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Biomedical subjects

L Mannessier

Publications and source records attributed to L Mannessier.

49 records · Page 3Linked to original sources

Congenital diserythropoietic anemia type I. Report on monozygotic twins with associated hemochromatosis and short stature.

We report the first occurrence of congenital dyserythropoietic anemia type I in monozygotic twins and the seventh familial occurrence to our knowledge. Mild hemochromatosis is present in the two children but has not yet required iron chelation. Moderate growth retardation, which seems to be related to pituitary failure, is also present.

Anemia, Dyserythropoietic, Congenital↗

[Ineffective erythropoiesis of immunologic origin with free bilirubin jaundice in an elderly subject].

We report on a 74-year-old male patient who presented with jaundice, highly increased unconjugated bilirubin and slight nonregenerative anemia. Ferrokinetic studies demonstrated ineffective erythropoiesis and red cell survival, studied by 51 Cr, showed moderately increased hemolysis. Other findings showed no evidence of an underlying myelodysplastic syndrome but strongly favored the autoimmune origin of the disorder, with immunologic destruction of erythroblasts (and to a far lesser extent erythrocytes) by autoantibodies.

Aged↗

Acquired loss of red-cell Wj antigen in a patient with Hodgkin's disease.

A patient with Hodgkin's disease became temporarily Wj-negative with alloanti-Wj in his serum. Four human autoantibodies, and 1 of 2 murine monoclonal antibodies, with serological characteristics of anti-Wj were nonreactive with his red cells, confirming that they have anti-Wj specificity. Six siblings of the patient are all Wj-positive. The patient was also temporarily Anton-negative, and cross-testing between Wj and Anton red cells and antisera showed mutual compatibility, indicating that the antigens are the same. The patient and 3 of his 6 siblings are also of the rare Lu: - 13 phenotype, providing the first evidence that this is an inherited characteristic.

Adult↗

Idiotypic and anti-idiotypic determinants on lymphocytes during anti-Rh immunization.

Evolution of idiotypic determinants on lymphocytes membrane and presence of other lymphocytes carrying anti-idiotypic determinants, were studied in Rh negative human volunteer blood donors during immunization towards Rh factor. For this purpose E-Rh Rosettes, direct immunofluorescence, inhibition of E-Rh Rosettes by anti-idiotypic sera as well as EA-Rh Rosettes--induced with Fab'2 fragments from anti-Rh antibodies and lymphocytes from the same subject--were examined and compared to the evolution of circulating antibodies. E-Rh Rosettes preceded or accompanied production of anti-Rh antibodies; their frequency decreased after the fifth month following immunization, meanwhile EA-Rh Rosettes increased in parallel with antibody decrease. The direct immunofluorescence and the inhibition of E-Rh Rosettes, by anti-idiotypic sera, show the presence of idiotypic determinants on lymphocyte membranes; the presence of EA-Rh Rosettes, coinciding with the decrease in antibodies demonstrate the existence of lymphocytes bearing auto-anti-idiotypic determinants.

Antibodies, Anti-Idiotypic↗

[A new case of deficient H phenotype in a secretor].

A new case of secreting Bombay phenotype has been discovered during a routine blood grouping in a Caucasian born from consanguineous parents. In spite of the presence of an anti-HI antibody a transfusion of crossmatched O units was very well tolerated by the patient.

ABO Blood-Group System↗

[Immunization with antigen D. Results obtained with 118 volunteers].

During the last four years, 118 blood donors have been immunized to obtain plasma with a high level anti-D in order to prepare anti-D immunoglobulins. The results of the immunizing schedule are very successful, as we have obtained anti-D of titer superior to 256 in 96,36% of the cases (Coombs technic). However, the development of unwanted antibodies outside the Rh system (anti-Jka: 6, anti-Fya: 5) has led us since November 1979 to use phenotyped blood without undesirable red blood cell antigens. No irregular antibody has developed since except for an anti-Yta. The anti-HLA have been observed with a frequency of 36%. The use of frozen/thawed and phenotyped blood without undesirable red blood cell antigens can allow to obtain a high level of anti-D without risk for the donors. Nevertheless, the exceptional immunization to a public antigen persists.

Adult↗

Blood group A and B activity associated with factor VIII - von Willebrand factor.

This report presents further progress on the characterization of blood group activities associated with F VIII/vWf. Evidence is provided that a relationship exists between the nature of the soluble blood group substance of plasma and of F VIII/vWf isolated from this plasma. A mixture of F VIII/vWf from O blood group plasma with A and B substances leads to a purified F VIII/vWf with AB blood group activity. Furthermore, chemical analysis of glycans released from F VIII/vWf by alkaline-borohydride treatment or hydrazinolysis shows the absence of N-acetylgalactosamine residues in non-reducing terminal positions. These results suggest that the blood group activity of F VIII/vWf preparations may be related to minor contamination of this molecule by glycolipidic or glycoproteinic plasma components with A or B oligosaccharide structures.

ABO Blood-Group System↗