Journal
BLOOD
Volume 105, Issue 4, Pages 1699-1705Publisher
AMER SOC HEMATOLOGY
DOI: 10.1182/blood-2004-06-2485
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Idiopathic myelofibrosis (IM) is characterized by the constitutive mobilization of CD34(+) cells. IM peripheral blood (PB) CD34(+) cells had a reduced cloning efficiency and a lower frequency of cobblestone areas compared with normal granulocyte colony-stimulating factor (G-CSF)-mobilized PB CD34(+) cells. IM CD34(+) cells engrafted nonobese diabetic/severe combined immunodeficient (NOD/SCID) mice, demonstrating that they contain bone marrow (BM)-repopulating cells. G-CSF-mobilized CD34(+) cells produced multiple hematopoietic lineages within the NOD/ SCID mice with a predominance of CD19(+) cells. By contrast, IM CD34(+) cells produced predominantly CD33(+) cells, increased numbers of CD41(+) cells, but fewer CD19(+) cells. Transcriptional clonality assays of the engrafted human IM cells demonstrated their clonal origin. CD34+ cells from one patient isolated prior to leukemic transformation Were capable of generating acute leukemia in NOD/SCID mice. The engrafted human cells exhibited the same abnormal karyotype as primary cells in a portion of the population. These findings demonstrate that BM-repopulating cells and more differentiated progenitor cells are constitutively mobilized into the PB in IM, and that their differentiation program is abnormal. In addition, the NOD/SCID model may be useful in gaining an Understanding of the events occurring during the transition of IM to acute leukemia. (C) 2005 by The American Society of Hematology.
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