Background Ischemic stroke as a presenting sign of essential thrombocy
themia has been infrequently reported. We describe six patients in who
m cerebrovascular disease was the first manifestation of this myelopro
liferative disease. A positive endogenous megakaryocyte and/or erythro
id colony growth from blood was a diagnostic criterion of essential th
rombocythemia in patients with platelets counts lower than 600 x 10(9)
/L. Case Descriptions These six patients represented 0.54% of all pati
ents with first stroke, 42.8% of all hematologic disorders associated
with stroke, and 12.5% of all patients with essential thrombocythemia
diagnosed from 1986 to 1992 at our institution. Eleven acute cerebrova
scular accidents (6 transient ischemic attacks, 5 definitive cerebral
infarcts) were registered. Mean time from ischemic stroke to diagnosis
of essential thrombocythemia was 4.5 months (range, 1 to 12 months).
The mean platelet count was 597 x 10(9)/L (range, 414 to 760 x 10(9)/L
). Four patients had platelets counts lower than 600 x 10(9)/L. All pa
tients had circulating erythroid progenitors, megakaryocytic progenito
rs, or both. Conclusions Ischemic stroke as a presenting manifestation
of essential thrombocythemia is probably underrecognized. The diagnos
is of thrombocythemia should not be excluded on the basis of platelet
counts lower than 600 x 10(9)/L. The availability of in vitro culture
of hematopoietic progenitors from peripheral blood makes it possible t
o diagnose early and atypical cases.