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Erythropoiesis in ha/ha and sph/sph mice, mutants which produce
spectrin-deficient erythrocytes
D Brookoff, L Maggio-Price, S Bernstein and L Weiss
In order to characterize chronically accelerated erythropoiesis, we studied
the ultrastructure of bone marrow and spleen of ha/ha and sph/sph mice, two
mutants with profound hemolytic anemia secondary to deficiency of the
erythrocyte membrane protein spectrin. The marrows and spleens of both
varieties were extremely erythropoietic and were without histological
abnormalities directly related to spectrin deficiency. Erythropoiesis was
consistently associated with distinctive, dark branched cells which
constituted large proportions of the stroma of the mutant spleens and
marrow. These dark cells were not present in untreated and acutely bled
controls. Plasma clot assays for erythroid progenitors revealed that CFU-E
concentrations in the mutant marrows were significantly increased over
those in untreated controls while BFU-E concentrations were approximately
half. In addition, mutant CFU-E often gave rise to abnormal appearing
colonies. Spectrin, though crucial to erythrocyte function is probably not
important to the process of erythroid differentiation and maturation. The
status of erythroid precursors in the marrows of the spectrin deficient
mice is similar to that of mice subjected to an acute bleed. The divergent
changes in CFU-E and BFU-E may indicate that these two cells play different
roles in accelerated erythropoiesis. The dark cells that we describe are
similar to stromal cells observed in models of the early stages of
erythropoiesis.
Volume 59,
Issue 3,
pp. 646-651,
03/01/1982
Copyright © 1982 by The American Society of Hematology
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