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PLENARY PAPER
From the Laboratoire d'hématologie, AP-HP,
Faculté de Médecine Paris XI, INSERM U473, Hôpital de
Bicêtre, Le Kremlin Bicêtre, France; Bayer
Diagnostics, Tarrytown, NY; Laboratoire d'hématologie,
Hôpital Bichat- Claude Bernard, Paris, France; and
Lawrence Berkeley National Laboratory, Berkeley, CA.
Spherocytic red cells with reduced membrane surface area are a
feature of hereditary spherocytosis (HS) and some forms of autoimmune
hemolytic anemia (AIHA). It is generally assumed that membrane loss in
spherocytic red cells occurs during their sojourn in circulation. The
structural basis for membrane loss in HS is improper assembly of
membrane proteins, whereas in AIHA it is due to partial
phagocytosis of circulating red cells by macrophages. A hypothesis was formed that these different mechanisms should lead to temporal differences in surface area loss during red cell genesis and during sojourn in circulation in these 2 spherocytic syndromes. It was proposed that cell surface loss could begin at the
reticulocyte stage in HS, whereas surface area loss in AIHA involves
only circulating mature red cells. The validity of this hypothesis was
established by documenting differences in cellular features of
reticulocytes in HS and AIHA. Using a novel technique to quantitate
cell surface area, the decreased membrane surface area of both
reticulocytes and mature red cells in HS compared with normal
cells was documented. In contrast, in AIHA only mature red
cells but not reticulocytes exhibited decreased membrane surface area.
These data imply that surface area loss in HS, but not in AIHA, is
already present at the circulating reticulocyte stage. These findings
imply that loss of cell surface area is an early event during genesis
of HS red cells and challenge the existing concepts that surface
area loss in HS occurs predominantly during the sojourn of mature red
cells in circulation. This article has been cited by other articles:
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| Copyright © 2001 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||