Refractory Normoblastic Anaemia: A Clinical and Haematological Study of Seven Cases

Iron Deficiency Anemia Associated with an Error of Iron Metabolism in Two Siblings *

“…Indeed, the anemia observed in these children is due to iron deficiency of the bone marrow, but the associated elevation of serum iron concentration and massive hepatic iron deposition are unique features that considerably complicate the problem. These two children differ markedly from patients with refractory hypochromic hypersideremic anemia whose case histories have been reported to date (23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38). Three major functions (iron stores, erythrocyte life span, and kinetics of Fe59 utilization) distinguish hypersideremic anemia from the present cases.…”

“…Thus, in previously studied cases (25,(27)(28)(29)(30)34), the calculated percentage of uptake of Fe59 by red cells, 10 days after iv administration of radioiron, varied from 5 to 36%o. Of this reduced Fe59 incorporation, an even smaller fraction may actually be located in the hemoglobin.…”

Hypoplastic Anaemia in Infancy and Childhood: Erythroid Hypoplasia

“…It has been suggested (Gasser, 1957;Smith, 1959) that steroid therapy is of little avail when transfusion haemosiderosis has developed. Only one of the present group of patients could be considered unduly refractory to treatment (Case 2), although only two patients (Cases 2 and 3) had received many blood transfusions before steroid therapy was tried.…”

“…Diamond and Blackfan (1938) gave a more detailed description of the disorder in four children, and emphasized the features of a chronic progressive anaemia beginning early in infancy, with a tendency to moderate depression of the leucocyte and thrombocyte counts, and selective hypoplasia of the red cell precursors in the bone marrow. Several reports of this disease have appeared since (Rinvik, 1940;Kohlbry, 1941;H0yer, 1942;Rubell, 1942;Robson and Sweeney, 1948;Smith, 1949Smith, , 1953Smith, , 1959Palmen and Vahlquist, 1950;Cathie, 1950; Lelong, Joseph, Polonow-ski, Desmonts and Colin, 1951; Anderson, 1952;Donnelly, 1953;Aldridge and Kidd, 1953; Kass and Sundal, 1953;Verger and Leger, 1953;Fisher and Allen, 1953; Arrowsmith, Burris and Segaloff, 1953;Burgert, Kennedy and Pease, 1954;Diamond, 1954;Harper and Geikie, 1955;Calvert and Robson, 1956;Sundal, 1956;Pearson and Cone, 1957;Gasser, 1957), and a total of about 70 cases have been recorded. The reports have conformed to the original descriptions, with the exception that marrow examinations in some instances have revealed normal or increased numbers of normoblasts (Cathie, 1950).…”

Primary or Essential Sideroachrestic Anaemias

“…Alkaline phosphatase concentration of leucocytes in the blood**: A few investigators have pointed out a low alkaline phosphatase activity in the segmented leucocytes in patients with sideroachrestic anaemia (8,15). In our 13 patients, blood smears were stained for alkaline phosphatase.…”