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Blood, 15 June 2001, Vol. 97, No. 12, pp. 3972-3975
RED CELLS
Reduced incidence of thrombosis in mice with hereditary
spherocytosis following neonatal treatment with normal
hematopoietic cells
Nancy J. Wandersee,
John C. Lee,
Susan A. Deveau, and
Jane E. Barker
From the Jackson Laboratory, Bar Harbor, ME.
Thrombosis is a life-threatening complication of hemolytic anemia
in humans. Cardiac thrombi are present in all adult
 -spectrin-deficient (sph/sph) mice with severe
hereditary spherocytosis, providing a model for events preceding
thrombosis. The current study evaluated (1) the timing of thrombosis
initiation and (2) the effect of postnatal transplantation of normal
cells on life span and thrombotic incidence in adult mice. Thrombi are
detected histologically following necropsy in untreated
sph/sph mice of various ages and are not observed until 6 weeks of age. Thrombotic incidence increases from 50% at 6 to 7 weeks
of age to 100% at 9 weeks of age. As a potential therapy, nonablated
sph/sph neonates were transfused with either genetically
marked normal peripheral blood (PB), bone marrow (BM), or both
and assessed for donor cells and thrombosis. A single transfusion
of PB, with or without BM, significantly increases the percentage of
sph/sph mice that survive to weaning (4 weeks of age).
Replacement in all sph/sph recipients is limited to red
blood cells (RBCs). RBCs derived from donor PB are lost within 5 weeks.
PB plus BM prolongs high-level donor PB cell production better
than BM alone. Thrombotic incidence is significantly reduced in all
sph/sph mice treated with PB, BM, or both. Hence, the
presence of normal blood cells in the peripheral circulation of
neonatal and adult sph/sph mice rescues the former and
abrogates the development of thrombosis in the latter.
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