International Journal of Cell Cloning, Vol 2, 316-326, Copyright © 1984 by AlphaMed Press
Complement sensitivity of erythroid and myeloid precursors in paroxysmal in nocturnal hemoglobinuria
Y Ishida, N Matsumoto, K Shinohara, K Yamada, M Inoue, T Kaneko and MJ Murphy Jr
To test the hypothesis that paroxysmal nocturnal hemoglobinuria (PNH) is a
hematopoietic stem cell disorder, the growth of BFU-e and CFU-gm and the
complement sensitivity of cultured cells from BFU-e and CFU-gm colonies, as
well as of unipotential progenitor cells (CFU-gm and BFU- e), were examined
in five PNH patients. BFU-e growth was reduced in the three patients
examined, and poor CFU-gm growth was noted in three of the five patients.
Compared to normals, BFU-e and CFU-gm colonies in all patients demonstrated
an increased susceptibility to the lytic action of complement when the
release of 59Fe and myeloperoxidase was measured as specific markers for
monitoring membrane damage. Compared to the growth of normal bone marrow
cells, CFU-gm growth was significantly inhibited by pretreatment of bone
marrow mononuclear cells with monoclonal OKIal antibody and complement.
These findings support the proposition that a membrane defect predisposing
blood cells to complement-mediated lysis may occur at the level of
unipotential progenitor cells.
