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[The preliminary investigation on proliferative and survival capabilities of normal and abnormal mononuclear cells in bone marrow of patients with paroxysmal nocturnal hemoglobinuria].

OBJECTIVE
To unfold the proliferation and survival behavior of normal and abnormal early hematopoietic cells in paroxysmal nocturnal hemoglobinuria(PNH).


METHODS
The bone marrow mononuclear cells (BMMNC) from 6 patients with PNH were separated into two subpopulations using CD59 monoclonal antibody combined with goat-anti-mouse IgG immunomagnetic beads, and then cultured in vitro.


RESULTS
The BMMNCs from normal control had better proliferation capacity and stronger potential of suvival than both CD59(+) and CD59(-) BMMNCs from PNH patients. The CD59(+) BMMNCs from PNH patients, which should be expected "normal" in view of the CD59 expression, were surprisingly found to have less capabilities to form CFU-GM and BFU-E in semisolid cultures and less abilities of expansion and survival in liquid cultures than their CD59(-) counterparts. The CD59 expression of CD59(+) BMMNC in PNH patients diminished to some extent after a period of liquid culture while the normal control remained unchanged indicating that some of CD59 antigen on the cells were gradually lost during cultivation.


CONCLUSION
In all cases of PNH studied, CD59(-) BMMNCs dominated hemopoiesis, and CD59(+) BMMNCs seems to have less potential to proliferate. The emergence of abnormal CD59(-) cells could not only result from PIG-A gene mutation but might also arise as the consequence of loss of GPI-anchored protein during the course of hematopoietic development and differentiation.