Dehydrated hereditary stomatocytosis (DHS), also known as 'hereditary xerocytosis', is a rare form of hereditary haemolytic anaemia with autosomal dominant transmission. This genetic disorder is provoked by a red cell membranopathy, leading to a well-compensated haemolytic anaemia. Approximately 30 families with DHS were described in the literature (Carella et al. 1998), but the exact prevalence is not yet known (Beaurain et al. 2007). Rare patients with DHS develop prenatal oedema. It resolves spontaneously during the last weeks of gestation or within the weeks following birth (Grootenboer et al. 2001). Here, we report a case of DHS associated with recurrent fetal ascites in two successive pregnancies. The mother showed haemolytic anaemia. Compensated hyperhaemolysis, high reticulocytosis, macrocytosis, presence of stomatocytes and gradient ektacytometric curves led to the diagnosis of DHS. Fetal ascites in the two pregnancies resolved spontaneously.
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