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Peripheral blood cells are predominantly chimeric of affected and normal
cells in patients with paroxysmal nocturnal hemoglobinuria: simultaneous
investigation on clonality and expression of
glycophosphatidylinositol-anchored proteins
H Ohashi, T Hotta, A Ichikawa, T Kinoshita, R Taguchi, T Kiguchi, H Ikezawa and H Saito
Department of Internal Medicine, Nagoya University School of Medicine,
Japan.
To investigate clonal compositions of hematologic cells in paroxysmal
nocturnal hemoglobinuria (PNH), we analyzed peripheral blood (PB) cells of
12 female patients with PNH, by clonality analysis using X- chromosome
inactivation and assessment of expression of
glycophosphatidylinositol-anchored proteins (GPI-APs) by flow cytometry.
Southern hybridization showed that granulocytes were monoclonal in three
and polyclonal in eight patients, respectively, whereas lymphocytes were
polyclonal in all nine patients examined. Expressions of CD16 and CD59 on
granulocytes varied greatly in seven patients examined. Clonality analysis
of granulocytes by the polymerase chain reaction showed that CD59- and
CD59low+ cells were monoclonal, whereas CD59+ cells were polyclonal. It was
shown that PB cells are predominantly chimeric of clonal (GPI-AP- or
GPI-APlow+) and nonclonal (GPI-AP+) cells in PNH, and that degrees of
chimerism differ greatly from patient to patient.
Volume 83,
Issue 3,
pp. 853-859,
02/01/1994
Copyright © 1994 by The American Society of Hematology
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