[Transfusion in sickle cell anemia]. / La transfusion dans la drépanocytose.

Transfusion is one of the fundamental treatments in complications of sickle cell anaemia, a disease with peculiar features requiring an appropriate transfusion policy. Owing to the higher risk of vascular occlusion it carries, simple blood transfusion is indicated only for acute anaemia and for the very rare long-term transfusion programmes. In patients with severe occlusive and/or septic accidents, the risk of decompensation makes it mandatory to promptly reduce the sickle cell concentration; this is achieved by exchange transfusion the modalities of which are described by the authors. General anaesthesia also requires exchange transfusion in volumes that depend on the risk incurred. Finally, some patients benefit from a long-term transfusion programme. Potentially repeatable transfusions imply the use of phenotypes and leucocyte-freed red cell concentrates as well as detection and prevention of viral infections transmitted by transfusion. This article summarizes the recommendations that can now be made concerning the use of perfusion in the management of sickle cell anaemia.

[Establishment of a range of phtalate density for studying profiles of erythrocyte density]. / Etablissement d'une gamme de densité de phtalate pour l'étude des profils de densité érythrocytaire.

The determination of the density distribution of erythrocyte population is one of the biological parameters used in several hemoglobin disorders. Many difficulties to obtain reproducible results between different laboratories lead to make a reference phtalate ester range for two hospitals. In this paper, the conditions of preparation and use of the method are described. Then, factors affecting determinations are discussed. Finally the authors give reference values in child and adult. This work forms the first stage of a standardized study of erythrocyte populations with hemoglobinopathies.

An acute leukaemia augured before clinical signs by blood group antigen abnormalities and low levels of A and H blood group transferase activities in erythrocytes.

A mixed field agglutination pattern with anti-A reagents and very low levels of A and H blood group antigen specific transferase activities were found in the erythrocytes of a 4-year-old girl who presented no clinical signs of haematological disease. Blood and marrow examination displayed some features consistent with a moderate dysmyelopoietic state. 18 months later an acute myeloblastic leukaemia confirmed the suspected haematological malignancy.

[Sickle-cell cardiomegaly in children. Study of 13 cases]. / Les cardiomégalies drépanocytaires de l'enfant. Etude de 13 observations.

Thirteen children, aged 18 months to 14 years and presenting with sickle cell anemia and cardiomegaly (Cardiothoracic ratio greater than or equal to 0.55) were investigated by echocardiography and radio-isotopic measurement of the cardiac output. Eight children presented with one or several criteria of anemic cardiopathy: left ventricular dilatation and hyperkinesia, increased cardiac index. On the contrary, 2 children presented with left ventricular dilatation and decreased contractility, without increase in the cardiac index; 3 children presented with echocardiographic findings similar to those of controls and a normal cardiac index, which is unusual in cases with chronic anemia. These results show that the cardiovascular system of children with sickle cell anemia does not always behave as one would expect in chronic anemia. They also suggest the possible early occurrence of cardiomyopathy, as already described in adults with sickle cell anemia.

[Immune deficiencies in nutritional anemias]. / Déficits immunitaires au cours des anémies nutritionnelles.

A transient cellular immunologic defect caused by folic acid deficiency was seen in a goat-milk-fed infant with severe enterocolitis. Data on the immunologic consequences of folic acid, protein and iron deficiencies were reviewed in the medical literature. Investigations are difficult because of the patients' poor general condition. Results are difficult to interpret as many etiologic factors are often combined and mechanisms of immunologic responses are complex. Attention is drawn to the danger of iron therapy in patients with transferrin deficiency.

[Asplenia: a study of 21 subjects splenectomized during childhood for spleen injury (author's transl)]. / L'asplénie. Etude de 21 sujets splénectomisés dans l'enfance pour traumatisme.

Clinical and biological studies were carried out in 21 subjects one to twenty-three years after the injured spleen had been removed. Asplenia did not give rise to clinical symptoms, did not facilitate infections and had no effect on growth. Laboratory investigations revealed a decrease in IgM in 6 subjects and minor quantitative and qualitative platelet abnormalities. The absence of Jolly's bodies in 7 subjects was suggestive of spleen regeneration, which was confirmed by scintigraphy in 2 cases. The significance of these findings in discussed.