CLINICAL-FEATURES AND SPLENIC PATHOLOGICAL-CHANGES IN PATIENTS WITH AUTOIMMUNE HEMOLYTIC-ANEMIA AND CONGENITAL HEMOLYTIC-ANEMIA

We characterized the pathologic changes in 50 spleens from patients wi th autoimmune hemolytic anemia and in 13 spleens from patients with co ngenital hemolytic anemia. The major pathologic findings in autoimmune hemolytic anemia were mild to pronounced cord congestion and variable white pulp area, erythrophagocytosis in conjunction with an increased polymorphonuclear neutrophil reaction, and increased deposition of he mosiderin and extramedullary hematopoiesis. In contrast, both the seve rity and the frequency of polymorphonuclear neutrophil reactions, depo sition of hemosiderin, and extramedullary hematopoiesis were less in p atients with congenital hemolytic anemia, and prominent cord congestio n associated with an empty or collapsed sinus was noted in patients wi th hereditary spherocytosis. The activity of erythrophagocytosis in th e sinus was more distinct in patients with congenital hemolytic anemia , especially those with nonspherocytic congenital hemolytic anemia, th an in patients with autoimmune hemolytic anemia. These findings are co nsistent with the existence of different mechanisms of trapping and de struction of erythrocytes in autoimmune hemolytic anemia and congenita l hemolytic anemia.