PubMed HealthSearch

Biomedical subjects

G Vaugier

Publications and source records attributed to G Vaugier.

10 recordsLinked to original sources

Partial trisomy 6p.

A case of trisomy 6p21 leads to 6pter resulting from a maternal balanced t(2;6)(p25;p21) translocation is reported. The main clinical abnormalities were psychomotor retardation, hypotrophy, blepharophimosis, nystagmus, high nasal bridge, small mouth, sacral dimple, and systolic murmur. Other anomalies might have been due to partial 2p monosomy. Comparison with seven other cases of trisomy 6p allowed the delineation of a clinical entity. Direct proof of the localization of HLA genes was given by the presence of three haplotypes in the index patient.

Abnormalities, Multiple

Identification of a pure splenic form of chronic lymphocytic leukaemia.

We have recently proposed a new staging system for chronic lymphocytic leukaemia (CLL) in which patients with isolated splenomegaly are classified into a distinct stage (stage II). Twenty-three such patients (from two institutions) have been studied without recorded death in a follow-up of 18 months to 30 years. This favourable prognosis justifies separation of these 'pure splenic forms' (SCLL) which must be distinguished from what Galton has termed prolymphocytic leukaemia (PL). This distinction can be made on the basis of three criteria: (i) Clinically, SCLL has a slow uneventful course and neither anaemia and/or thrombocytopenia: (ii) cytologically PL can be distinguished from other forms of CLL though atypical forms of CLL may be confused with the former; and (iii) the study of surface membrane immunoglobulins (SmIg) showed that while lymphocytes from most patients with both PL and SCLL bore uniform SmIg, suggesting a monoclonal B-cell proliferation, there was a major quantitative difference in that whereas PL lymphocytes had a number of antigenic sites close to that of normal lymphocytes (mean: 82 000 sites per cell), SCLL lymphocytes had a drastically reduced number of sites. It is our opinion that this is an important criterion for the differential diagnosis between PL and SCLL.

Aged

[Comparative study of the peripheral lymphocyte count in controls and in patients with chronic lymphocytic leukemias 4 hours after injection of hydrocortisone (author's transl)].

The peripheral lymphocyte count was investigated prior to and 4 h after a single intravenous injection of 400 mg of hydrocortisone (HSHC) in 23 controls and 43 patients with chronic lymphocytic leukemia. A reduction in the peripheral lymphocyte count was observed in all normal controls, the mean decrease being 51.9%, with differences according to age.

Aged

[A new parameter for chronic lymphocytic leukemia : determination of the large lymphocytes by means of Hemalog D (author's transl)].

The munber of large peripheral lymphoid cells and the ratio of these large unstained cells (LUC) to the total number of peripheral lymphocytes were determined by means of the Hemalog D in 57 patients with chronic lymphocytic leukemia (CLL) and in 100 controls. While the absolute number of LUC per mm3 is simply a reflection of peripheral lymphocytosis, the ratio LUC/total lymphocyte count was shown to correlate with clinical staging. In controls, this ratio ranged from 3.2% to 11.2%. In CLL is was less than 11.2% in 43 patients and less than 11.2% in 14 patients. This latter group corresponded statistically to patients with advanced disease in our clinical staging system (stages III and IV). An increase in the LUC/total lymphocyte ratio is therefore a statistical criterion of poor prognosis.

Autoanalysis

Investigation of a new parameter in chronic lymphocytic leukemia: the percentage of large peripheral lymphocytes determined by the Hemalog D. Prognostic significance.

The number of large peripheral lymphoid cells and the ratio of these large unstained cells to the total number of peripheral lymphocytes was determined by means of the Hemalog D in 57 patients with chronic lymphocytic leukemia (CLL) and in 100 control subjects. Although the absolute number of large unstained cells/mm3 is simply a reflection of peripheral lymphocytosis, the ratio large unstained cells to total lymphocyte count was shown to correlate with clinical staging. In control subjects, this ratio ranged from 3.2 per cent to 11¿per cent. In those with CLL it was less than 11.2 per cent in 43 patients and greater than 11.2 per cent in 14 patients. These 14 patients corresponded statistically to patients with advanced disease in our clinical staging system (stages III and IV). An increase in the large unstained cells to total lymphocyte ratio is therefore a statistical criterion of poor prognosis.

Humans

Variations in lymphocyte counts four hours after administration of hydrocortisone in patients with chronic lymphocytic leukemia.

The peripheral lymphocyte count and the number of large unstained cells (LUC) were investigated prior to and 4 hr after a single intravenous injection of 400 mg of hydrocortisone in 23 controls and 51 patients with lymphoid disorders (43 chronic lymphocytic leukemia, 3 cases of Waldenström macroglobulinemia, 2 hairy cell leukemias, 1 Sézary syndrome, and 2 cases of infectious mononucleosis). A reduction in both the peripheral lymphocyte counts and the number of LUC was observed in all normal controls, the mean decrease being 54% and greater than 60%, respectively, with differences according to age. In chronic lymphocytic leukemia (CLL), the peripheral lymphocyte count showed a variable response: decrease, no change, or increase. A correlation was shown to exist between a decrease in peripheral lymphocyte counts and anatomical-clinical staging: patients with involvement restricted to blood and bone marrow very often exhibited a drop in their peripheral lymphocyte count (p less than 0.01). In addition, the percentage of circulating T lymphocytes was higher (54%) in CLL patients whose peripheral lymphocyte count dropped than in other CLL patients (p less than 0.001).

Age Factors