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Preferential hematopoiesis by paroxysmal nocturnal hemoglobinuria clone
engrafted in SCID mice
N Iwamoto, T Kawaguchi, K Horikawa, S Nagakura, T Kagimoto, T Suda, K Takatsuki and H Nakakuma
Second Department of Internal Medicine, Kumamoto University School of
Medicine, Japan.
In paroxysmal nocturnal hemoglobinuria (PNH), little is known about the
molecular events leading to the clinical manifestations except for the
hemolysis. To unfold the complex pathophysiology, it is necessary to
elucidate the nature of the PNH clone. PNH exhibits an acquired stem cell
disorder, a clonal expansion of affected cells, concomitant depression of
normal hematopoiesis in bone marrow (BM), and, although infrequently, the
development of leukemia. The PNH clone is thus expected to exhibit some
neoplastic features. We report here that CD34+ hematopoietic progenitor
cells of PNH-BM yielded blood cells of three lineages with PNH phenotype
alone when transplanted into sublethally irradiated severe combined
immunedeficient mice. The hematopoiesis persisted for more than 10 months
and did not always need human cytokines. In contrast, the hematopoiesis by
control grafts obtained from healthy volunteers required an intense
cytokine treatment. This in vivo model defines the preferential
hematopoiesis of pluripotent PNH progenitor cells, indicating the intrinsic
growth abnormality of PNH clone.
Volume 87,
Issue 12,
pp. 4944-4948,
06/15/1996
Copyright © 1996 by The American Society of Hematology
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