Nouvelle revue francaise d'hematologie最新文献

Circulating thrombomodulin (TM) and von Willebrand factor (vWF) were determined in smokers before and after smoking of two filter cigarettes and in control subjects. The basal levels of TM and vWF were significantly increased in smokers relative to controls (p < 0.001). However, levels of these two factors remained unchanged immediately after smoking of two filter cigarettes, while a statistically significant correlation was observed only between plasma TM and number of years of smoking (p < 0.05).

Protein C levels were determined in 40 Gabonese children with sickle cell disease, in the steady state and during vasoocclusive crisis. In comparison with 40 healthy controls matched for age and sex, there was a significant decrease in protein C activity in the patients, although no difference was found between protein C levels in the steady state and during crisis.

The pharmacokinetics of two formulations of chlorambucil, Chloraminophene capsules and Chloraminophene tablets, were compared in 12 patients in a randomized cross-over study. Chlorambucil concentrations in plasma were measured by HPLC over a period of 24 h after drug intake. The peak concentration (Cmax) occurred earlier after administration of capsules than after administration of tablets [median (range)]: 0.50 (0.33-0.66) h vs 2.00 (0.66-4.00) h (p < 0.01). Although values of Cmax and the area under the plasma concentration versus time curve (AUC) were not significantly different, the two formulations were not bioequivalent. Tolerance was in both cases acceptable, with only a transient decrease in haemoglobin one day after last drug intake. The variability of chlorambucil pharmacokinetics tended to be less important for capsules than for tablets: 38% vs 71% and 35% vs 113% for Cmax and AUC respectively. Capsules are therefore likely to be more reliable than tablets for clinical use.

The aim of the present work was to study the changes undergone by proteins in ghost cells, vesicles and membrane protein aggregates during erythrocyte aging. As a model of cell, whole blood collected into CPD was stored for eight weeks at +4 degrees C. SDS-PAGE and immunoblotting with antibodies against spectrin and band 3 showed that vesicles were mainly composed of band 3 and were devoid of spectrin, whereas membrane protein aggregates contained several membrane proteins but in a more advanced state of degradation. A study of spectrin and band 3 in the ghosts, vesicles and protein aggregates revealed increasing fragmentation of both proteins with storage time. Since this degradation was most important in membrane protein aggregates, it was possible to establish the chronological order of appearance of vesicles and aggregates. In view of our observations, we propose that membrane protein aggregates may be regarded as replacement structures resulting from membrane rearrangements occurring after the emission of vesicles.

Although the occurrence of skin lesions during long-term hydroxyurea therapy is well known, longitudinal melanonychia (LM) are more rarely described. In the present paper, we report four cases of LM associated with skin lesions induced by long-term daily hydroxyurea therapy (4 to 10 years), characterized by two uncommon aspects: late onset (2.5 to 5 years) and predominance of toenail involvement in three cases.

Intrachromosomal rearrangements of the long arm of chromosome X, between gene A (F8A) in intron 22 of the factor VIII gene and one of its two telomeric copies, are responsible for about half of the severe cases of haemophilia A. A group of 98 unrelated patients from Northern France with moderate to severe haemophilia A was screened for this gene inversion using a non-radioactive Southern blotting method. Whereas none of the 18 moderately affected patients presented the FVIII gene rearrangement, gene inversion was found in 38 (48%) of the 80 severe haemophilia A patients. Recombinations involving the distal copy of gene A (group 1) were more frequent (79%) than those involving the proximal copy (group 2). Individual variation in the number of gene A copies on the X chromosome probably explains an alternative Southern blot profile, referred to as group 3 inversion, which was observed in one of our patients. In the severely affected patients, neither the prevalence of inhibitor development nor the frequency of sporadic cases differed significantly in the group presenting gene inversion as compared to the group without chromosomal rearrangement. Study of four families where no patient was available enabled in one case direct carrier detection and prenatal diagnosis in the absence of an affected member. The Southern blotting technique described in the present work is relevant to about 50% of cases of severe haemophilia A, can be performed without use of a radiolabelled probe and represents a major advance in the diagnosis of the disease.