Nouvelle revue francaise d'hematologie; blood cells最新文献

The authors determined the agglutination percentages with anti-B, eel and Ulex europeus anti-H reagents for the kinetic study of the in vitro conversion O red blood cells in B. The agglutination with the anti-B increases in proportion as the agglutination with eel anti-H decreases; the agglutination with Ulex anti-H remains constant. They converted (time=18 h) O red blood cells with a more or less high substance H content (adults in good health, africans and europeans, patients, newborn and one "Bombay" phenotype). They showed that there is a good correlation between the agglutination percentages with eel anti-H or Ulex anti-H before conversion and the agglutination percentages with anti-B after conversion. These "O converted" look like the B phenotype defined by a check sample of B subjects.

Relapsing erythroblastopenia has been observed in a girl from when she was a baby to 22 years of age. The three episodic crisis of erythroblastopenia occurred when she was 2 months, then 6 and 19 years old, and recovered spontaneously after periods of 3, 18 and 30 months respectively. The authors did not find any cause for the disease and the various treatments were useless. This case presents hardly any relation to the rare relapsing erythroblastopenias reported in the literature.

From rat bone marrow in which erythropoiesis has been depressed (by suppression of erythropoietin production), liquid culture has yielded a predominantly synchronous amplification of erythroblast development. Groups of 2, 4, 8, 16 and 32 erythrolbasts are observed at 9-15 hour intervals in successive stages of maturation, similar (by their staining characteristics and nuclear size) to that seen in vivo.

Hb G Philadelphia (alpha68 Asn leads to Lys) is widely distributed in black people but is uncommon in North-Africa. Only one case has been previously described in an Arab immigrant. The latter and our propositus originated from North-East morocco. Hb G alpha Philadelphia is stable. The abnormal hemoglobin represented 28% of total hemoglobin in the hemolysate of the propositus, a 7 month old child. At birth, 4 fractions were detected on electrophoresis: Hb A, Hb F, Hb G Philadelphia, which migrated like Hb S, and a mutant alpha2Ggamma2. The oxygen affinity of Hb G Philadelphia was slightly elevated. Cooperativity and Bohr effect were normal. The abnormal hemoglobin was also detected in the father of the propositus in the heterozygote state: its clinical and hematological data were normal. No evidence of thalassemia trait was found in the family. The percentage of abnormal hemoglobin obtained in the propositus and his father is in accordance with the presence of two loci for the alpha chain on the homologous chromosome.

Screening for red cell defects, and exploration of cellular and humoral immunity has been performed in 33 patients : 31 had agnogenic myelosclerosis with myeloid metaplasia, 3 had polycythemia vera with secondary myelosclerosis. No patient had the biological abnormalities characteristical of paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli). In 19 out of 21 cases, red cells had antigen i on their membrane, thus suggesting that splenic erythropoiesis could give rise to immature erythrocytes. Two patients had a monoclonal dysglobulinemia, 5 a positive Coombs test, 6 a rhumatoid factor in the serum, 3 antitissue antibodies, 1 LE cells, 3 a positive Paul-Bunnel-Davidsohn test without mononucleosis, 11 a negative skin test. Implications of the uncommon occurrence of these dissorders are discussed.