Chronic myeloproliferative diseases, such as chronic myeloid leukemia
and polycythemia vera, are associated with neutrophil dysfunction. Ver
y little data is available on essential thrombocythemia (ET). In the c
urrent study we evaluated 21 patients with ET. All patients were studi
ed at least 16 weeks after any cytostatic therapy and 10 days after an
y other therapy. Neutrophil functions were investigated as follows: fl
ow cytometric evaluation of whole blood phagocytosis of opsonized FITC
-conjugated E. coli; whole blood chemiluminescence after stimulation w
ith opsonized zymosan and evaluation by an automated, computer-assiste
d luminometer (LB 950, Berthold); and chemiluminescence and superoxide
anion generation by purified neutrophils after f-MLP and PMA stimulat
ion. Chemiluminescence and superoxide anion generation after f-MLP sti
mulation were found to be significantly lower than in normal subjects,
whereas values within the normal ranges were registered after PMA sti
mulation, Phagocytosis-associated chemiluminescence was found to be im
paired both by using zymosan opsonized with autologous plasma and zymo
san opsonized with normal plasma, despite a normal phagocytic activity
. These data show the presence in ET of a complex neutrophil dysfuncti
on that may be related to an impaired signal transduction during both
the phagocytic process and f-MLP stimulation.