ABSTRACT
In this chapter, the authors present an outline of the bleeding symptoms, of the associated coagulopathy, and of the therapeutic attempts to combat the hemorrhagic disorder. They discuss the pathophysiological mechanisms proposed and describe their own observations revealing that enhanced fibrinolysis/proteolysis is the predominant cause of the coagulopathy. Except for a severe thrombocytopenia, which is manifest in the early stage of acute promyelocytic leukemia (APL) in almost 90% of the patients, a coagulopathy is almost always observed. Direct evidence for enhanced turnover of coagulation factors in disseminated intravascular coagulation (DIC) or fibrinolysis/proteolysis states may be derived from kinetic studies employing radiolabeled proteins. Over 600 APL patients are available in the literature for reviewing of the management of their coagulopathy and bleeding disorder. In some patients other pharmacological approaches have been applied to combat the coagulopathy also based on the concept that mechanisms other than DIC could be the cause of the coagulopathy.
