Summary The red blood picture was studied in detail from birth to 12 weeks. The low point in the red cell count was reached in the seventh week, which represented the turning point from a negative to a positive balance between cell production and destruction. From the dynamics of the total number of circulating red cells it was concluded that, although erythropoiesis slows down after birth, a considerable amount of red cell production takes place during the first few postnatal weeks. The MCHC increased significantly over the first 5–6 postnatal weeks and remained constant thereafter. Macrocytes predominated at birth. Their percentage distribution decreased after the second week with a corresponding increase in microcytes. The red cell density distribution curve in the neonatal period is sigmoid in shape. The range of densities is, however, wider than in the adult. The curve shifts gradually to the heavy side from birth to 5 weeks, while from 5 to 12 weeks a shift back to the light side is observed. Macrocytes preponderate in the light fractions.

The incidence and severity of osteolytic bone changes in patients with chronic (Type I) Gaucher disease splenectomized in the first decade of life were compared to those in patients of the same age group and similar degree of severity of the disease in whom the spleen remained intact at least until the second half of the second decade. The size of the spleen, measured by palpation, was used as an index of severity. In the splenectomized group osteolytic changes appeared within a few months following splenectomy in six out of eight cases. The changes were severe in five cases and moderate in one. In contrast, in the non-splenectomized group, evidence of bone destruction was found in two out of eight patients and classified as mild in both cases. Furthermore, in three patients in this group, who remained free of bone destruction until splenectomy in the second half of the second decade, osteolytic lesions appeared soon after the operation. Children with chronic Gaucher disease can be spared a great deal of suffering caused by bone disease, if splenectomy is avoided or postponed as far as possible.

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