Sickle-cell disease in pregnant immigrants.

Introduction The clinical features of sickle-cell disease have been recognized for many years, the disease being described by Herrick (1910), later Sydenstricker (1924) reported on a large series of cases. In 1931, Yater & Mollari described a case with fatal outcome; Kobak, Stein & Daro (1941) made a detailed study of pregnancy in association with sickle-cell disease. The demonstration by Pauling et al. (1949) of an abnormal haemoglobin in the erythrocytes of patients with the disease led rapidly to the identification of additional haemoglobin variants and the introduction of filter-paper electrophoresis by Spaet (1953) allowed of identification of abnormal haemoglobins in general hospital practice, so allowing separation of cases which may previously have been included as sickle-cell disease but which were, in fact, cases of sickle-cell/haemoglobin C disease or cases of sickle-cell/thalassaemia. An increasing number of papers has since appeared dealing with the problem in the pregnant woman (Adams, Whitacre & Diggs, 1953; Eisenstein, Posner & Friedman, 1956; Curtis, 1959; Abrams & Schwartz, 1959; Anderson et al., 1960). These papers have described cases occurring in the United States of America and the West Indies and to date there has been little information concerning cases in the United Kingdom (Reiss, 1962; Apthorp, Measday & Lehmann, 1963). Routine ante-natal haemoglobin electrophoresis screening of negro women has been reported from the United States of America (Curtis, 1959; Cotter & Prystowsky, 1963), from the West Indies (Anderson et al., 1960) and from the United Kingdom (Buckle, Hanning & Holman, 1964). The results obtained from such screening show a comparable incidence of cases of sicklecell disease, sickle-cell/haemoglobin C disease and sickle-cell/thalassaemia, an incidence of 0 18% for these three conditions being reported by Buckle et al. (1964) in immigrants into the United Kingdom from West Africa, the West Indies and the Mediterranean littoral. The conditions will, therefore, be infrequently encountered in association with pregnancy and individual experience in management necessarily limited. In the present paper, the course, outcome and management of thirteen pregnancies in patients with sickle-cell anaemia or variants will be described.