the CNTS. Sixty-one percent of the investigated knew where blood is donated. All the investigated were unanimous about the vital role of blood. Whatever their religion, they recognized its sacred nature. The majority of the investigated (85%) were willing to donate blood. Only 95 people were donors. The reasons for non-donation were mainly related to the fear of catching diseases (31,71%) especially the HIV (9,76%), the lack of information (25,37%), the religious beliefs (19,51%) and the fear of knowing the result of one's HIV test. 277 people (92,33%) said that blood should be donated without remuneration.There is a growing interest in the blood donation among the population of Lome but new strategies must be elaborated to inform the population and motivate blood donors.

Update the data collected in 1990 in order to assess the distribution and management of thalassemic patients presently living in Metropolitan France.A survey conducted in France in the clinical and biological departments of haematology permitted collection of epidemiological, clinical and biological data in a population of thalassemic patients followed-up in metropolitan France.Analysis of the replies revealed a total of 362 thalassemia with 249 beta-thalassemia major, 81 beta-thalassemia intermedia and 32 E-beta thalassemia. These patients predominated in the East of France and in the large cities. The total number of patients has remained stable over the last decade and new cases are decreasing. Among the 249 patients presenting with a ss-thalassemia major, 42 had received a bone marrow graft, whereas 207 were systematically transfused and 189 regularly underwent iron chelating.Management is standardised and efficient but could be improved on with regard to iron chelating therapy.

Hemoglobinopathies have become a significant national health problem in France. The biologists have a pivotal role in the genetic diagnoses. Although sickle cell disease (SCD) is the most frequent abnormality found: not less than 200 new cases are observed each year at birth, many other globin gene variations are found in the various ethnic groups. Since 1995 a neonatal sickle cell screening program has been established for at risk newborns. This programme is supported by the "Association française de dépistage et prévention des handicaps de l'enfant" (AFDPHE). The characterization of hemoglobin genetic variations requires a comprehensive set of laboratory techniques for which we specify here main clinical and technical recommendations.

In order to compare sensitivity, five anti-hepatitis C virus (HCV) immunoblot assays were tested (Deciscan plus, Inno-Lia III, Matrix, Murex Western blot and RIBA-3).The test panel (50 samples for each assay) included 6 anti-HCV-negative samples and 44 samples from 36 HCV-infected subjects.There were minor differences in core reactivity among the assays. The smallest number of NS3-reactive results occurred with the Murex and Matrix assays, and the smallest number of NS4 reactives with RIBA-3 and Matrix. Among the 20 discrepant results for NS5 there was one clear false-negative with Inno-Lia. Only 28 of the 50 samples of the panel gave the same results in all the assays: 5 negatives and 23 positives. One of the 6 negative samples were indeterminate in 3 assays. Eighteen of the 21 other divergent results were interpreted as either indeterminate or positive, a common reactivity being exhibited by all 5 assays. The most important discrepancies occurred on 3 HCV-RNA-positive samples which came up negative in some assays: 2 samples with isolated NS3 reactivity were negative by Matrix and Murex Western blot, 1 of them being also negative by Inno-Lia III; another sample was negative by RIBA-3 and Matrix due to weak signals (1) on core and NS3 proteins, which did not exceed 1+ with the other assays.With more uniform criteria for interpretation, the results would have been less divergent. Some assays should improve their sensitivity to the NS3 protein.

Controls were performed using a common internal panel specific for anti-HIV, anti-HTLV and HBs Ag. During the past 32 months, 5 anti-HIV1 + 2 antibody kits (Abbott, Behring, Biotest, Murex D. and Organon T., total of 199 lots), 4 anti-HTLV I/II antibody kits (Abbott, Murex D., Organon T. and Ortho C.D., total of 153 lots) and 5 HBs Ag kits (Abbott, S.D. Pasteur, Murex D., Organon T. and Ortho C.D., total of 244 lots) were used and controlled by the 14 participating laboratories. Analysis of the results showed the values obtained for the most representative sample of each panel to be within the limits m +/- 2 SD in 95 to 100% of the HIV1 + 2 kits, in 94 to 97% of the HTLV kits and in 92 to 100% of the HBs Ag kits. The specificity of the kits evaluated was on an average equal or superior to 99.5%, as required by the French Drug Agency in a blood donor population.

