Preferential hematopoiesis by paroxysmal nocturnal hemoglobinuria clone engrafted in SCID mice

Norihiro Iwamoto, Tatsuya Kawaguchi, Kentaro Horikawa, Shoichi Nagakura, Tadashi Kagimoto, Toshio Suda, Kiyoshi Takatsuki, Hideki Nakakuma

Research output: Contribution to journalArticlepeer-review

45 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)4944-4948
Number of pages5
Issue number12
Publication statusPublished - 1996 Jun 15
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology


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