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Evidence that essential thrombocythemia is a clonal disorder with origin in
a multipotent stem cell
PJ Fialkow, GB Faguet, RJ Jacobson, K Vaidya and S Murphy
Essential thrombocythemia is characterized by proliferation of
hematopoietic tissue predominantly involving megakaryocytes and resulting
in marked thrombocytosis. The disorder has some clinical and laboratory
features that resemble those seen in the clonal multipotent stem cell
disorders chronic myelogenous leukemia, polycythemia vera, and agnogenic
myeloid metaplasia. It has been argued that essential thrombocythemia
should be classified together with those disorders as a myeloproliferative
syndrome. However, without knowledge of the numbers and types of cells that
are involved in essential thrombocythemia, this suggestion remains
speculative. Three patients with thrombocytosis were studied. The diagnosis
of essential thrombocythemia was considered to be firm in two patients and
probable in the third one. The X-linked glucose-6-phosphate dehydrogenase
locus was used as a cell marker. Whereas both A and B types of
glucose-6-phosphate dehydrogenase were found in nonhematopoietic tissues,
only a single-enzyme type was found in the granulocytes, red cells, and
platelets from each patient. These data indicate that the disorders in
these three patients are clonal and involve multipotent stem cells.
Volume 58,
Issue 5,
pp. 916-919,
11/01/1981
Copyright © 1981 by The American Society of Hematology

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