Reduced incidence of thrombosis in mice with hereditary spherocytosis following neonatal treatment with normal hematopoietic cells. Blood 2001 Jun 15;97(12):3972-5
Date
06/05/2001Pubmed ID
11389042DOI
10.1182/blood.v97.12.3972Scopus ID
2-s2.0-0035877987 (requires institutional sign-in at Scopus site) 12 CitationsAbstract
Thrombosis is a life-threatening complication of hemolytic anemia in humans. Cardiac thrombi are present in all adult alpha-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. (Blood. 2001;97:3972-3975)
Author List
Wandersee NJ, Lee JC, Deveau SA, Barker JEMESH terms used to index this publication - Major topics in bold
AnimalsAnimals, Newborn
Bone Marrow Transplantation
Erythrocyte Transfusion
Female
Graft Survival
Incidence
Mice
Mice, Inbred C57BL
Mice, Mutant Strains
Spherocytosis, Hereditary
Survival Rate
Thrombosis