Hb Puttelange [beta 140(H18)Ala--Val] was found as a de novo mutation in two siblings of a French family suffering from polycythemia. Both parents were phenotypically normal and exclusion of paternity has been ruled out by the study of several polymorphic markers located on different chromosomes. The structural modification of Hb Puttelange was established by reversed-phase HPLC analysis of the tryptic digest of the abnormal chain. The amino acid composition of an abnormal beta T14 peptide revealed that one of the four residues of Ala was replaced by a Val. Tandem mass spectrometry demonstrated that the substitution concerned position beta 140 (H18). This hemoglobin displays an increased oxygen affinity that is responsible for the polycythemia. De novo mutations, as demonstrated again in the case of this variant, have the highest probabilities of detection when they lead to pathological manifestations. They may result either from a somatic mutation in a very early stage of the embryological development of the propositus or may have a parental origin with occurrence of a germline mosaicism. The study of the beta-globin gene indicated that this case of Hb Puttelange probably arose from a mutation affecting a part of the germline of the father, therefore leading to a true recurrence risk.

The number of subjects with heterozygous beta-thalassaemia and sickle-cell anaemia in metropolitan France can be evaluated by the distribution of populations originating from countries with a high prevalence of genetic haemoglobinopathies. Taking into account the movements of these populations observed since the 1982 census, the current prevalences of beta-thalassaemic and drepanocytic traits are higher than the figures of 180,000 and 130,000 respectively found at that date. On the other hand, it appears from episodic screenings performed during the last few years that the percentage of subjects with heterozygous beta-thalassaemia is 3.09% in Corsica, 0.77% in school-age population in the Marseille area, and 0.72% in the general population of Marseille, Toulon and Nice. In 1992, the number of patients with heterozygous beta-thalassaemia and with major sickle-cell syndrome is estimated at 250-350 and 1000-3000 respectively. Patients with sickle-cell anemia predominate in the Paris area, whereas those with heterozygous beta-thalassemia predominate in the provinces, notably in the Provence-Côte d'Azur and Corsica regions.

Several possible diagnoses are proposed to explain the symptomatology observed in a slightly jaundiced 10 month-old suckling infant suffering from fever and anaemia. Major beta-thalassaemia in its most severe form (Cooley's syndrome) proved to be the correct diagnosis. The importance of family background is stressed for these haemolytic cases.

HIV seroprevalence decreased from 0.62% in 1985 to 0.11% in the first 1990 semester. However, the number of regular blood donors screened as seropositive remains constant since 1988: 48 in 1988, 49 in 1989 and 25 in the first 6 months of 1990. From these data, from reports on recipients and due to the exclusion of 30% of such donors by anti-HBc screening, the residual risk to transmit HIV by blood transfusion was estimated to 17 blood donations per year in France. No significant change was observed throughout these years either in sex ratio or in the repartition into age groups. The number of HIV-infected subjects through the heterosexual route has not increased. The number of homosexuals and of IVDA has dramatically decreased since 1985, homosexuals still representing the major at-risk group.

Since december 1st 1988, the Retrovirus study Group of the French Society of Blood Transfusion has initiated a systematic multicentric control of all anti-HIV screening EIA test kits lots used by each member of the group. For this purpose a special panel (GRV) has been designed and forwarded to each laboratory. From december 1st 1988 to august 8 1989, 6 HIV-1 kits have been in use (Diagnostics Pasteur Elavia 1; Diagnostics Pasteur Rapid'Elavia; Organon Vironostika; Abbott HIV recombinant; Du Pont env 9; Wellcome Wellcozyme). The GRV panel has been run 204 times on 102 different lots in 12 laboratories. Results after 9 months show that these 6 kits almost always properly recognize the 5 HIV 1 positive samples of the panel. However they differ in their ability to recognize HIV 2 positive samples. Variations in results are observed from lot to lot as well as between the first half of this period and the second.

We studied alpha 1-antitrypsin deficiency in a large family of 10 siblings: 3 subjects had PiZZ phenotype, but only 1 had emphysema; 2 subjects had no respiratory complaint. The patient with emphysema was a heavy smoker. According to the literature, this case suggests that, in PiZZ phenotype, emphysema appears earlier and is more severe if the patients smoke.

