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Blood, Vol. 93 No. 2 (January 15), 1999:
pp. 417-424
A Large Proportion of Patients With a Diagnosis of Essential
Thrombocythemia Do Not Have a Clonal Disorder and May Be at Lower
Risk of Thrombotic Complications
Claire N. Harrison,
Rosemary E. Gale,
Samuel J. Machin, and
David
C. Linch
From the Department of Haematology, University College London Medical
School, London, UK.
Essential thrombocythemia (ET) is traditionally considered to be a
clonal disorder. No specific karyotypic abnormalities have been
described, but the demonstration of clonality using X-chromosome inactivation patterns (XCIPs) has been used to differentiate ET from a
non-clonal reactive thrombocytosis. However, these assays may be
difficult to interpret, and contradictory results have been reported.
We have studied 46 females with a diagnosis of ET according to the
Polycythemia Vera Study Group (PVSG) criteria. XCIP results in 23 patients (50%) were uninterpretable due to either constitutive or
possible acquired age-related skewing. Monoclonal myelopoiesis could be
definitively shown in only 10 patients. Thirteen patients had
polyclonal myelopoiesis, and in 8, it was possible to exclude clonal
restriction to the megakaryocytic lineage. Furthermore, there was no
evidence of clonal progenitors in purified
CD34+CD33 and
CD34+CD33+ subpopulations from bone marrow
of 2 of these 13 patients. There was no difference between patients
with monoclonal and polyclonal myelopoiesis with respect to age or
platelet count at diagnosis, duration of follow-up, incidence of
hepatosplenomegaly, or hemorrhagic complications. However, polyclonal
patients were less likely to have experienced thrombotic events
(P = .039). These results suggest that ET is a
heterogeneous disorder, and the clinical significance of clonality
status warrants investigation in a larger study.
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