Different distribution of decay-accelerating factor on hematopoietic
progenitors from normal individuals and patients with paroxysmal nocturnal
hemoglobinuria
A Kanamaru, K Okuda, E Ueda, T Kitani, T Kinoshita and K Nagai
2nd Department of Internal Medicine, Hyogo College of Medicine, Japan.
Deficiency of decay-accelerating factor (DAF) occurs in blood cells in
paroxysmal nocturnal hemoglobinuria (PNH), characterized by an unusual
susceptibility to hemolysis by complement activation. This study examined
DAF expression on hematopoietic progenitors from normal individuals and PNH
patients using a fluorescence-activated cell sorter (FACS) with monoclonal
antibodies to DAF. Nonphagocytic mononuclear marrow cells expressing
different density distributions of DAF were sorted into DAF-, DAF+/-, DAF+,
and DAF++ fractions. The cells from each fraction were cultured in
methylcellulose and assayed for CFU-E, BFU-E, CFU-GM, and CFU-Mix. The
percentages of distribution of DAF- negative normal progenitors increased
in the order of CFU-E, CFU-GM, BFU-E, and CFU-Mix, whereas those of
DAF-positive cells inversely decreased in this order. These results
indicate that DAF expression may accompany differentiation from CFU-Mix to
CFU-E. On the other hand, most progenitors in PNH patients had little, if
any, expression of DAF on their cell surfaces. These findings were
supported by another approach using a complement-dependent cytotoxicity
method with the anti- DAF monoclonal antibodies. Abnormal expression of DAF
was found on the progenitors in the bone marrow as well as on mature cells
circulating in the blood in PNH.
Volume 72,
Issue 2,
pp. 507-511,
08/01/1988
Copyright © 1988 by The American Society of Hematology