The quantitative expression of three allotypes--G1m(1), G3m(5) and G3m(21)--has been studied in normal caucasoid sera. A gene dosage effect, previously described for all of these allotypes, is not noticed for G3m(5) in the present study. The different content of G3m(5) and G3m(21) IgG3 molecules in heterozygous Gm5/Gm21 sera has been verified: these contents are respectively 75% and 25% of the IgG3 content. The same allotypes have been studied in a family which shows the inheritance of a weak Gm1,17;21 haplotype. The gene responsible for this abnormality is probably a new regulatory gene, and is transmitted without the 'aimed' haplotype.

A new G6PD variant, called G6PD Vientiane, has been discovered in a patient from Laos. The characteristics of this variant are: mild enzyme deficiency (about 50% of the normal activity) in the granulocytes and the red cells, with normal G6PD-related antigen concentration; increased stability; normal Km glucose 6-phosphate and NADP+; increased inhibition constant by NADPH; decreased inhibition by ATP; slightly increased utilization of the substrate analogue; abnormal pH curve, with maximum activity at pH 9.5; slightly reduced starch gel electrophoretic migration. The implications of the molecular stability of a deficient mutant variant are discussed.

Resume Du Pont de Nemours preconise une nouvelle methodologie dans l'utilisation de sa trousse immuno-enzymatique de depistage des anticorps anti-HIV 1. La nouvelle methode se caracterise par sa rapidite : 2 heures d'incubation, alors que l'ancienne necessitait 3 h 30 d'incubation. Nous avons etudie en methode courte et en methode longue 4 025 serums de donneurs de sang, 55 echantillons du panel de la SNTS et 10 serums servant de reference pour le Western-Blot dans le groupe « Retrovirusde la SNTS. Nous en concluons que la methode courte presente des avantages certains comparee a la methode longue : - gain de temps important (1 h 30) ; - gain de specificite : de 0,37 % en methode longue dans notre etude, le taux de faux positifs passe a 0,07 % en methode courte ; - bonne sensibilite.

Hb J-Cordoba [α2Aβ295(FG2)Lys→Met], is one of the few hemoglobin variants discovered in Argentina. The structure and functional abnormalities are described. Hb J-Cordoba exhibits a slightly increased oxygen affinity, low cooperativity, and normal interaction with heterotropic cofactors.

Summary HLA-A, B, C and Bf antigens were studied in 153 blood donors who developped a titre of ⩾ 25 i.u./ml antitetanus antibodies following tetanus anti-toxin vaccination. Antigen frequency was compared with that of 412–464 normal blood donors living in the same area. Few significant differences were demonstrated: absence of Bw21 antigen and slight increase of Bf F antigen were observed in « good responders .

SUMMARY An unexpected MLR reaction has been observed between three HLA-identical sibs; it consists of a bidirectional positive MLR between identical female twins and a sister. No argument for a lymphoid mosaic could be found, although twins were frequent in the family; similary no HLA-A/B or HLA-B/D recombinant could be demonstrated. The MLR, although weak, was highly reproducible. PLTs could be raised between the sibs, without an apparent segregation in this family nor in five other families, but such PLTs discriminated well between the positive and negative controls. In the absence of any proof that such a weak MLR locus could be on another chromosome than chromosome 6, two lines of argument are indirect evidences that such a locus could be indeed on chromosome 6: one of the sibs differs from the two others for two markers outside HLA-D-DR-Bf: glyoxalate (GLO) and red blood group P.

In 1973 Asakura demonstrated that, following mechanical shaking, oxy-Hb S was much less stable than oxy-Hb A (Nature 244: 437, 1973). We have studied the mechanical stability of Hb Crétil (beta 89 Ser leads to Asn), Hb Hope (beta 136 Gly leads to Asp), Hb Strasbourg (beta 23 Val leads to Asp), and the hybrid Hb S/Stanleyville-II (beta 6 Glu leads to Val: alpha 78 Asn leads to Lys) by the method of Roth et al. (Blood 45:377, 1975). Hb Créteil, Hb Hope, and Hb S/St-II were sensitive to mechanical shaking, while Hb Strasbourg was more stable than Hb A, a hitherto undescribed finding. The precise mechanisms responsible for this precipitation are not known. From comparisons with published results, but excluding thermosensitive Hbs, we conclude that: - standard methods for isolation of hemoglobins modify its mechanical stability, - alpha mutation increases the mechanical stability of Hb S in the hybrid Hb S/St-II, - some mutations produce a more stable hemoglobin than Hb A.

