A case of severe congenital anaemia (Hb levels 3–4 g/dl) is presented. The patient, a 21-year-old female, requires, since the age of two, periodical blood transfusions, but is nevertheless able to lead a normal life as a housewife. Although the case has to be considered as a form of congenital hypoplastic disorder of the erythroid tissue, some of the clinical and haematological features are compatible with a congenital dyserythropoietic state. On the basis of clinical findings it is suggested that it may represent a previously undescribed form of anaemia which shares the features of both hypoplastic and dysplastic congenital affections of erythropoiesis

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