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Discordant and heterogeneous expression of GPI-anchored membrane proteins
on leukemic cells in a patient with paroxysmal nocturnal hemoglobinuria
T Shichishima, T Terasawa, C Hashimoto, H Ohto, M Takahashi, A Shibata and Y Maruyama
First Department of Internal Medicine, Fukushima Medical College, Japan.
We performed a flow cytometric analysis using monoclonal antibodies to
decay accelerating factor (DAF) and CD59/membrane attack complex inhibitory
factor (CD59/MACIF) in order to investigate the leukemic cells and
erythrocytes from a patient with paroxysmal nocturnal hemoglobinuria (PNH)
who developed acute myelocytic leukemia. In May 1990, the leukemic cells
comprised 70% of the mononuclear cells in the bone marrow and 76% of those
in the peripheral blood. They consisted of a mixture of positive and
negative populations, including single DAF- positive cells. In August 1990,
almost 100% of the peripheral mononuclear cells were leukemic blasts, and
these consisted of a single population with reduced DAF expression.
Single-color flow cytometric analysis showed that the leukemic cells lacked
CD59/MACIF, while control leukemic cells (n = 3) expressed both DAF and
CD59/MACIF. Leukemic blasts from this patient and six control patients
expressed lymphocyte function-associated antigen 3 and FcIII receptors (CD
16) both before and after treatment with phosphatidylinositol-specific
phospholipase C. The patient's erythrocytes lacking DAF and CD59/MACIF
expression corresponded to the proportion of complement-sensitive cells at
the onset of acute leukemia. These DAF- and CD59/MACIF-deficient
erythrocytes disappeared almost completely with progression of the
leukemia. In conclusion, it appears that the expression of
glycosylphosphatidylinositol-linked membrane proteins by leukemic cells was
heterogeneous and discordant in our patient, and that the leukemic cells
were derived from the PNH clone because of their deficiency of CD59/MACIF.
It is also suggested that DAF could compete more effectively than
CD59/MACIF for a limited number of anchor molecules available on the
proliferating leukemic cells.
Volume 81,
Issue 7,
pp. 1855-1862,
04/01/1993
Copyright © 1993 by The American Society of Hematology
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