A new G-6PD varient with enzyme deficiency is described in a 7-month-old Turkish boy without any hemolytic manifestation, except neonatal hyperbilirubinemia. The main characteristics of this variant were the following: Severe enzyme deficiency in erythrocytes (8% of normal), fast starch-gel-electrophoretic mobility (110% of normal), increased Ki NADPH with respect to NADP+, slightly biphasic pH curve, enzyme instability, in vivo and in vitro, decreased molecular specific activity (58% of normal).

Therapeutic fractions were obtained by fractionating human plasma containing HBs antigen, by the Cohn ethanol technique modified by Nitschmann. Plasmas with various HBs antigen titers were selected. The antigen was sought in each fraction by the techniques of counterelectrophoresis and radioimmunological detection in liquid phase. With the exception of very pure albumins, all fractions were found to contain the HBs antigen, though in variable proportions. A technique for the purification of albumin, applicable on an industrial scale, is proposed. It makes it possible to obtain an albumin fraction in which the HBs antigen cannot be detected by the most sensitive techniques, even starting with a plasma which is very rich in HBs antigen (titer 1/64 by counterelectro phoresis).

Resume Une enquete, portant sur la prevalence des anticorps anti-HIV chez les donneurs de sang francais pendant les neuf premiers mois du depistage systematique, a fourni les renseignements suivants : -- prevalence de 0,59 pour mille (1661/2.809.744) ; -- difference tres significative entre les nouveaux et les anciens donneurs (2,04 ‰ et 0,39 ‰ respectivement) ; -- predominance masculine (85 %) ; -- 65 % ont moins de 30 ans ; -- les facteurs de risque majoritaires sont l'homosexualite (49 %) et l'utilisation de drogues par voie intra-veineuse (28 %).

Resume Nous avons teste la trousse Wellcozyme anti-HTLV III sur : - 600 serums frais provenant de donneurs de sang non selectionnes, testes simultanement par Elavia (Diagnostics Pasteur) ou Vironostika (Organon) ; - 20 plasmas connus comme etant anti-HIV negatifs ; - 22 serums connus et 10 serums d'un panel du groupe de travail « Retrovirusde la Societe Nationale de Transfusion Sanguine, testes par Elavia, Vironostika et Abbott HTLV III EIA et par deux tests de confirmation: Immuno-blot (Diagnostics Pasteur) et test de confirmation Abbott. La trousse Wellcozyme est actuellement la seule trousse de depistage anti-HIV a utiliser une reaction elisa par competition et a ne pas necessiter de dilution prealable du serum. La technique est donc aisee, rapide (resultat obtenu en 1 h 30) et elle s'applique aussi bien a des echantillons de serum que de plasma. Il n'a ete observe aucune interference de fortes concentrations lipidiques ou d'une hemolyse importante sur la reaction enzymatique. Ses performances sont satisfaisantes. Nous n'avons pas trouve de faux positifs. Par contre, il apparait que les trousses Wellcozyme manquent de sensibilite vis-a-vis des anticorps anti-P25 qui apparaissent en debut de seroconversion si la zone des valeurs douteuses n'est pas etendue. Cette recommandation, faite par les laboratoires Wellcome depuis peu, augmente la sensibilite du test actuel dans la detection des debuts de seroconversion sans nuire a sa specificite.

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Resume L'antigene Australia peut etre present dans certaines fractions plasmatiques et l'E.I.D. manque nettement de sensibilite pour le detecter, si le plasma d'origine a un taux d'antigene relativement faible. L'utilisation de la D.R.I. serait donc souhaitable pour la detection de l'antigene dans les pools de plasma et les fractions plasmatiques, ce qui devrait permettre d'eliminer au maximum les risques d'hepatite apres injections de derives sanguins. Cette technique est appliquee au C.N.T.S. depuis 1971.

Resume La survie d'une preparation de gamma-globulines humaines a injection intraveineuse a ete etudiee chez 9 sujets normaux et volontaires. La proteine etait marquee par l'iode 125 ou l'iode 131 . Avec un modele a deux exponentielles, la periode de decroissance de la radio-activite plasmatique pour la deuxieme exponentielle a ete determinee a 11,8 ± 2,3 jours.

