[Transfusion in sickle cell anemia].

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.

[Establishment of a range of phtalate density for studying profiles of erythrocyte density].

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.

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].

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.

[Immune deficiencies in nutritional anemias].

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.

[Asplenia: a study of 21 subjects splenectomized during childhood for spleen injury (author's transl)].

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.