[Epidemiology of infections caused by HIV-1, HIV-2 and HTLV-1 in the republic of Chad]. / Epidémiologie des infections à rétrovirus VIH1-VIH2 et HTLV1 en Republique du Tchad.

From March to July 1989 sero-epidemiological surveys have been carried out in four Chadian towns on representative samples of adults aged 15-44 years in the general population. For the first time, HIV1 virus circulation was shown off with seroprevalence rates varying from 0 to 1.1%. Rates are non-existent for HIV2 and they vary from 0 to 1.6% for HTLV1. These low seroprevalence rates make it difficult to identify special risk factors. There was confirmed a preponderance of heterosexual transmissions of these viruses.

[Immune response in splenectomized children to anti-pneumococcal vaccination]. / Réponse immunologique de l'enfant splénectomisé à la vaccination anti-pneumococcique.

The antibody response following anti-pneumococcal immunization with a 14-valent vaccine was studied in 54 children, aged 2 to 18 years after splenectomy. Splenectomy had been performed for traumatic, hematologic or immunologic reasons. Antibody titration with respect to 12 serotypes was performed by radioimmunoassay immediately before and one month after immunization. Changes in responses were studied according to the indication for splenectomy and to the vaccine serotype. The main conclusions of this study are the excellent tolerance of immunization, the lack of significant difference between the average responses according to the indications for splenectomy, the obtainment of a titer probably protective for 80% of the patient studied and the confirmation of the low immunogenic character of serotype 6A. These results suggest that anti-pneumococcal prophylaxis by immunization can be highly recommended before splenectomy but it can also be performed after surgery.

Common variable hypogammaglobulinemia in children. Clinical and immunologic observations in 30 patients.

We made clinical and immunologic observations of 30 children with common variable hypogammaglobulinemia. The mean age at diagnosis was 10.5 years, five years after clinical onset. Diagnosis was initially made based on a history of recurrent otobronchopulmonary infections, diarrhea, or both. The most common complications included short stature, bronchiectasis, and malabsorption, often associated with giardiasis or sprue. Nine patients had associated autoimmune diseases (eg, atrophic gastritis, arthritis, and hemolytic anemia). Three patients died, one of chronic respiratory insufficiency, one of chronic persisting hepatitis, and one of osteogenic sarcoma. Humoral and cellular immune functions of all patients were examined.

Evidence that abnormal platelet functions in human Chédiak-Higashi syndrome are the result of a lack of dense bodies.

The structure and functions of platelets from three patients with the Chédiak-Higashi syndrome were examined. Electron-microscopic observations revealed a large reduction in the number of serotonin-storage granules or dense bodies but otherwise normal ultrastructure and normal amounts of alpha-granules and catalase-positive granules. The number of mepacrine-labeled granules was also reduced. Platelets contained normal amounts of beta-thromboglobulin and Platelet Factor 4. The platelet release reaction studied with thrombin as the inducer was impaired. The serotonin uptake by the patients' platelets was low and not inhibited by reserpine, and its metabolism was increased. These findings clearly show that platelets from human Chédiak-Higashi syndrome are deficient in the storage pool of dense granule substances and suggest that this granule defect has an influence on the release mechanism of other granule constituents.

[Primary immunologic deficiencies and cancer. 5 anatomo-clinical case reports]. / Déficits immunitaires primitifs et cancers. Cinq observations anatomo-cliniques.

Five patients with primary immunodeficiency and cancer are presented. Two children with ataxia-telangiectasia developed acute lymphoblastic leukemia and malignant lymphoma of B-like origin with chromosome damage and unusual prevalence of antibodies to E.B.V. early antigen. A bone sarcoma occurred in a patient with common variable hypogammaglobulinemia. At least two infants who died with severe combined immunodeficiency had at autopsy congenital myelomonocytic leukemia and malignant lymphoma. These cases indicate the high risk for development of cancer in patients with primary abnormalities of the immune system and suggest the heterogeneity and complexity of pathogenic mechanisms.

[Susceptibility to infections and hyper IgE: 19 new case reports ]. / Syndrome de susceptibilité aux infections avec hyper IgE. A propos de 19 nouvelles observations.

Nineteen patients exhibiting a susceptibility to infections due to staphylococcus and fungi, allergy and hyper IgE are reported (syndrome described by R. Buckley). Prognathism, coarse features and marked osteoporosis were observed in more than half the patients. Immune disorders were characterized by a defective chemotaxis of granulocytes and depressed in vivo and in vitro immune responses to antigens. These cellular abnormalities could be secondary to a dysregulation of IgE synthesis resulting in an excess of secretion of histamine, heparin and other substances from mast cells. According to this possible mechanism, we suggest a therapy associating anti-histaminic substances which block H1 and H2 receptors and an agent which inhibits mast cell degranulation.