Red cell glyoxalase I polymorphism was investigated in a population sample from the Strasbourg area. Gene frequency distribution in 337 unrelated subjects was GL01 = 0.455, GL02 = 0.545. There was no

Haemoglobin Stanleyville II has been found several times in individuals from Central Africa. This paper now reports its discovery in Alsace, in a family of long-standing local ancestry.

A G-6-PD deficiency has been found in a Turkish male premature with neo-natal hyperbilirubinemia. His parents have no hemolytic antecedents. The propositus has a severe enzym deficiency in erythrocytes, a very decreased activity in leukocytes and platelets. His mother was heterozygous for this variant and his father had no abnormality. The deficient enzym was a new variant: G-6-PD Ankara. The main characteristics of this variant were the following: 1 degree severe enzym deficiency in erythrocytes (8% of normal); 2 degrees fast starch gel electrophoretic mobility (110% of normal); 3 degrees enzym instability in vivo and in vitro; 4 degrees increased KiNADPH; 5 degrees decreased molecular specific activity (58% of normal). Only variant B(-) G-6-PD deficiency have hitherto been described in Turkey. In contrast, G-6-PD Ankara is a fast variant and is unlike any other known variants.

Basal plasma levels of thyroxine (T4), triiodothyronine (T3) and reverse T3 were determined by radioimmunoassay in 44 control subjects, 44 Type 1 (insulin-dependent) and 39 Type 2 (non insulin-dependent) diabetic patients aged from 15 to 75 years. All were clinically euthyroid. The quality of diabetic control was assessed by the percentage of glycosylated haemoglobin. In both the diabetic groups there was a significant decrease in T3 and a rise in reverse T3 whereas T4 was normal. We found no significant differences between plasma thyroid hormone levels in Type 1 and Type 2 diabetic patients. In the poorly controlled diabetics (glycosylated haemoglobin greater than or equal to 12%), T3 was 90 +/- 5 ng/dl, which differed significantly from the level found in the better controlled patients (106 +/- 5 ng/dl, p less than 0.01). In the diabetic patients without associated illness, a negative linear correlation was found between T3 and glycosylated haemoglobin and a positive correlation between reverse T3/T3 and glycosylated haemoglobin. No correlation between T3 or reverse T3 and fasting blood glucose could be established. In conclusion, many diabetics showed a low T3 syndrome suggesting that there may be an impairment in the extrathyroidal conversion of T4 to T3. This may well be enhanced by a poor diabetic control (glycosylated haemoglobin greater than or equal to 12%).

A patient with lymphosarcoma and cold urticaria showed evidence of complement activation by the classical pathway with low levels of the early complement components, normal levels of late acting components, normal functioning of the alternate pathway and reduction of the C1-inhibitor level. The serum contained an IgG1 monoclonal cryoglobulin responsible for the complement activation. In vitro tests demonstrated a high capacity of the serum to activate C1.

Variations in haemoglobin A1c were studied at different stages of pregnancy in 306 non-diabetic women, using a specific method. It was found that HbA1c levels progressively decreased during the first 25 weeks of pregnancy, then remained stable. No correlation was noted between carbohydrate metabolis and Hb A1c levels. The new assay provides additional information on glucose tolerance in pregnant women and could be used to detect gestational diabetes.

We tested the Wellcozyme anti-HTLV III kit on: 600 blood donors fresh sera, simultaneously tested by Elavia (Diagnostics Pasteur) or Vironostika (Organon); 20 plasmas, known to be anti-HIV negative; 22 sera, already labelled, and a panel of 10 specimens from the "Retrovirus" study group of the French National Society of Blood Transfusion, also tested by Elavia, Vironostika, Abbott HTLV III EIA and by two confirmatory tests: Immuno-blot (Diagnostics Pasteur) and Abbott confirmatory test. The Wellcome kit is, at present, the only one to use a competitive Elisa method and not to require any sample predilution. The method is easy to perform, rapid (results in 1 h 30 time) and it tests as well serum as plasma. No interferences have been observed from high lipid concentrations or strong hemolysis on the Elisa reaction. The performances of the Wellcozyme test are satisfactory. We did not find any "false positive" result. But the study shows that this kit is not sensitive enough for the detection of anti-P25 antibodies, which appear on the beginning of seroconversion, unless the "grey range" is extended: the Wellcome Laboratories have just made this recommendation.