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The appearance of schistocytes in a peripheral blood film is considered to be an important diagnostic marker for thrombotic microangiopathy. However, the morphological analysis of schistocytes remains uneasy. To determine practice patterns in the biological management of schistocytosis, the French Group of Cellular Hematology from the French Society of Hematology conducted a survey on the approach of the diagnosis of microangiopathy. A guideline is proposed in order to cancel the substantial variation among biologists.

First described in 1948, Bernard-Soulier syndrome is an uncommon hereditary thrombopathy characterized by abnormal expression of the GPIb-IX-V complex which inhibits platelet migration to the site of endothelial trauma. Our case illustrates the pathophysiological mechanisms involved and points out the similarity with idiopathic thrombopenic purpura.

Aims: To compare by a prospective study in high risk polycythemia vera (PV) patients 33P alone and 32P followed by low-dose hydroxyurea (HU) maintenance therapy. Toxicity, efficiency, and leukemogenic potential were studied., Patients: 483 patients with a documented PV, aged more than 65 years at diagnosis, were included between 1980 and 1996 in a prospective study comparing 32P alone and 32P followed by low-dose HU maintenance therapy. Blood cell counts were performed every two months and a clinical evaluation by a specialist was obtained every four or six months., Results: Treatments were well tolerated, but chronic leg ulcers were observed in the maintenance therapy arm. The risk of leukemia was about 15% at the 15th year in the group of patients treated by 32P alone, but reached 30% in the group receiving maintenance therapy. In both arms, there was no significant correlation between occurrence of leukemia and the total dose of 32P. There was a correlation between the leukemic risk and disease severity, estimated on the frequency of relapse. Cancer occurrence was slightly higher than expected in the maintenance arm. HU treatment did not protect against progression to myelofibrosis, probably due to the lack of maintenance of an efficient myeloid or megakaryocytic suppression. Median life-span was slightly shorter in the group receiving HU maintenance. In all cases, life-span was only one year lower than that observed in the reference population., Conclusion: For all these reasons, we suggest the us of 32P alone in elderly patients; complementary chemotherapy should only be prescribed in the cases with short-term relapse, and late resistance to 32P.

In four members of a family presenting hereditary elliptocytosis erythrocytes were studied, membranes were extracted and proteins were analysed by gel electrophoresis in a polyacrylamide gradient. In one of the patients suffering from severe haemolytic anaemia successfully treated by splenectomy, an almost complete deficiency in band 4(1) was discovered. Endogenous protein kinase activities revealed the absence of radioactivity of band 4(1) in the proband, a result not modified by cAMP. The kinase activity was normal in the parents, which confirms the almost complete absence of protein 4(1) seen in the stained gel of the proband. A moderate increase in the phosphorylation of band 3 was observed in all the members of this family. Deformability was measured by a visco-diffractometric method (Ektacytometry) in a medium of low viscosity (11 cp at 22 degrees C), allowing the recording of curves characteristic of elliptocytosis and revealing a markedly reduced deformability index (DI). At low shear stress, elliptocytes were oriented perpendicular to the flow (a result common to all elliptocytosis). In the proband several years after splenectomy, the DI was extremely reduced at high shear stress, which can be explained by the simultaneous presence of elliptocytes, schizocytes and spherocytes. A more detailed comparative study of the proteins of the various members of the family could lead to more precise information on the possible role of band 4(1) as a linkage protein maintaining the erythrocyte membrane stability.

The presence of vacuolated polymorphonuclear neutrophils in blood smears of patients suffering from infection appears to be associated with massive bacterial growth and to constitute a very early symptom of rapidly life-threatening septicaemia. When these cells persist for more than 36 hours, the disease may be considered as beyond control.

Prostaglandins, thromboxanes and leukotrienes are oxygen metabolites of arachidonic acid forming a family of lipidic substances with intrinsic biological activities. The significance of biosynthesis of these mediators in response to cell stimulation remains unclear. Numerous data suggest that these compounds, produced by blood cells and vascular cells, play an important role in cardiovascular pathology, allergy or inflammation. The concept, based on biochemistry, of distinct metabolic pathways should be reappraised, using a physiological approach that involves a biological effect resulting from a synergistic action of different compounds. The development of specific assay methods will permit to investigate compounds obtained from biological fluids or from cells present in recognized pathological situations. Such in vitro data should help to clarify the role of these autacoids in cardiovascular, allergic or inflammatory diseases.

Two cases of Henoch-Schönlein purpura in adults are reported. Both patients presented with severe digestive symptoms suggesting malabsorption or exudative enteropathy. Roentgenological examinations showed marked anomalies of the small intestine as well as changes suggestive in intra-mural hematoma located in the stomach in one case and in the duodenum in the other. Hypoalbuminemia occurred in both patients during the course of the disease and was highly suggestive of protein-loosing enteropathy. Disseminated intravascular coagulation was seen in one patient. Both patients recovered from their gastrointestinal disease. Roentgenologic changes disappeared in one case; in the other, marked compression and distension of the duodenum were still visible six months after onset.