Cortisone Therapy in Erythrogenesis

A rare but distinctive condition, which may be called erythrogenesis imperfecta (Cathie, 1950) or infantile pure red-cell anaemia, is gaining recognition. The nomenclature is, however, confusing. For example, some cases have been reported in later childhood, rendering inappropriate the word 'infantile'. Again, the varied titles illustrate the confusion of terminology. In the widest sense, i.e., without special reference to the infantile type, this erythropoietic disorder has been denoted by erythrophthisis (Kaznelson, 1922), pure red-cell anaemia (Lescher and Hubble, 1932), congenital hypoplastic anaemia (Diamond and Blackfan, 1938), chronic congenital aregenerative anaemia (Smith, 1949), chronic idiopathic erythroblastopenia (Gasser, 1949), erythrogenesis imperfecta (Cathie, 1950) and anerythrocytogenic anaemia (Loeb, 1951). Over 30 cases of infantile type (referred to here as erythrogenesis imperfecta) are on record (Josephs, 1936; Diamond and Blackfan, 1938; Kohlbry, 1941; H0yer, 1942; Rubell, 1942; Robson and Sweeney, 1948; Smith, 1949; Cathie, 1950; Lelong, Joseph Polonowski, Desmonts and Colin, 1951; Anderson, 1952; Donelly, 1953; Aldridge and Kidd, 1953; Kass and Sundal, 1953; Verger and Liger, 1953; Fisher and Allen, 1953; Burgert, Kennedy and Pease, 1954; Bernard, 1954). These descriptions monotonously reiterate that hitherto the only treatment capable of prolonging life was regular, and usually frequent, blood transfusion. The case which we refer to here has been fully described (Robson and Sweeney, 1948) from the clinical and haematological aspects. The ineffectiveness of iron and liver preparations was then emphasized, while the life-prolonging effect of frequent blood transfusions was graphically illustrated. More recently, Coles (1955) reported on the failure of cobaltous chloride to produce a haematological remission in this child. A dose of 50 mg. daily was used for a fortnight and followed immediately by 100 mg. daily for a similar period. During