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

The immunological analysis of the membrane phenotype of the leukemic cells, by studies of various markers and antigens unmasks some degree of heterogeneity of chronic lymphocytic leukemias and of acute lymphoblastic leukemias. This analysis gives indications of the nature and origin of the proliferating cells. The analysis gives indications of the nature and origin of the proliferating cells. The data provided by these studies are useful for a modern classification of these diseases and allow new nosologic groupings.

The biological syndrome in von Willebrand's disease is presently defined by four parameters: bleeding time, factor VIII coagulant activity (VIII:C), Willebrand factor activity (VIIR:WF) and factor VIII related antigen (VIIR:AG). The study of 48 patients from 25 families confirms the heterogeneity of the von Willebrand's syndrome including both quantitative as well as qualitative defects. Classical von Willebrand's disease results from the quantitative decrease of a plasma protein named "Willebrand factor" which is the primary gene-product of an altosomal chromosome. Both severe and moderate forms can be observed. In very severe forms, the apparent lack of the VIIIR:AG as measured by an immunoradiometric assay suggest the possibility that some patients are homozygous. "Variants" of von Willebrand's disease have been recently described in some patients, based on the discrepancy between the observed subnormal levels of VIIIR:AG and low levels of VIIIR:WF. The qualitative abnormality of the factor VIII/Willebrand protein can be assessed by different methods such as double cross immuno-electrophoresis, or immunoradiometric assay.

The concept of geographical haematology described by Jean Bernard in 1965-1966, found a remarkable application field in Anthropology. The human ecology which allows to measure the influence of environmental factors in man through some haematological variations, has been added to the genetics of human populations, revealed by the blood groups discovery. It has been now demonstrated that, in man, cultural or sociological features can act a genetical isolation more effective that natural boundaries. So, an ethnological haematology is superimposed on the geographical haematology of which it can modify outlines.

90 patients with hemopoietic dysplasia (preleukemia) have been studied with iron kinetics for the mechanism of the anaemia, 23 patients had a bone marrow autoradiography and 18 a bone marrow culture in semi-solid medium. The death was caused in half the cases by acute myeloblastic leukaemia transformation (LAM) and in half the cases by complications of pancytopenia (infection, haemorrhage) or hemochromatosis. Three data give prognostic factors at short or long term: the bone marrow hypoplasia, no patient with major bone marrow hypoplasia (ratio of 59Fe fixation in liver and sacrum of more than 2) lived more than 2 years after the examination. The low labeling index of myeloblasts and promyelocytes, the mean LI is 0.20 patients having lived less than one year after the study and 0.35 for those who lived more than two years. The bone marrow culture of the macroclusters type, no patient whose bone marrow grew with macroclusters and no colonies survived more than 8 months after the study. These three data seem to be essential in the regular survey of the patients with hemopoïetic dysplasia.