Abstract

Erythrokinetic and red cell survival studies were performed in ten patients with macroglobulinemia. Inadequate red cell synthesis, decreased erythrocyte survival and iron deficiency, alone or in combination, were responsible for anemia in eight of these patients. In six patients there was a relative failure of red cell production with inadequate compensation for excessive red cell destruction. In two patients there was an absolute failure of erythropoiesis with functional aplasia. No simple correlation between rate of erythropoiesis and serum gamma macroglobulin concentration was evident. Shortened erythrocyte life span was found in eight of the ten patients and was most prominent in those with active disease. Serial studies before and after plasmapheresis in two patients showed that reduction of serum viscosity and macroglobulin level was associated with increased red cell survival. Causes of shortened erythrocyte survival included bleeding disorders, Coombs' positive hemolytic anemia and erythrocyte sequestration by the spleen. Two patients had advanced iron deficiency and four had evidence of early iron deficiency. Depletion of body iron stores usually could be related to external blood loss. The typical features of iron depletion were present when iron deficiency was advanced. Therapy of anemia in these patients consisted of plasmapheresis with or without chemotherapy for disease manifestations associated with high serum viscosity, corticosteroids when immune hemolysis was demonstrable, and iron for iron deficiency.

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