Two populations of erythroid cell progenitors in paroxysmal nocturnal
hemoglobinuria
B Rotoli, R Robledo, N Scarpato and L Luzzatto
We have grown erythroid cell colonies from two patients with paroxysmal
nocturnal hemoglobinuria (PNH). At 11 to 13 days, individual bursts were
picked and incubated for 24 hours with 3H-leucine in order to label total
cell protein (mainly hemoglobin). After appropriate washing, each burst was
subjected to a miniaturized acidified serum test, and lysis was measured by
the release of radioactivity. In bursts from normal controls, lysis was 19%
+/- 13% SD. By contrast, of 58 bursts from PNH patients, 14 had lysis
similar to that of controls (mean 15.4% +/- 10.6%), while 44 had lysis
ranging from 42.2% to 85.8% (mean 70.3% +/- 10.4%). Colonies sensitive to
acidified serum were acetylcholinesterase (AchE) negative, whereas normal
colonies were AchE- positive. Thus, based on two independent criteria, a
dual population of erythroid burst-forming units (BFU-E) can be
demonstrated in PNH. These data confirm directly the somatic mutation model
of the pathogenesis of PNH, and by these methods the relative sizes of the
normal and the PNH cell populations can be measured at the level of the
erythroid cell precursors.
Volume 64,
Issue 4,
pp. 847-851,
10/01/1984
Copyright © 1984 by The American Society of Hematology
