Hypoplastic Anaemia in Infancy and Childhood: Erythroid Hypoplasia

Abstract

In 1936, Josephs reported 'a hypoplastic or aplastic type of anaemia confined to a failure of erythropoiesis' in two children. 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 (

Endocrine Studies in a Case of Congenital (Erythroid) Hypoplastic Anaemia

“…The association of dwarfism and sexual infantilism with congenital (erythroid) hypoplastic anaemia has been taken as evidence that the un derlying (presumably biochemical) lesion is a multisystem one, not con- fined to the red cell precursors of the bone marrow [8]. In the present case, absence of virilisation in response to androgens may be most easily explained by this interpretation but until the fundamental chemistry of the condition is better understood this hypothesis cannot be proved.…”

A case of red cell aplasia in a Negro child.

“…Of 30 cases reported by Diamond et al (1961), 2 of the mothers had been receiving drugs, one stilboestrol and the other thyroid, throughout pregnancy. The mother of one of the eight cases described by Hughes (1961) received chlorothiazide and reserpine. Chlorothiazide can cause thrombocytopenia (Nordqvist, Cramer, and Bjorntorp, 1959), but Lucey (1961) does not report blood dyscrasias following reserpine or stilboestrol.…”

Bone‐Marrow Depression in Childhood

“…Before corticosteroid therapy was used spontaneous remissions occurred in about one-third of patients (Diamond, Allen and McGill, 1961), while the remainder were doomed to a life of repeated transfusions with the complications of iron overload. In 1951 Gasser demonstrated the efficacy of corticosteroids for this disease and steroid therapy is effective in inducing remissions in over 50% of patients (Allen and Diamond, 1961;O'Gorman Hughes, 1961).…”

“…Associated moderate or intermittent neutropenia or thrombocytopenia did not exclude the patients. These features were discussed in a previous paper where eight cases were described (O'Gorman Hughes, 1961). Of the acquired types of erythroid hypoplasia, patients with haemopoietic toxicity attributed to chloramphenicol are described separately in Chapter 9.…”