[Chronic neutropenia and Crohn's disease in childhood. Report of 2 cases ]. / Neutropénie primitive et congénitale et maladie de Crohn. A propos de deux observations chez l'enfant.

Two children in whom Crohn's disease complicated the course of chronic neutropenia are reported. These two cases had other features in common: consanguinity of the parents, relatively good clinical tolerance of neutropenia and almost complete lack of blood and marrow eosinophils. The possible relationships between the 2 diseases are discussed.

[Glucagon-induced neutrophil mobilization (author's transl)]. / Mobilisation des neutrophiles induite par le glucagon.

In 19 normal subjects an increase in the number of circulating neutrophils was observed after intramuscular injection of 1 mg glucagon. The response began at the end of the 1st hour following the injection and persisted beyond the 8th hour, with a peak between 2 and 5 hours. No response was obtained in patients with bone marrow aplasia, either primary or associated with acute leukaemia. In 20 patients with chronic primary neutropenia, the degree of response was proportional to the percentage of medullary polymorphonuclears. A comparison between the kinetics of the glucagon-induced granulocyte response and that of the response induced by other neutrophil mobilizing agents suggested that glucagon acts by releasing granulocytes from the bone marrow reserve compartment. The finding that an infusion of dibutyryl cyclic AMP results in granulocyte mobilization suggests that the effects of glucagon are mediated by cAMP at cell level. Since the glucagon response test is harmless and gives rapid and pronounced results, it may be useful in investigation of patients with neutropenia. In addition, the glucagon-induced granulocyte mobilization might improve leucocyte yield in blood donors used for transfusion in agranulocytosis.

[Immunologic study of familial lymphohistiocytosis. Eight new case reports (author's transl)]. / Etude immunologique de la lymphohistiocytose familiale. A propos de huit nouvelles observations.

We report 8 cases of familial lymphohistiocytosis collected in 6 families. Several data argue for an hyperactivation of the reticuloendothelial system (RES). An abnormal visualization of all organs was observed in a scintigraphic study after 99technetium labelled red blood cells injection. Blood monocytes contained very low peroxidase activity as detected by cytoenzymology and secreted large quantities of prostaglandin E2 (PGE2). Adherent cells isolated from blood exercised a strong suppressor effect on the proliferation of normal lymphocytes induced by phytohemaglutinin. This effect was reduced by indomethacin and therefore appears PGE2-dependent. One patient's serum exerced an inhibitory activity on antigen-induced proliferation of normal lymphocytes and on mixed leucocyte reaction. In contrast, cellular and humoral functions were not deeply impaired. The hyperactivation of the RES remains unexplained and not related to a graft versus host reaction, which could be excluded in 3 of our patients. Therapeutic attempts were not efficient, all patients dying despite steroid, vincaleucoblastin and indomethacin therapy.

The cell cycle of lymphocytes in Fanconi anemia.

BrdU-incorporation techniques were used to study the cell cycle in 18 cases of Fanconi's anemia (FA). By comparison with controls, a significant slowing of the cell cycle of lymphocytes in vitro was observed in all FA patients, and possibly in FA heterozygotes, although to a lesser degree. It is probable that the demonstration of the slowing is dependent on the culture conditions. No slowing was observed in other patients affected by at least one of the symptoms of FA. The slow cell cycle of FA cells is mostly due to a very long G2-phase. A relationship between slow cell cycle and chromatid anomalies exists, the slower cells being significantly more frequently carriers of radial figures than the faster cells, in the same patient.

[Acute lymphoblastic leukemia in childhood: importance of sex as a prognostic factor (author's transl)]. / Leucémie aiguë lymphoblastique de l'enfant. Importance du sexe parmi les facteurs de pronostic.

In order to appreciate their prognostic value in acute lymphoblastic leukemia (ALL) of childhood, the main initial clinical and biological features have been analysed in 63 patients. In all cases, follow-up was longer than 3 years; in 43 it was longer than 5 years. The survival rate in first remission was 52% after 3 years and 30% after 5 years. No relapse was observed after 5 years. In this series, the presence of very high leukocyte count (less than 30 x 10 9/l or 30,000/mm3) and sex were the only significant prognostic factors. A poorer prognosis of ALL was indeed present in boys. The sex-related difference is independent from other prognostic factors and may require special attention.