NEUTROPHIL LIFE-SPAN IN PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

Abstract

The neutrophil intravascular life span was studied in 6 patients with paroxysmal nocturnal hemoglobinuria (PNH); 4 had normal neutrophil counts when studied and 2 were neutropenic. Five patients had enough circulating neutrophils to isolate for tests in vitro. Lysis of labeled neutrophils was greatly increased, compared to that of normal volunteers, when these neutrophils were incubated with acidified fresh serum as a source of active complement plus serum containing antineutrophil antibodies (from 3 different sources). Despite the in vitro lesion, each of these patients had a normal neutrophil intravascular life span as measured by the 32P-diisopropylfluorophosphate technique. One neutropenic patient, who had a normal neutrophil life span, had a shift of cells from the circulating to marginated pool of sufficient degree to cause the neutropenia. A 2nd (severely) neutropenic patient developed extreme marrow hypoplasia, also explaining the neutropenia. In contrast to the shortened red cell life span, a shortened neutrophil life span was not found in PNH.