Summary
The bleeding syndrome of acute promyelocytic leukemia (APL) is complex and consists
of disseminated intravascular coagulation (DIC) and hyperfibrinolysis. Elastase, derived
from malignant promyelocytes, is believed to mediate the fibrinogeno- and fibrinolysis
by aspecific proteolysis. In this study we measured the role of elastase in fifteen
patients with APL by using an assay for elastase degraded fibrin(ogen) and the results
were compared with those obtained in patients with sepsis induced DIC.
High levels of elastase were observed in sepsis and APL. The levels of fibrinogen
and fibrin degradation products were significantly higher in APL patients compared
to patients with sepsis induced DIC. Nevertheless, the level of elastase degraded
fibrin(ogen) was higher in the sepsis group (635.3 ng/ml, compared to 144.3 ng/ml
in APL; p <0.0001). So, the enormous increase in fibrin and fibrinogen degradation
products in APL cannot be explained by elastase activity. This study suggests a minor
role for elastase mediated proteolysis in the hemorrhagic diathesis in APL patients.
Key words
Acute promyelocytic leukemia - fibrinolysis - coagulation - sepsis - elastase
