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Deficiency of glycosyl-phosphatidylinositol-linked membrane glycoproteins
of leukocytes in paroxysmal nocturnal hemoglobinuria, description of a new
diagnostic cytofluorometric assay
CE van der Schoot, TW Huizinga, ET van 't Veer-Korthof, R Wijmans, J Pinkster and AE von dem Borne
Department of Immunological Hematology, University of Amsterdam, The
Netherlands.
Paroxysmal nocturnal hemoglobinuria (PNH) is a disease that affects not
only red cells, but other blood cells as well. The common defect is
supposed to be an acquired deficiency of glycosyl-phosphatidylinositol
(GPI)-anchored membrane proteins, which may be present already at the
hematopoietic stem cell level. Recently, a panel of monoclonal antibodies
(MoAbs) has become available directed against various GPI- linked membrane
proteins. This makes it possible to study various cell lineages for the
deficiency of such proteins in PNH in more detail. Using cytofluorography,
we could show that the granulocytes of 20 different PNH patients miss not
only GPI-linked FcRIII (CD16 antigen), but also three other GPI-linked
proteins, ie, CD24 antigen, CD67 antigen and a granulocyte-specific 50 to
80 Kd antigen. The affected granulocytes were not only neutrophils but also
eosinophils, as was found in a more detailed analysis of three patients.
Moreover, in all 10 PNH patients tested, the monocytes were found to be
deficient for the GPI-linked CD14 antigen, and we found with CD24 and CD55
(DAF) antibodies that lymphocytes may be involved as well. However,
abnormal B and T lymphocytes were detected only in a subset of patients (2
of 10 tested). The uniform deficiency of GPI-linked proteins of
granulocytes allows the introduction of a new diagnostic cytofluorometric
assay for PNH with MoAbs against GPI-linked granulocytic antigens. This
test was positive in all PNH patients studied and not in a group of 40
control patients or 50 normal donors, with the exception of three of 16
aplastic anemia (AA) patients. In the three AA patients, subpopulations
(10% to 20%) of PNH granulocytes could be detected, whereas these patients
had a negative acidified serum (Ham) test. This indicates that the new test
is more sensitive than the Ham test and allows the early diagnosis of PNH
in AA. An advantage of the neutrophil assay is that, in contrast to the Ham
test, it is not influenced by recent red-cell transfusions. Moreover, it is
possible to quantify the number of affected cells by single cell analysis.
Volume 76,
Issue 9,
pp. 1853-1859,
11/01/1990
Copyright © 1990 by The American Society of Hematology
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