Differentiation of blast cells from a Down's syndrome patient with
transient myeloproliferative disorder
J Suda, M Eguchi, Y Akiyama, Y Iwama, T Furukawa, Y Sato, Y Miura, T Suda and M Saito
A male neonate with Down's syndrome and congenital myeloproliferative
disorder was studied. His blood picture showed the unique coexistence of
leukocytosis with matured cells and a large number of blast cells. The in
vitro proliferation and differentiation of blast cells into various
lineages in the presence of phytohemagglutinin-stimulated leukocyte
conditioned medium (PHA-LCM) was examined by using a liquid culture and a
methylcellulose culture system. The differentiation of blast cells into
myeloid cells was confirmed by specific cytochemical stainings, electron
microscopy, and an immunologic study. No specific factors in the plasma of
the patient promoted the proliferation or differentiation of blast cells.
The cellular composition of colonies grown in methylcellulose culture from
single blast cells was studied by a micromanipulation technique. High
plating efficiency was observed. Of 136 cultures, 78 showed colony growth.
Half of the blast cells were colony-forming cells that could proliferate
and differentiate into basophils, neutrophils, eosinophils, macrophages,
and erythrocytes in the presence of PHA-LCM. Using the blast cells with a
high differentiation capacity to the basophil pathway, we studied the
effect of recombinant granulocyte-macrophage colony-stimulating factor (GM-
CSF). Recombinant GM-CSF support neutrophils, eosinophils, and macrophages
but not typical basophils. These findings of the cell differentiation of
blast cells into various kinds of cells in vitro were in agreement with the
finding of neutrophilia, eosinophilia, basophilia, and thrombocythemia in
this patient.
Volume 69,
Issue 2,
pp. 508-512,
02/01/1987
Copyright © 1987 by The American Society of Hematology
