Chronic hemolytic anemia with erythrocyte fragility to cold and acid. II. Serum hemolytic activity and its relation to serum globulins and complement and the role of guinea pig serum.

By S. H. Liu, M.D. T HE AIM of this paper is to report 2 cases of severe anemia which were thought to belong to the group of aplastic or “refractory” anemias, frequently seen in this locality’ and recently restudied in the United States by Rhoads.2 However, the history of passage of (lark urine in one of the cases le(l to examinations for paroxysmal cold hemoglobinuria (Donath-Landsteiner) and for paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli). While the erythrocytes of this patient were found to be abnormally susceptible to hemolysis with chilling and on addition of acid, the diagnosis of either one or the other type of hemoglohinuria could not be properly made. Following the experience in this case, all the patients with peculiar or “refractory” anemia that were available at the time were similarly studied with the result that another case possessing the same abnormality of the red blood cells was found. The hemolytic nature of the anemia in these 2 patients was not evident on ordinary examination, but the demonstration of excessive hemoglohinemia in the serum and large iron excretion in the urine in both cases and finally the heavy hemosiderin deposits in the organs at autopsy in one case pointed to the presence of an abnormal in vivo hemolysis. The present report deals with the clinical data of the two patients together with the autopsy findings in one. The laboratory investigations characterizing the abnormality of the erythrocytes and the results of chilling and administering acid to the patients are included herewith. The studies concerning the serum factor necessary for hemolysis are reported in a companion paper.