Hematopoietic cells from -spectrin-deficient mice are sufficient to induce thrombotic events in hematopoietically ablated recipients. Blood 1998 Dec 15;92(12):4856-63
Date
12/09/1998Pubmed ID
9845553Scopus ID
2-s2.0-0032534223 (requires institutional sign-in at Scopus site) 13 CitationsAbstract
Thrombotic events are life-threatening complications of human hemolytic anemias such as paroxysmal nocturnal hemoglobinuria, sickle cell disease, and thalassemia. It is not clear whether these events are solely influenced by aberrant hematopoietic cells or also involve aberrant nonhematopoietic cells. Spherocytosis mutant (Spna1(sph)/Spna1(sph); for simplicity referred to as sph/sph) mice develop a severe hemolytic anemia postnatally due to deficiencies in -spectrin in erythroid and other as yet incompletely defined nonerythroid tissues. Thrombotic lesions occur in all adult sph/sph mice, thus providing a hematopoietically stressed model in which to assess putative causes of thrombus formation. To determine whether hematopoietic cells from sph/sph mice are sufficient to initiate thrombi, bone marrow from sph/sph or +/+ mice was transplanted into mice with no hemolytic anemia. One set of recipients was lethally irradiated; the other set was genetically stem cell deficient. All mice implanted with sph/sph marrow, but not +/+ marrow, developed severe anemia and histopathology typical of sph/sph mice. Histological analyses of marrow recipients showed that thrombi were present in the recipients of sph/sph marrow, but not +/+ marrow. The results indicate that the -spectrin-deficient hematopoietic cells of sph/sph mice are the primary causative agents of the thrombotic events.
Author List
Wandersee NJ, Lee JC, Kaysser TM, Bronson RT, Barker JEMESH terms used to index this publication - Major topics in bold
Anemia, HemolyticAnimals
Body Weight
Bone Marrow Transplantation
Cerebellum
Erythrocyte Count
Female
Genetic Markers
Hematopoietic Stem Cells
Kidney
Liver
Male
Mice
Mice, Inbred C57BL
Mice, Mutant Strains
Myocardium
Organ Size
Platelet Glycoprotein GPIb-IX Complex
Spectrin
Spleen
Thrombosis
