Abstract
We studied to clarify the mechanism of hemolysis with the use of 51Cr-labeled red cells and ferrokinetics, in 7 patients with hereditary spherocytosis (HS) and in 3 patients with abnormal pyruvate kinase (PK) deficiency, in pre-and post-splenectomy. In HS patients it was observed that reticulocyte counts increased, the red cell life span was reduced, and that there was an accumulation of 51Cr-labeled red cells in the spleen before splenectomy. The red cell life span returned nearly to normal after splenectomy. HS patients had levels of hemoglobin and reticulocyte counts within normal range. But in PK-deficient patients it was found that reticulocyte counts increased, the red cell life span was short or subnormal, and that abnormal PK-deficient reticulocyte was sequestrated and destroyed in the spleen and the liver before splenectomy. The red cell life span was reduced and its survival curve was bimodal (that is, short-and long-lived erythrocytes) after splenectomy. Reticulocyte count conversely increased and the level of hemoglobin was higher 1 to 2 g/dl than pre-splenectomy level. It was thought that PK-deficient patients with splenectomy had short-lived red cells of two groups. In one group, when reticulocytes reach matured red cells, those cells lost mitochondria, could not produce ATP, and were sequestrated and destroyed regularly in the liver and the bone marrow. In the other group, at the reticulocyte stage it is sequestrated and destroyed at random in the liver and the bone marrow.
