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Evidence for a multistep pathogenesis of a myelodysplastic syndrome
WH Raskind, N Tirumali, R Jacobson, J Singer and PJ Fialkow
Somatic cell genetic approaches utilizing the cellular mosaicism present in
women heterozygous for glucose-6-phosphate dehydrogenase (G6PD) have
provided information relevant to the pathogenesis of some neoplastic
disorders. With these techniques, we studied a 61-year-old woman with a
myelodysplastic syndrome. GdB/GdA heterozygosity was demonstrated in skin
and cultured T lymphocytes, which exhibited both A and B type G6PD. In
contrast, erythrocytes, platelets, granulocytes, and marrow nucleated cells
displayed almost exclusively G6PD type B. In addition, 21 of 24
Epstein-Barr virus-transformed B lymphoblastoid lines that expressed a
single immunoglobulin light chain showed only type B G6PD, suggesting that
the stem cells involved by this disease were clonal and could differentiate
to B lymphocytes as well as to mature granulocytes, erythrocytes , and
platelets. Cultured skin fibroblasts and phytohemagglutinin-stimulated
lymphocytes were karyotypically normal, but two independent abnormalities
were found in marrow--47,XX, +8 and 46,XX,del(11)(q23). None of 14 type B
G6PD lymphoblastoid lines analyzed in detail contained these karyotypic
abnormalities, which strongly suggests that a visible chromosomal
alteration is not the sole step in the development of this disease. We
hypothesize that at least two events are involved in the pathogenesis of
this patient's myelodysplasia: one causing proliferation of a clone of
genetically unstable pluripotent stem cells and another inducing
chromosomal abnormalities in its descendants.
Volume 63,
Issue 6,
pp. 1318-1323,
06/01/1984
Copyright © 1984 by The American Society of Hematology
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