[Drug-induced immune hemolytic anemia: a retrospective study of 10 cases]. / Les anémies hémolytiques immunologiques médicamenteuses: étude rétrospective de 10 observations.

PURPOSE: Drug-induced immune haemolytic anemia occurs in one case per million and can be fatal. Our aim was to describe the main characteristics and the type of drug involved. METHODS: Cases were retrospectively identified using spontaneous notifications collected by our pharmacovigilance centre and the results of immuno-hematological investigations performed by the laboratory of French blood establishment of Lyon between 2000 and 2012. Inclusion criteria were: an immune (positive direct antiglobulin test), hemolytic, anemia (haemoglobin <100 g/L), with at least a plausible causal relationship with drug exposure according to the French method of imputability or the presence of drug-dependent antibodies, and exclusion of other causes of hemolysis. RESULTS: Ten cases (5 men and 5 women, median age 54.4 years) were identified. Causal drugs were ambroxol, beta-interferon, cefotetan, ceftriaxone, loratadine, oxacillin, oxaliplatine, piperacillin-tazobactam, pristinamycin, and quinine. The median time to onset of anemia after starting the culprit drug was 6 days (2 hours to 16 days). The median nadir of hemoglobin was 57.9 g/L (range: 34-78). The direct antiglobulin test was positive in 8 patients: IgG only (n=4), IgG and complement (n=3), and IgA (n=1). Drug-induced immune haemolytic anemia was considered as definite in 5 cases with positive drug-induced antibodies, probable in 4 cases negative for the detection of drug-induced antibodies but with plausible or likely causal relationship with drug exposure, and probable with an autoimmune mechanism in 1 case. CONCLUSION: The diagnosis of DIIHA is often difficult because of the similarities with autoimmune haemolytic anemia and the inconstant sensitivity of immunologic tests that sometimes required repetitive assessment.

[Non-invasive fetal RHD genotyping: Validation of the method with 200 patients]. / RHD fœtal sur plasma maternel : validation de méthode sur 200 patientes.

BACKGROUND: Non-invasive fetal RHD genotyping is an important tool to assess the risk of fetuse's hemolytic disease of anti-D allo-immunized pregnant woman by non-invasive method. A method of genotyping has been developed in the laboratory of Lyon-GHE according to Minon's team (J Gynecol Obstet Biol Reprod 2005): exon 4, 5, and 10 are amplified by real time PCR. At first, genotyping results of 200 pregnant women have been compared with RH1 phenotype at birth. The most important parameters of validation have been tested: the sensibility and the specificity; the negative predictive value; the correlation study permitted to define criteria of biological interpretation. The validation of this method permitted to determine critical points and the limits of the method due to the minor amount of fetal DNA in the maternal plasma and existence of many variant forms of the RHD gene. CONCLUSION: We worked too in the perspective to the accreditation for our genetic laboratory.

[Management of feto-maternal red cell allo-immunizations]. / Prise en charge des allo-immunisations fœto-maternelles antiérythrocytaires.

Feto-maternal red cell alloimmunization is defined by the presence in a pregnant woman of alloantibodies directed against blood group antigens present on the red blood cells of the fetus and inherited from the father. It arises from the immune response to a first contact to these same antigens during a prior transfusion, transplant or pregnancy. The placental transfer and the fixation of the antibodies on the fetal red cells antigenic targets lead to a haemolysis in the fetus and the newborn. The resulting haemolytic disease can show different clinical forms, from a mild anaemia with neonatal hyperbilirubinemia to a major fetal damage with stillbirth caused by hydrops fetalis. The objective of management strategies of feto-maternal alloimmunization is to detect and monitor maternal alloimmunization and to appreciate the effects on the fetus or the newborn. Since a few years, some new non-invasive techniques of surveillance are used, for instance fetal RHD genotyping on maternal plasma and evaluation of fetal anaemia through velocimetry measurement of the blood flow in the middle cerebral artery. The need for a careful postnatal surveillance has to be emphasized due to the neonatal anaemia, which can be prolonged, and to the resurgence of cases of severe neonatal icteruses recently reported by the Académie de Médecine. The policy of prevention of anti-RH1 alloimmunization should also benefit from the evolution of biological techniques by allowing an improved targeting of concerned women.

Parental structure and ways of interaction in relation to the development of impaired newborns in the first year of life.

In a follow-up study of 144 newborns at high neuropsychic risk the parents were interviewed during the hospital stay and the infants were followed up one week after discharge and at 3, 6, 9 and 12 months of corrected age. The protocol included pediatric examination, neuromotor assessment, caring observation and assessment on the Brunet-Lézine developmental scale at 16 and 12 months of corrected age. An attempt was made to correlate the parents' personality structure, present experience and capacity for attunement with the infant to his development and wellbeing. After the exclusion of 11 infants with severe neuropsychic sequelae, the sample was made up of 133 infants. It was found that: despite prolonged hospitalization, in some cases for over 3 months, most of the parents by the end of the hospital stay and nearly all within a month of discharge, even if their own structures were pathological or they were suffering from reactive depression, managed (sometimes with the help of the staff) to achieve attunement with their child. This enabled him to fulfil his competences, catching up quickly in performances as soon as the parents were able to understand his messages; after 6 months of corrected age blocks and regressions emerged, related to the pathological structures of the parents or to problems bearing on their own conflicts with adverse effects on the child when he started his separation-individuation process.

[Ectodermal dysplasia. Clinical case]. / Displasia ectodermica. Caso clinico

A case of ectodermal dysplasia with anodontia, and severe hypotrichosis is described. Clinical aspects and differential diagnosis with Huth-chinson-Gildorf syndrome are also discussed.